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1. CNS Tumours
Written by: Abdullatif Aydin from Bart's and The London,
Brain tumours occur in approximately 7-21 persons per 100 000 per year. They are responsible for 2% of
cancer deaths and 2% of reported neoplasms.
Metastases are the most common intracranial tumours
Astrocytomas are the most common primary brain tumours
Meningiomas are the commonest benign tumours together with acoustic neuromas
Pathological classification
WHO Classification of intra-cranial tumours (Kleihus & Cavenee, 2000) is based on their tissue of origin.
Tumours of Neuroepithelial Tissue
- Astrocytomas are the most common primary brain tumours. They are classified into 4 grades (see
N.B. Grade IV tumours (GBM) are the most malignant type and have a poor prognosis. Patients usually
die within several months.
- Oligodendrogliomas are usually slow-growing, sharply defined tumours that originate from

Tumours of the Nerve Sheath
A schwannoma is a non-invasive, slowly growing tumour of the Schwann cells that surround cranial
nerve roots or peripheral nerves. The most common is a vestibular schwannoma (acoustic neuroma),
which grows around the root of CN VIII at the cerebellopontine angle.
Neurofibromas originate from Schwann cells, fibroblasts or perineural-like cells (cells that form and
support the nerve sheath). They involve spinal nerve roots or peripheral nerves but rarely cranial
nerves and have a greater tendency to undergo malignant change than Schwannomas.
Primary CNS lymphoma (PCNSL): forms around periventricular parenchymal blood vessels. It may be
solitary or multifocal and generally occurs in immunocompromised patients.

Germ Cell Tumours
A germinoma is a primitive spheroidal cell tumour comparable to seminoma of the testis
A teratoma is a tumour containing a mixture of well differentiated tissues dermis, muscle, bone.

Tumours of the Sellar Region
- Pituitary adenomas are benign tumours and often secrete pituitary hormones:
Prolactinomas are the most common, accounting for 40-50%
GH-secreting tumours account for 20% (associated with acromegaly)
ACTH-secreting tumours form 10-15% (associated with Cushing's disease)
TSH-secreting tumours are the least common pituitary adenomas (associated with both hypo- and
Gonadotropin-secreting adenomas rarely cause a clinical syndrome and account for 20% of non-
functioning pituitary adenomas.
- Craniopharyngiomas arise from the embryonic remnants of Rathkes cleft and lie in close proximity to
the pituitary stalk. They are usually nodular tumours with cystic areas containing greenish fluid and
cholesteatomous material

Tumours of the Meninges
Meningiomas are tumours that arise from the arachnoid granulations, usually closely related to the
venous sinuses.
They are mostly benign but some undergo sarcomatous change and present in similar ways to
malignant neoplasms.
Meningiomas compress rather than invade the adjacent brain.

Metastatic Tumours
These may arise from any primary site but most commonly arise from bronchial or breast tumours.
Nervous system metastases occur in 25% of patients with disseminated cancer
Classification according to site


Supratentorial Tumours
- Raised intracranial pressure (ICP)
Headache, vomiting, depressed conscious level
- Focal neurological deficit
- Usually relentlessly progressive
- Seizures

Posterior Fossa Tumours
- Never present with seizures
- Focal deficits
CN lesions (e.g. diplopia, facial numbness/weakness, dysphagia)
Cerebellar lesions (ataxic gait, dysarthria, dysmetria)
Brainstem compression (decreased consciousness/coma)
- Relentless progression
- Obstructive hydrocephalus causes symptoms of raised ICP, which may develop rapidly.
Pituitary Tumours
- Focal neurological deficit
Chiasmatic compression - bitemporal hemianopia
Cavernous sinus syndrome - diplopia, facial numbness
- Hydrocephalus from compression of the third ventricle from below
- CSF leak
- Pituitary apoplexy (resulting from either acute haemorrhage or ischaemic necrosis)
- Endocrine presentation; panhypopituitarism, amenorrhoea, impotence, acromegaly (adults), gigantism
(paediatrics), Cushings disease
Paediatric brain tumours are not covered in depth in this article. They are almost always in the
posterior fossa and may be one of the following:
Pilocytic astrocytoma
They present with failure to thrive, developmental delay and hydrocephalus.
Management is with surgery followed by radio- and chemotherapy.

The following tests may confirm the presence of a brain tumour and find its location:
CT scan - will detect >90% of tumours. However, it is not very sensitive for:
Small pituitary tumours
Tumours adjacent to bone
Brain stem tumours
Low grade astrocytomas
Small metastases
Gadolinium-enhanced cranial MRI - preferred study for the initial anatomic evaluation of CNS
tumours. Most tumours are hypointense on T1-weighted images and hyperintense on fluid-attenuated
inversion recovery, T2-weighted, and proton-weighted images.
Biopsy of the tumour during surgery or CT-scan may confirm the type of tumour
EEG - used primarily to complement CT and MRI by evaluating functional changes in the patient's
condition. It demonstrates aspects of brain physiology that are not reflected in structural neuroimaging.
CSF analysis - Lumbar puncture contraindicated in many patients with brain tumours, but may be
useful in some where it may show cancerous changes.
Many other imaging techniques (see table below), including MR spectroscopy, diffusion and perfusion
imaging, single photon emission computed tomography (SPECT) and positron emission tomography
(PET) may help to identify foci of high-grade tumour prior to surgery, thereby improving the accuracy of
histopathological evaluation.

The aims of management is diagnosis, symptomatic relief and tumour control (with minimal morbidity for
malignant disease and maximal aggression for benign disease).

Conservative Management
- Immediate introduction of palliative care may be appropriate when there is no doubt regarding
diagnosis and if the patient is in extremis.
- Asymptomatic lesions may be left alone, especially in the elderly and in surgically difficult spots.
- Watch and wait with serial imaging for:
Small acoustic neuroma - 66% show no growth
Small meningiomata - mean doubling time of 26 years
Low grade glioma - radiation and (for oligodendrogliomas) chemotherapy both have a role, but it
may not matter when they are given

Medical Management
- Corticosteroids (Dexamethasone) for ICP (reduces vasogenic oedema). Steroids may also
temporarily improve focal neurological deficits by treating brain oedema.
- Anti-epileptic drugs for epilepsy. Carbamazepine, lamotrigine, oxcarbazepine, sodium valproate, and
topiramate are the drugs of choice for partial (focal) seizures.
Phenytoin first line anti-epileptic in neurosurgery as it can be given quickly
- Prolactinomas respond well to dopamine agonists and these are the treatment of choice. Acromegaly
can be treated with Octreotide and Pegvisomant.

Radiation Therapy
- Postsurgical radiation is the standard treatment for high-grade gliomas.
- Stereotactic radiosurgery used to palliate small, well-demarcated recurrent glioblastoma multiforme
and as a boost after conventional external beam radiotherapy.
- Interstitial radiotherapy (brachytherapy) involves surgically implanting radioactive material directly
inside the tumour.

- Combining Temozolomide therapy with radiation improves survival in patients with high-
grade gliomas.
- Irinotecan and targeted agents such as Bevacizumab, which inhibits vascular endothelial growth
factor; and Gefitinib, Erlotinib, and Imatinib, which inhibit the epidermal and platelet-derived growth factor
receptors, have shown some promise in the treatment of recurrent malignant gliomas.
Surgical Management
Most patients with intracranial tumours require one of the following surgical approaches (as shown in the

The surgical procedure may involve biopsy, debulking/cytoreduction or complete removal of the tumour.
However this depends upon the nature of the tumour and its site.
Primary malignant tumours are infiltrative in nature and hence surgery is restricted to partial removal
(debulking) or biopsy.
Benign tumours such as meningiomas or craniopharyngiomas have better prospects following
complete removal.
It is important to note, that if any of the tumour tissue is overlooked, or if fragments remain attached to
deep structures, recurrence may be seen.

Craniotomy and radiotherapy prolong prognosis for glioblastoma multiforme from 4 to 14 months.
Surgery can prolong survival with single metastasis from 15 to 40 weeks.
For low grade glioma, prognosis is 5-7 years with inevitable deterioration associated with tumour
Lindsay KW, Bone I, Fuller G. Neurology and Neurosurgery Illustrated. 5th ed. Edinburgh; New York:
Churchill Livingstone; 2010.
Ginsberg L. Lecture Notes: Neurology. 9th ed. UK: Wiley-Blackwell; 2010.
Jasmin L. Brain tumor - primary - adults [online] 2011. Available
from: [accessed 19/05/2012].
Chandana SR, Movva S, Arora M, Singh T. Primary Brain Tumors in Adults. American Family Physician
2008; 77(10): 1423-30
NICE. Guidance on Cancer Services: Improving Outcomes for People with Brain and Other CNS
Tumours - The Manual. June 2006: Developed by the National Collaborating Centre for Cancer.
Bromfield EB, Benbadis SR et al. EEG in Brain Tumors [online] 2009. Available
from: [accessed 19/05/2012].

2. Epilepsy
Written by: Hannah Shereef from Birmingham University,

A seizure is defined as transient signs or symptoms due to abnormal, excessive or synchronous
neuronal activity in the brain.
Incidence: approx 80/100 000 per year
Lifetime prevalence: 9% (1/3 benign febrile convulsions)

Epilepsy is a tendency towards recurrent seizures unprovoked by systemic or neurological insults.
It is a functional disorder of complex cortical circuits. For a given patient, seizures tend to be
Incidence: approx 50/100 000 per year
Prevelance: 0.5-1% (5x higher in developing countries).

A convulsion is the motor signs of a seizure.

Types of epilepsy

Causes of epilepsy

Status epilepticus

Medical Emergency (10-15% mortality)
Continuous seizures without regaining consciousness
50% cases occur without previous history of epilepsy

1. Ensure airway is maintained during seizure and postictal coma.
2. Treat prolonged seizure with rectal (10mg) or IV diazapem or midazolam. If hypoglycaemic, administer
IV glucose.
3. Treat quickly in ITU with cardiorespiratory support

Sudden Unexpected Death in Epilepsy (SUDEP)

Mortality rate is 3x higher in epilepsy patients than general population.
More common in uncontrolled epilepsy.
May be related to nocturnal seizure-associated apnoea or asystole.


Epileptic activity indicated by spike and wave abnormalities.
Patients with epilepsy can have normal EEG between seizures.

CT/MR Imaging
Indicated for all focal cases with associated symptoms e.g. first onset status epilepticus and
Aids diagnosis of tumors.


Anti-epileptic drugs (AEDs)
Indicated where there is a confirmed diagnosis of epilepsy and the risk of reccurence is high.
Drug levels should be closely monitored. There may be side effects (e.g. ataxia, nystagmus,
Dual therapy necessary in <10%.
Despite AED therapy, in 20-35% seizures persist.
Controlled epilepsy may remain in remission. It is possible to withdraw AEDs, although less than
50% of attempts at AED withdrawal are successful.

AED Treatments for Epilepsy

Neurosurgical treatment
If a single epileptogenic focus can be identified such as hippocampal sclerosis or a small low-grade
tumor, neurological resection e.g. temporal lobectomy may be indicated and offers up to 70% chance of

Vagal nerve stimulation
Vagal nerve stimulation has been shown to reduce seizure frequency and duration in 1/3 of patients.

Driving and epilepsy

Patients with epilepsy must be seizure free for at least 12 months to be legally permitted to drive in the
UK/EU. The patient must inform the DVLA.
Women and AEDs

Teratogenicity of AEDs: 5 mg/d folic acid supplements should be taken by all women of child-bearing
age. Valproate should be avoided. Lamotrigine is recommended.
The risk of fetal abnormality is 5%. Pre-conceptual counselling should be offered.

Breastfeeding: Most AEDs present in breast milk (except carbemazepine and valproate). Lamotrigine is
is not known to be harmful to infants.

Enzyme inducing AEDs may interfere with the effectiveness of oral contraceptives, so higher doses
may be required (50g oestrogen).


Clark M, Kumar P. 2009 Kumar & Clarks Clinical Medicine 7/e Saunders
Longmore M, Wilkinson I, Davidson E, Foulkes A, Mafi A. 2010 Oxford Handbook of Clinical
Medicine 8/e Oxford OUP

3. Facial Nerve Palsy
Written by: Dhruv Panchal from Oxford Deanery,

Facial nerve palsy is a common and often distressing presenting complaint for patients. They are often
worried they have suffered a stroke. Although this is one cause of this condition, a thorough history and
examination with a sound understanding of the neuroanatomy can help decipher the underlying cause
and direct appropriate treatment.

Functional anatomy
When faced with a patient with a facial nerve palsy, the first question you must consider is if this is an
upper or lower motor neurone lesion. An upper motor neurone lesion will classically spare the forehead
muscle frontalis, and so there will be preservation of forehead wrinkles when the patient raises their
eyebrows. This is not the case with a lower motor neurone lesion.

When asked, many think the following statement is true: The forehead is spared in upper motor neurone
lesions because there is bilateral innervation of frontalis. Strictly speaking, this is wrong. There is
bilateral cortical representation of frontalis. The former statement implies the left facial nerve innervates
both left and right frontalis and vice versa, but this is not true. Consider Figure 1, a depiction of a patient
with a right hemispheric stroke.

This patient has a right cortical defect and is asked to look up and smile. The patient cannot raise the left
side of his mouth, but there is forehead sparing as evidenced by creases along the length of the
forehead. The left cortex innervates the right upper and lower facial nucleus but only the upper part of
the left facial nucleus. This is the explanation behind bilateral cortical representation of the forehead as
both cortices can control both sides of frontalis. This cortical representation is also situated in the
anterior cingulated cortex rather than the motor cortex. This explains the dissociation between voluntary
and emotional facial movements in some patients classically a patient will have a normal smile but an
impairment when they are asked to show their teeth (as seen in figure 2).
Fig 2: Volitional-emotional dissociation of facial movements

If an upper motor neurone lesion is suspected, a good clinical history and examination can often localise
the lesion to either the cortex or brainstem. Cortical lesions, often strokes, will have symptoms and signs
in the contralateral limbs, whereas brain stem lesions may have crossed cranial nerve signs e.g. upper
motor neurone facial nerve palsy with a 3rd nerve palsy suggests a midbrain lesion. At this point, further
imaging is usually indicated to characterise the exact location and nature of the lesion. These options will
be discussed later.

If a lower motor neurone lesion is suspected i.e. the forehead is not spared, an understanding of the
facial nerve route can help locate where in the course of the facial nerve it has got damaged. Figure 3 is
a schematic diagram showing the course of the facial nerve:
Fig 3: Facial nerve anatomy

The origin of the facial nerve is shown in the left panel of figure 3. This is a cross section of the pons
where the nucleus of the facial nerve originates (VII). Note the facial nerve actually takes a slight detour,
looping around the VIth nerve nucleus before exiting the ventral surface of the pons alongside the VIIIth
nerve and the nervus intermedius. This detour is important to know as nuclear facial nerve lesions are
often associated with ipsilateral 6th nerve palsies.

The facial nerve, along with the VIIIth nerve and nervus intermedius occupies a space called
thecerebellopontine angle before entering the facial canal via the internal auditory meatus (number
1 in the right panel fig 2). The facial nerve travels a short distance to merge with the nervus intermedius
to form the geniculate ganglion (numbers 2 and 3). This ganglion is important as it supplies nerves to
the lacrimal and nasal glands via the greater superficial petrosal nerve and the sphenopalatine ganglion.
The main bulk of the facial nerve continues giving a small branch to stapedius (number 4). This tiny
muscle contracts to dampen down the transmission of loud and potentially harmful sounds to the ear
drum by reducing the oscillation of the ossicles. If this nerve is damaged, patients become sensitive to
loud noises (hyperacusis). This is therefore a useful question to ask to localise the lesion proximal to
this branch of the facial nerve. Before leaving the facial canal via the stylomastoid foramen,
the chorda tympani branches from the main facial nerve to supply taste to the anterior 2/3rds of the
tongue as well as salivary gland stimulation via the submaxillary ganglion. Again, asking about
ageusia (loss of taste) can help localise a lesion proximal to the origin of the chorda tympani. As the
facial nerve exits the stylomastoid foramen, it gives off a small sensory nerve called the posterior
auricular nerve (number 5) which supplies a small patch of skin behind the ear. This may explain the
ear pain some patients get in the days preceding Bells palsy and is certainly affected when varicella
zoster virus infects the facial nerve resulting in vesicles around the ear. Finally the facial nerve travels
within the parotid gland (not shown) to split into its five main motor branches serving the face
temporal, zygomatic, buccal, marginal mandibular and cervical (sometimes remembered as
ToZanzibar By Motor Car). A parotid mass with a facial nerve palsy could indicate infiltration and
therefore should be treated as a malignancy until proven otherwise.

The facial nerve functions which are clinically important can be summarised as the following:

Causes of Facial nerve palsy
The causes of facial nerve palsy may be categorised by anatomical location and pathological process.
Upper motor neurone lesions may be caused by a lesion in the motor cortex, the subcortex or
corticobulbar tracts. Commonly the aetiology is vascular in origin (i.e. stroke) but can include masses
(e.g. neoplasm, abscess) or neurodegenerative disease (e.g. a supranuclear palsy can cause a facial
apraxia even in the absence of overt muscle weakness).

Lower motor neurone lesions can be categorised anatomically to a few key areas in the facial nerve
1) Nuclear: vascular, inflammatory and occasionally infiltrative pathology can affect the facial nerve
nucleus or intrapontine fascicle. It is usually associated with an ipsilateral 6th nerve palsy and may also
affect the descending corticospinal tracts causing a contralateral limb weakness the Millard Gubler
2) Cerebellopontine angle syndrome: the cerebellopontine angle is a triangular recess bordered by
the pons, cerebellar peduncle and petrous temporal bone laterally. Its contents include the Vth nerve
superiorly, the IXth and Xth nerve inferiorly and the VIIth and VIIIth nerve in between. One of the first
signs of this syndrome is the loss of corneal reflex on the ipsilateral side. It is usually caused by an
acoustic neuroma but can also be caused by meningiomas, metastases, cholesteotomas or even
3) Facial canal: the proximal part of the canal (called the labyrinthine portion) is particularly prone to
ischaemia and compression due to its relatively poor blood supply and small calibre. Transverse
fractures of the temporal bone are a common cause of facial nerve injury. Infection such as malignant
otitis externa or suppurative otitis media can spread to the skull base leading to facial nerve palsy.
Varicella zoster infection affecting the geniculate ganglion can lead to a facial nerve palsy with vesicles
around the ear. This is called Ramsay Hunt syndrome. Neoplastic processes including metastases or
nasopharyngeal carcinoma can also affect the facial nerve here. Finally, it is thought that damage to the
nerve in the facial canal causes Bells palsy.
4) Parotid region: a parotid mass with a facial nerve palsy should be treated with a high index of
suspicion for malignancy. Inflammatory parotitis from infection or granulomatous conditions e.g.
sarcoidosis can cause facial nerve lesions. Other aetiologies distal to the stylomastoid foramen include
iatrogenic e.g. complication of carotid endarterectomy or temporal artery biopsy.

Examination technique for when a patient presents with facial nerve palsy:
A full cranial and peripheral nerve examination should be performed. Once you decide whether the
lesion is upper motor neurone or lower motor neurone, the examination should be tailored to find more
clues as to the location of the lesion and potential complications of facial nerve palsy.

Looking for the location of the lesion:
For upper motor neurone lesions, evidence of forehead sparing is usually sufficient. An examination of
the other cranial nerves should be performed to see if this is a lesion in the corticobulbar tracts, in the
brainstem or higher up. The limbs should be examined for increased tone, hyperreflexia, pyramidal
weakness and upward going plantars.

For lower motor neurone lesions, answering the following questions may help to localise the lesion:
A) Look:
Are there any scars? E.g. surgical (parotid removal, carotid endarterectomy) or trauma?
Is there parotid swelling to suggest a malignant or inflammatory parotid mass?
Is there a rash? Vesicles around the ear suggest varicella zoster infection (Ramsay Hunt syndrome),
inflammation or pus from the ear may indicate malignant otitis externa.
Are the ears clear on otoscopy? This may reveal otitis media or cholesteotoma.
Are there retinal changes associated with hypertension or diabetes? Both of these conditions are
associated with idiopathic facial nerve palsy.

B) Feel:
Is there mastoid tenderness? This may suggest infection spreading from the middle ear
Is there an ipsilateral corneal reflex? Absence is an early sign of cerebellopontine angle syndrome.

C) Move:
Is there an ipsilateral 6th nerve palsy? This may suggest a pontine lesion.

Looking for complications of facial nerve palsy:
The main complications include drying of the eyes, particularly at night where the eyes cannot stay shut.
This may lead to an exposure keratitis. Some patients with persistent facial nerve palsy may have their
eyelid surgically manipulated to correct for this an operation called tarsorrhaphy.Patients can also
suffer from epiphora excessive lacrimation due to orbicularis oculi muscle weakness which impairs the
pumping action of the canaliculi. Drooling may occur around the mouth and drinking may also be
Bilateral facial nerve palsy
This is an unusual occurrence and usually prompts a diagnostic hunt for the underlying cause. The top
three causes of this condition include:
1. Sarcoidosis: a granulomatous disease which can affect almost any organ but most commonly the
lungs. It is usually treated with immunosuppressants such as steroids. Diagnostic clues include
hypercalcaemia, raised serum angiotensin converting enzyme and bilateral hilar lymphadenopathy on
chest X-ray.
2. Myasthenia gravis: this autoimmune neuromuscular junction disease is characterised by fatigueability.
Although not strictly a cause of facial nerve palsy, it can mimic it. Treatment is with anti-cholinesterase
inhibitors to increase synaptic acetylcholine concentration. Diagnostic clues include symptoms
worsening at the end of the day and a positive Tensilon test; it is sometimes associated with a
3. Guillain-Barr syndrome: this is an ascending inflammatory peripheral neuropathy which is usually
preceded by a gastrointestinal infection. Lung function tests should be performed as a reduction in
lung capacity can occur from reduced mechanical ventilation. Treatment is with intravenous

Other causes of bilateral facial nerve palsy may also be categorised by the anatomical location of the
1. Central bilateral cerebellopontine angle tumours e.g. in neurofibromatosis type 2
2. Peripheral nerve disease motor neurone disease, Lyme disease, Leprosy
3. Muscular myopathy

There are also some rare conditions which cause facial nerve palsies including:
Rosenthal Melkersson syndrome: this is characterised by seventh nerve palsy, facial oedema and
tongue fissuring. Symptoms occur from teenage years and recurrent facial nerve palsies have been
Moebius syndrome: a rare neurological disease where children are born with facial nerve and
abducens nerve underdevelopment leading to facial muscle weakness and inability to abduct the

Bells Palsy
In essence, Bells palsy is an acute lower motor neurone facial nerve palsy which was previously thought
to be of unknown cause but this opinion is changing (see pathology). Provided is a summary of the
disease characteristics:
1) Incidence: 20/100,000 per year (1)
2) Age: may occur in childhood but risk increases with age.
3) Sex: equally affected
4) Geography: no correlation with location
5) Predisposing factors: weak associations with diabetes (2) and hypertension, risk is three times
greater during pregnancy, particularly during the 3rd trimester or in the first postpartum week (3)
6) Pathology: Detection of herpes simplex virus type I DNA in endoneurial fluid is present in most
patients (4) . Both primary infection and reactivation of latent infection have been implicated.
7) Clinical features:
Patients often develop unilateral facial weakness over hours and complain of eyebrow sagging,
inability to close the eye or disappearance of the nasolabial folds.
Depending on the location of the lesion, patients may also complain of hyperacusis, loss of taste on
the anterior 2/3rds of the tongue, and decreased tear production.
The main way to distinguish upper from lower motor neurone disease is by observing for forehead
sparing. However, a lower motor neurone lesion can occasionally give forehead sparing if the facial
nerve is damaged in the pes anserinus near the parotid. This lesion spares the temporal branch thus
sparing the frontalis.
8) Diagnosis:
Diffuse facial nerve involvement with facial muscle paralysis, with or without loss of taste on the
anterior 2/3rds of the tongue, or altered secretions of the lacrimal and salivary glands.
Onset is acute, over 24-48 hours; maximal weakness is within 3 weeks or less from the first day of
visible weakness; recovery is within 6 months to some extent.
9) Differential diagnosis:
A diagnosis of Bells palsy is doubtful if some facial function has not returned within 3-4 months (5).
Additional investigation is required.
Prolonged, slowly progressive or facial palsy affecting only one or two branches could suggest
Herpes Zoster is diagnosed if vesicles can be seen around the external meatus. Occasionally,
herpes zoster is seen without vesicles (zoster sine herpete). A history of dermatomal pain or
dysasthesia may be suggestive of this diagnosis.
10) Investigations:
There is no gold standard diagnostic investigation
Electrodiagnostic studies help determine prognosis: Compound muscle action potential (CMAP) at
10 days; those with 10% of normal CMAP have a poor prognosis, those with 2% have a very poor
prognosis (6); facial nerve stimulation is most useful within two weeks after progression to complete
facial paralysis if surgery is contemplated.
Imaging is indicated if a malignancy is suspected. This may be prompted by atypical signs, slow
progression beyond three weeks, no improvement at six months, facial twitch or spasm preceding
paralysis (which suggests nerve irritation from tumour). (7)
11) Treatment
Conservative educate the patient and reassure them that they have not had a stroke! Monitor the
patient to ensure this is Bells palsy and there is no sinister underlying cause.
Medical If quickly diagnosed within 2-3 days, high dose steroids with or without antivirals can be
used. Steroids improve the rate of complete recovery . Regardless of this, eye care is important
this can include artificial tears for dry eyes and taping the eyes shut at night to prevent exposure
12) Complications:
Failure to recover: prognosis depends on the degree of initial weakness - the less the better.
Synkinesis this is where the regenerating facial nerve fibres are thought to erroneously innervate
different targets. Examples include eyes closing whilst muscles of the mouth contract, lacrimation
during eating (crocodile tears) or smiling causing eye closure.
Recurrence this is uncommon, and occurs in around 1 in 10 people.
1) Peitersen E. The natural history of Bell's palsy. Am J Otol. 1982;4(2):107.
2) Mountain RE, Murray JA, Quaba A, Maynard C. The Edinburgh facial palsy clinic: a review of three
years' activity. J R Coll Surg Edinb. 1994;39(5):275
3) Hilsinger RL Jr, Adour KK, Doty HE Idiopathic facial paralysis, pregnancy, and the menstrual cycle.
Ann Otol Rhinol Laryngol. 1975;84(4 Pt 1):433
4) Murakami S, Mizobuchi M, Nakashiro Y, Doi T, Hato N, Yanagihara N. Bell palsy and herpes simplex
virus: identification of viral DNA in endoneurial fluid and muscle.Ann Intern Med. 1996;124(1 Pt 1):27.
5) Hashisaki GT. Medical management of Bell's palsy. Compr Ther. 1997;23(11):715.
6) Esslen E. Investigations on the localization and pathogenesis of meato-labyrinthine facial palsies. In:
The Acute Facial Palsies, Esslen E (Ed), Springer-Verlag, Berlin 1977. p.41.
7) Gilden DH. Clinical practice. Bell's Palsy. N Engl J Med. 2004;351(13):1323.

4. Head Injury
Written by: Yee Yen Tan from Manchester University,
6-10 deaths per 100,000 per annum
70% male
40% children
90% mild (GCS 13)
10% moderate (GCS 9-12) and severe (GCS 8)
Most common = fall and assault, followed by road traffic accident
Alcohol involved in up to 65% of cases
The introduction of seat belt regulation, air bag, crash helmet and alcohol control have prevented
many severe head injuries.

Impact damage often unavoidable
1) Cortical contusion and laceration
Cortical contusion = bruising on the brain, commonly affecting the frontal and temporal lobes.
Especially common at the under or opposite the site of impact (contra-coup)
2) Diffuse axonal injuries
Disruption and tearing of axons form the mechanical shearing following deceleration.

Secondary brain damage occurs after initial injury, may be preventable
1) Intracranial haematoma
Extradural (27%): Bleeding of the middle meningeal artery into the extradural space
Subdural (26%): Rupture of the bridging veins from cortical surface to the venous sinus
Intracerebal subdural (38%)
Extra intradural (8%)
2) Cerebral swelling
From vasodilation and oedema
3) Tentorial or tonsillar herniation
Supratentorial haematoma Increasing ICP Midline shift Lateral tentorial herniation
(Herniation of the medial temporal lobe) Midbrain compression and damage
Uncontrolled lateral tentorial herniation Diffuse bilateral hemispheric swelling Central tentorial
Central tentorial herniation Tonsillar herniation (Herniation of the cerebellar tonsils through the
foramen magnum) Brainstem compression
4) Cerebral ischaemia
Hypotension + Increased ICP Reduced cerebral perfusion
Reduced cerebral perfusion Hypoxia Cerebral ischaemia
5) Infection
Skull fracture Dural tear Meningitis Cerebral abscess
Seldom within 48 h of injury
Meningitis may develop after months or years
Assessment and Immediate Management
Airway Any obstruction? Is it maintained? Oropharynheal airway needed?Cervical spine Is
immobilization needed?Breathing Administer oxygen. Is respiratory movement
adequate?Circulation Is there hypotension or tachycardia? Large bore cannula and IV fluid. Take
blood including group and save/cross matchDisability Pupils equal and reactive to light? GCS or
AVPU? Consider intubation if GCS 8.Exposure/Everything else
Chest: Flail segment, haemo/pneumothorax
Abdomen: Bleeding (Pain, tenderness, rigidity, bruising)
Head and spine: Focal deficits, conscious level
Limbs: Lacerations and fractures

History for head injury
Mechanism of injury
Duration of loss of consciousness
Duration of post-traumatic amnesia
Presence of headache or vomiting
Presence of seizure
Cause and circumstances of injury

Examination of head injury
Sign of depressed fracture of the skull
- Explore deep laceration with a gloved finger
Sign of basal skull fracture
- CSF otorrhoea or rhinorhoea. Clear fluid from the ear or nose. Dipstick test reveals glucose
- Bilateral periorbital haematoma. Black eyes with no associated damage around the eyes
- Subconjuctival haemorrhage extending to the posterior limit of the sclera
- Battles sign. Brusing over mastoid.
Conscious level
Pupillary response
- Medial temporal lobe herniation through the tentorial hiatus compresses the occulomotor nerve (III)
Dilated pupil with impaired or absent reaction to light on the ipsilateral side of lesion
Focal neurological deficits
- Signs of upper motor neuron lesions from limb examination. Increased tone, decreased power, brisk
reflexes, upgoing extensor plantar, impaired coordination
- Cranial nerve palsy.
Glasgow Coma Scale (GCS)
Motor response
6 Obey command
5 Localize pain
4 Withdraw from pain
3 Abnormal flexion to pain
2 Abnormal extension to pain
1 No movement
Vocal response
5 - Orientated
4 - Confused speech
3 - Inappropriate words
2 - Incomprehensible sound
1 No sound
Eye opening
4 - Spontaneously
3 - To voice
2 - To pain
1 - No eye opening

Generally head injuries are classified as follows:
Severe GCS 8
Moderate GCS 9 - 12
Minor GCS 13

Further Management
Further management aims to prevent secondary brain damage
Ensure that airway is patent and blood oxygenation is adequate. Hypoxia worsens cerebral damage.
Urgent evacuation of space occupying haematoma. If conscious level deteriorates, consider
Monitor intracranial pressure (ICP) and blood pressure (BP) to maintain adequate cerebral perfusion
pressure (CPP). CPP = Mean BP ICP
Anticonvulsants if seizure occurs
Surgical repair of persisting dural defect

C-spine immobilization until full risk assessment including clinical assessment imaging for:
GCS 15 on initial presentation
Neck pain or tenderness
Focal neurological deficit
Parasthesia in the extremities
Any clinical suspicion of cervical spine injury

Any other clinical suspicion of cervical spine injury CT within an hour for:
GCS 13 on initial assessment in the emergency department;
GCS 15 at 2 hr after the injury;
Suspected open or depressed skull fracture;
Any sign of basal skull fracture;
1 episode of vomiting in adult; 3 episodes of vomiting in children;
Post-traumatic seizure;
Coagulopathy (eg clotting disorder, warfarin) + some loss of consciousness or amnesia; or
Focal neurological deficit

CT within 8 hour (or immediately if present 8hr after injury) for:
None of the above indication (for CT within an hour) and either:
Amnesia for 30mins before impact;
Age 65 years + some loss of consciousness or amnesia; or
Some loss of consciousness or amnesia + dangerous mechanism of injury, for example
- A pedestrian struck by a motor vehicle
- ejected from motor vehicle
- fall from a height greater than 1m or 5 stairs
CT Diagnoses Not To Be Missed

Outcomes after Head Injury
Outcomes after severe head injury
Predicted by the initial GCS and the duration of coma
Residual disability physically (hemiparesis, dysphasia, epilepsy) and mentally (intellect, memory,
Most recovery occurs in the first 6 months. Physiotherapy and occupational therapy are important in
the process of rehabilitation.
40% eventually back to normal. 2% vegetative state with no awareness or ability to communicate
with the environment.

1. Lindsay KW, Bone I, Callander R. Neurology and neurosurgery illustrated, 4th ed. Churchill
Livingstone, Edinburgh, 2004.
2. National collaborating centre for acute care. Head injury: Triage, assessment, investigation and early
management of head injury in infants, children and adults. NICE, September 2007.

5. Motor Neurone Disease
Written by: Dominic Wu from North West Deanery,
Motor neuron disease (MND) is a disorder that involves rapidly progressive degeneration of the upper
and/or lower motor neurons (UMN, LMN).
The most common form of MND is amyotrophic lateral sclerosis (ALS), which is 10% familial and 90%
sporadic adult-onset.
The rarer subtypes of MND include progressive muscular atrophy (PMA) that affects LMNs and primary
lateral sclerosis (PLA) that prinicipally destroys UMNs.
Irrespective of these distinctions, experts in the field have seemed to favor the suggestion that they will
become ALS at some stage during the progression of disease.
The condition of MND is highly debilitating and it often carries a five-year mortality of 70% following the
onset of symptoms.
The worldwide prevalence of MND/ALS is frequently cited as 5-7 per 100,000 person-years. There are
several epidemiological studies reported the presence of geographical variation, Caucasian and male
predominance in the occurrence of disease.
However, these statistics may subject to change owing to the growing interest in research, the ageing
population and the implementation of the Awaji-Shima recommendations as a replacement to the
traditional Revised El Escorial criteria for diagnosing MND, which has been in use since 2006.
How sensitive and specific are the two diagnostic tools?
The Awaji-Shima consensus recommendations are a set of combined clinical and neurophysiological
tools that were shown to be 32.7% (60.7% versus 28%) more sensitive, but equally specific (95.9%), to
the traditional Revised El Escorial criteria in a retrospective study by Douglas et al in 2010. A more
recent systematic review and meta-analysis by Costa et al also echoed that the Awaji-shima are superior
to the Revised El Escorial criteria and suggested that the use of the former may allow earlier diagnosis
and patient entry into clinical trials in ALS.

Our current understanding of MND centres around the excitatory hypothesis of glutamate: a
neurotransmitter which has the potential to cause flooding of calcium ions in the post-synaptic motor
neurons; mitochondrial dysfunction and the formation of reactive oxygen species and proteases that are
responsible for apoptosis of the motor fibres.
It was estimated that 15% of familial MND sufferers have chromosomal defects which produce functional
superoxide dismutase (SOD), an antioxidant that counteracts neuronal destruction caused by free
oxygen radicals.
The aetiology of sporadic MND is largely unknown. There is plentiful literature that suggests prolonged
heavy cigarette smoking and exposure to organic solvents and heavy metals as modifiable risk factors
for MND. However, further research is required in order to prove these hypotheses.

Clinical features
Paralysis of the skeletal muscles is almost inevitable within five years of the diagnosis of MND/ALS.
Disabling factors such as wrist drop, foot drop, cramps and spasticities in the upper and/or lower
extremities cause major setback to patients' activities of daily living.
Bulbar symptoms, notably dysphagia, wasting of the tongue and reduced gag reflex are contributory to
malnutrition, multiple choking episodes and aspiration pneumonia.
The fatigue of respiratory muscles is also well described as a risk factor for nocturnal hypercapnia and
respiratory failure. The onset of acute ventilation insufficiency in patients inevitably requires
consideration of NIV or ventilation.
Differentiating UMN from LMN signs

MND does not affect motor neurons alone!
There is emerging evidence to suggest MND as a multi-system disorder that affects more than just the
motor components. Several neuropsychological studies have estimated that almost half of all MND
patients will demonstrate evidence of frontal lobe dysfunction as the disease progresses. Other than
cognitive and behavioral impairments, case reports in MND have also reported elements of salivary
dysfunction, and revealed evidence of reduced heart rate variation and denervation of the adrenergic
cardiac fibres which are consistent with deregulation of the autonomic nervous system.
The denervation of Onufs nucleus (neurons located at the ventral horn of spinal cord at sacral region
that are responsible for micturition and defaecation) that were evident on post-mortem studies would
also suggest the likely involvements of bowel and bladder sphincters in MND.
The concurrent features of MND also include sensory disturbances, opthalmoplegia and parkinsonism.
However, because of their relative rare presentation, it shall be of good medical practice to consider
other aetiologies; and to perform the appropriate neuroimaging and electrophysiological studies before
establishing the final diagnosis.
Differential Diagnosis
The differential diagnosis for MND is huge. The conditions that are not to be missed:
Multi-focal motor neuropathy
Brainstem lesions from stroke or syrinx
Multi-level degenerative changes/ diseases of the cervical spine
Polymyositis, inclusion body myositis (IBM)
Benign fasciculation syndrome

The diagnosis of MND is largely clinical. So far there is no single laboratory marker that can confirm
MND. The use of electrophysiological studies (EMG/NCS) have been heavily weighed in the Awaji-shima
consensus recommendations to increase the "true positives" for diagnosing ALS.
Lumbar punctures and neuroimaging techniques such as CT and MRI, are useful to exclude conditions
that may micmic ALS but they are generally of limited value in the workup for MND.
The additional tests that may be required include:
FBC/ESR/U&E/Vitamin B12 & folate levels
TFT (?Thyrotoxicosis)
Anti-GM1 antibodies (?MMN)
CK +/- muscle biopsy (?Myositis)
Anti-acetylcholine receptor antibodies, anti-MUSK antibodies (?Myasthenia Gravis)
Androgen receptor gene mutation (?Kennedy's disease)
Anti-neuronal antibodies +/- CT chest/abdomen/pelvis (?Paraneoplastic)
Toxicology (?Heavy metal poisoning)
Syphillis serology (?Neurosyphillis)
The care of MND patients should consist of experienced neurologists, general practitioners, specialist
and community nurses and allied health care professionas such as physiotherapists and occupational
therapists, speech and language therapists and dieticians. As patients are approaching their end-of-life,
the options of palliative care should also be raised.
The following aims to review the three key aspects surrounding the care of patients with MND:
Symptomatic relief
Optimization of function
Disease modifying therapy
Symptomatic relief
Spasticity is common in MND. The hyper-excited alpha motor neurons cause tightness and stiffness
of the body extremities; and hence problems including joint contractures, poor coordination and difficulty
in toileting. The fatigue or irritability of muscles may also cause cramps which can become very painful.
Exercise such as stretching; strengthening and endurance may preserve soft tissue extensibility;
improve myofibre remodeling, CNS adaptation and reduce inflammation of neurons in MND. With regard
to therapeutics, the three most common skeletal muscle relaxants that we used in clinical practice are
baclofen, tizanidine and benzodiazepine. Baclofen is a GABA agonist that is thought to improve muscle
contraction, flexor spasms and disability. The agent can either be taken orally or be injected intrathecally
for intractable case. Its oral preparation is commonly served as a comparator to benzodiazepine, which
is also GABA agonistic; and tizanidine, a centrally-acting alpha 2 agonist in clinical trials. Clinically, they
are all of similar efficacy but the side effects of drowsiness and worsening muscle weakeness are
generally less frequent in patients with tizanidine.
Optimization of function
Respiratory function is a strong indicator of function and survival. The diagnosis of MND should prompt
early referral for assessment of patients peripheral oxygen saturation (SpO2), forced vital capacity
(FVC), vital capacity (VC); and measurements of their sniff nasal inspiratory pressure (SNIP) or maximal
inspiratory pressure (MIP) by the respiratory specialists. As according to NICE, patients should be
enrolled into a 3 monthly review as surveilence for further deterioration.

Non-invasive ventilation (NIV)
Patients with irreversible deterioration of respiratory function, for instance, a drop in:
1. FVC below 50% predicted or less than 80% with signs of respiratory compromise*; or
2. SNIP/MIP of 40cmH2O or below 60 cmH2O with evidence of respiratory compromise*
should always warrant discussion about non-invasive ventilation (NIV) and other treatment options with
patients and their careers. (* The signs of respiratory compromise include dyspnoea at rest,
hypercapnia, nocturnal hypoventilation and orthopnea)
The landmark randomized controlled trial (RCT) in NIV came from a single-centre in Newcastle, UK. In
this study, 41 ALS patients with symptomatic hypercapnia or orthopnea with MIP below 80% predicted
were randomized to receive either NIV (n = 22) with bi-level positive airway pressure (BIPAP; at mean
inspiratory and expiratory pressures of 15cmH2O and 4cmH2O respectively) or standard care (n = 19).
With clear study protocol and plans for follow-up, the study demonstrated significantly longer survival
and more favorable QOL in patients who received NIV than those without by a median of 205 days (p =
0.0059). The lack of survival and functional benefits in patients who suffered poor bulbar function
suggests that bulbar impairment is a poor prognostic indicator in MND.
Additionally, NIV for at least four consecutive hours a day has been shown to delay the time for
tracheostomy and mechanical ventilation (TV, MV). There has also been a suggestion that NIV could
slow down the deterioration of FVC (3.5% and 8.3% reduction per month in NIV users and non-users
respectively). However, as FVC is poorly related to other pulmonary function studies and symptoms of
early respiratory deficiency, its correlation with the presumed benefits of NIV remains uncertain.
Tracheostomy ventilation (TV)
Tracheostomy generally serves two purposes in MND. It is either used to facilitate rehabilitation of
patients who demonstrate slow weaning from invasive ventilation following critical illness or is done
electively for those who progressively deteriorate or suffer bulbar and cognitive dysfunctions that impair
their compliance on NIV. In comparison to NIV, TV is more complex, costly and involves a high level of
The uptake of tracheostomy is hugely dependent on patient autonomy; healthcare funding and the
geographical area that they reside. There are some low quality studies that suggest TV could extend life
for longer than patients on NIV. Despite the higher survival rate, the loss of ability to communicate in
patients that would inevitably occur around five year of the diagnosis; the risks of stoma infection,
recurrent pneumonia and tracheo-oesophageal fistula have frequently sparked off debates on QOL and
whether TV is worth initiating in the first place.

Enteral nutrition (EN)
Enteral feeding via a Percutaneous Endoscopic Gastrostomy (PEG) or Radiologically Inserted
Gastrostomy (RIG) is often offered to patients with dysphagia.
The uptake of EN is reported to be lowest in Italy (11-24%) and highest in Japan (21-60%). Although
there is no consensus as to when enteral feeding should be initiated in MND; recurrent choking and
gagging episodes, evidence of weight loss more than 10% of normal or evidence of clinical anorexia
(BMI < 18kg/m^2) are generally triggers for EN.
A FVC of 50% is often used as the reference value for safe gastrostomy. A FVC of more than 50%
predicted is generally considered safe for the insertion of PEG. RIG requires less sedation and it is
placed at FVC of 50% predicted or below. The lowest safety margin for RIG is yet determined by clinical
Despite extensive publications in the field, the benefits in survival and nutrition outcomes of patients with
EN were only concluded as tentatively favorable by the most recent systematic review of 10 non-
randomized trials in 2011. The study with the best methodological quality was a retrospective 26-centre
study of 221 patients. Concerning survival statistics, the study demonstrated higher hazard ratios in
those who received no PEG feeding; and with more rapid decline of their bulbar and respiratory function.
The results of this study are also comparable with similar trials published in later years.
Riluzole is the sole disease-modifying agent in MND. As a glutamate release antagonist, riluzole is
proposed to reduce excitotoxicity through glutamatergic transmission in CNS. The most recent
systematic review has shown that riluzole, if taken 100mg daily, prolong ssurvival for a median of two-to-
three months but had negligible effect on muscle strength, bulbar and limb functions in patients who fall
under the definite or clinical probable Revised El Escorial classification.
The pooled analysis of trials showed that Riluzole was generally tolerable in patients. Some rare side
effects can occur, for example raised alanine transference (ALT) above five times of its upper limit in 4%
of patients. The other rare abnormalities include anaemia, neutropenia, acute lung injury,
hypersensitivity and pancreatitis.
MND is a rare but rapidly progressive degenerative disorder that has the potential to involve UMN, LMN
and the rest of the nervous system. MND has no cure and no effective life-prolonging treatment. The
mainstay of management should therefore focus on symptomatic relief, optimization of function and the
preservation of patients' QOL.
References and Suggested reading
Sathasivam S. Motor neurone disease: clinical features, diagnosis, diagnostic pitfalls and prognostic
markers. Singapore Med J. 2010 May;51(5):367-72 (
Simmons Z. Management strategies for patients with amyotrophic lateral sclerosis from diagnosis
through death (
Rafiq MK, Proctor AR, McDermott CJ, Shaw PJ. Respiratory management of motor neurone disease: a
review of current practice and new developments. Practical Neurology. 2012 June 1, 2012;12(3):166-76.

6. Other Movement Disorders
Written by: Ania Crawshaw from Manchester University,

This article covers types of dyskinesia and their causes, with later focus on:
Drug-induced dyskinesias
Huntington's disease
Multi-system atrophy
Progressive supranuclear palsy
Types of dyskinesia
Dyskinesias are defined as abnormal involuntary movements. They include:
1. Athetosis
2. Chorea
3. Hemiballismus
4. Dystonias
5. Myoclonus
6. Tardive dyskinesia (see drug-induced dyskinesias)
7. Tics
8. Tremor (this is covered in another article)
Athetosis and chorea
Athetosis is characterised by slow, writhing movements, most commonly seen in the fingers and hands.
It should be differentiated from pseudoathetosis, the 'piano-playing' movement of fingers, present in
patients with deficits in proprioception. This phenomenon is elicited by asking the patient to hold their
hands out in front of them with closed eyes.

Chorea is characterised by rapid, jerky, 'dancelike' movements, most commonly in the limbs and head.
Movements may appear semi-purposeful, and are not repetitive, but move fluidly to different parts of the

These two dyskinesias often occur together as choreoathetosis. Causes include:
IATROGENIC - medications for Parkinson's disease, epilepsy and schizophrenia
GENETIC - Huntington's disease (discussed further down)
DEVELOPMENTAL - cerebral palsy
AUTOIMMUNE - as a now rare complication of acute rheumatic fever, Sydenham's chorea, or as a
symptom of SLE
HORMONAL - as an isolated symptom in pregnancy, or rarely as a side effect of theoral
ontraceptive pill, hyperthyroidism
METABOLIC - sodium or glucose imbalance and vitamin B1/B12 deficiencies
VASCULAR - stroke

Hemiballismus affects one side of the body, most commonly the arm. It is a sudden flinging movement
that can often be problematic as objects may be unintentionally thrown, or the arm might hit other
people/objects in the vicinity. Most commonly caused by subthalamic stroke.
Dystonias are spasmodic movements that may occur during intentional movement, either rhythmically
(giving a repetitive appearance), or persistently (resulting in typical fixed postures). Focal dystonias may
be responsive to treatment with botox. They may be :
In the neck such movements are calledspasmodic torticollis
In the back they may result in a twistingscoliosis or an arched lordosis
In the eye, blepharospasm results in involuntary eye closure
Oromandibular dystonia affects the mouth, jaw, larynx and pharynx
Writer's cramp may appear in the hand
These focal dystonias are usually acquired. In contrast, generalized dystonias are
normallyhereditary. Some can be treated with carbidopa.

Rapid, sometimes repetitive, jerks of a muscle or group of muscles. Hiccoughs are a benign example,
and people often experience myoclonus as they are falling asleep. Pathological manifestations include:
IATROGENIC - antihistamines, antidepressants, levodopa
METABOLIC - kidney or liver failure, hypo/hyperglycaemia
HYPOXIA - Lance-Adams syndrome
INFECTION - viral encephalopathy
VASCULAR - stroke
NEUROLOGICAL DISEASES - epilepsy, MS, Parkinson's, Alzheimer's or CJD
IDIOPATHIC - essential myoclonus
Tics are rapid, repetitive, stereotyped movements. Patients may describe feeling an urge to do the tic,
similar to an urge to sneeze, that can sometimes be supressed for short periods of time. However the
urge usually becomes irresistable after several minutes, and the patient may feel momentary relief
during and after performing the tic.

Simple tics include excessive blinking, grimacing, A quarter of children develop tics at one point in their
development that usually resolve within a year.

Complex tics may be caused by Gilles de la Tourette syndrome. This may also include vocal tics,
such as involuntary swearing.

Drug-induced dyskinesias
Neuroleptics (antipsychotics) can induce tardive dyskinesia. This is characterised by movements of
the mouth such as chewing, lip-smacking and protrusion of the tongue. Extrapyramidal movements are
also common side effects. These include rigidity, pill-rolling tremor, and bradykinesia (slowing of
movement). Extrapyramidal movements induced by medication tend to besymmetrical, in contrast with
Parkinson's disease where onset of tremor and rigidity is usually asymmetrical.

Parkinson's medications such as levodopa can induce choreatic and athetoid movements. They may
also induce myoclonus, as can antihistamines and tricyclic antidepressants.

Huntington's disease
This is an autosomal dominant, neurodegenerative disease affecting the basal ganglia. Onset
isinsidious, and usually between the ages of 35 and 50.

Signs and symptoms
The early signs and symptoms, which may persist for 10 years before onset of chorea, include:
Personality change
Mild psychosis
Fidgeting and fleeting grimaces

Core features are:
Dysarthria, dysphagia, myoclonus and tics are also common. Later in the disease chorea may be
superseded by dystonia and parkinsonism.

No known treatments are effective in slowing or stopping disease progress. Instead treatment is
1. Chorea may respond to benzodiazepines and neuroleptics
2. Psychosis should be treated with neuroleptics
3. Depression should be treated with antidepressants, or in refractory cases ECT
Genetic counselling is strongly recommended, as is the early arrangement of an advance directive.
Most patients die within 13-15 years of disease onset.

Though Parkinson's disease is the main form of parkinsonism, some other rarer diseases can also
manifest with some or all of the triad of rigidity, tremor and bradykinesia:

Multiple system atrophy (MSA)
Extrapyramidal symptoms in this disease is mostly unresponsive to treatment. The tremor is
generally not 'pill-rolling' as in parkinson's disease, and postural instability is often a core feature.
Aside from extrapyramidal symptoms, this is characterised by changes in:
1. THE AUTONOMIC SYSTEM. When this feature predominates, MSA is sometimes called Shy-Drager
Postural hypotension (resulting in dizziness and syncope)
Urinary dysfunction
Ataxic gait
Ataxic dysarthria

Progressive supranuclear palsy (PSP)
This is characterised by:
Symmetric rigidity (greater proximally than distally) without cogwheeling
Neck hyperextension (dystonia)
Vertical gaze palsy
Postural instability
Dysarthria and dysphagia (pseudobulbar palsy)
Cognitive impairment
It is unresponsive to therapy, and as suggested by the name, characterisd by progressive

7. Parkinson's Disease
Written by: Ali Mohsen from Manchester University,
Parkinsons disease (PD), also known as primary or idiopathic parkinsonism, is a neurodegenerative
condition of unknown aetiology. It is the chief cause of parkinsonism, a clinical syndrome characterised
by extrapyramidal symptoms such as a resting tremor, muscular rigidity and bradykinesia. Parkinsonism
is primarily a motor disorder; however non-motor symptoms, such as dementia and depression, can also

PD is diagnosed in 11-19 patients per 100,000 every year in Europe (1). It is estimated that 1-2% of
people over the age of 65 years suffer from the condition (2). The causes of PD remain unknown;
however it is associated with older age and non-smokers (3). PD is rare in adults under the age of 50. It
is more common in white Caucasians than Africans and Asians. Genetic mutations are reported to be
responsible for about 10% of PD (4).
Famous people with Parkinsonism

Parkinsonian syndromes result from the depletion of the neurotransmitter dopamine in certain brain
areas including the basal ganglia. The basal ganglia are a group of subcortical grey matter aggregates
that have an important role in facilitating movements and posture. In PD, degeneration of dopaminergic
neurons in the nigrostriatal pathways of the basal ganglia leads to dopamine depletion and a chemical
imbalance with the neurotransmitter acetylcholine leading to the development of the motor signs and
symptoms. Abnormal intracellular inclusions called Lewy-bodies may also accumulate in neurons. It is
believed that these bodies are responsible for the development of dementia during the course of the
Primary parkinsonism
Idiopathic (90%)
Familial (10%)

Secondary parkinsonism
Drug-induced (E.g. dopamine antagonists such as metoclopramide, neuroleptics)
Intoxication (MPTP, carbon monoxide, lithium)
Infections (CJD, subacute sclerosing panencephalitis)
Post-infectious (Encephalitis lethargica)
Neoplastic and paraneoplastic syndromes
Normal pressure hydrocephalus
Metabolic (Hypoxia, Wilson's disease)

'Parkinson's plus' disorders
Multiple system atrophy
Progressive supranuclear palsy
Clinical presentation
Parkinsonian (extrapyramidal) signs and symptoms can be remembered using the mnemonic TRAP,
where patients are trapped in their poorly mobile body. Symptoms typically start unilaterally before
involving the contralateral part.

A unilateral resting tremor (3-5Hz oscillation frequency) is the most common initial presentation of
PD and may be seen in the hands or feet. In the hands it has a characteristic 'pill-rolling
appearance'. In later stages of the disease it often becomes bilateral, though may remain
Rigidity describes the increased muscle tone (hypertonia) causing stiffness of limbs, neck and trunk.
Rigidity can take 2 forms in PD;
1. Lead-pipe rigidity describes the continuous increased muscle resistance felt on passive movements of
2. Cogwheel rigidity describes the ratchet-like fluctuation of muscle resistance felt on passive
movements of joints
Rigidity is different to spasticity, which is clasp-knife like hypertonia of muscles seen in upper motor
neuron lesions. The clasp-knife phenomenon describes the rapid drop in resistance of a joint to
passive movement after an initially high resistance (as if opening a clasp-knife) and is characteristic
of pyramidal, not extrapyramidal deficit.
Rigidity of laryngeal muscles is thought to be responsible for the reduced volume of speech in
patients with PD (hypophonia)

Akinesia and bradykinesia
Akinesia describes the difficulty in the initiation of movement in PD, where patients show
considerable delay when starting to walk or talk
Bradykinesia describe the reduced range and speed of movements. It is responsible for the following
1. Reduced facial expressions (hypomimia) producing the classical masked faces
2. Drooling, which occurs due to bradykinesia and rigidity affecting throat muscles rather than
3. Problems in speech articulation (hypokinetic dysarthria)
4. Small and spidery handwriting (micrographia)
5. Gait disturbance including more frequent, shorter and shuffling steps (festinating gait), reduced arm
swing, and slow-turning
Postural instability
Patients with PD often develop a flexed (stooped) posture
Falls are common
Typical signs of parkinsonism

The diagnosis of PD is difficult due to the numerous causes of parkinsonism. It is diagnosed clinically as
there is no biomarker or clinical test available to confirm the diagnosis. NICE recommends using the
diagnostic criteria developed by the UK Parkinsons Disease Society Brain Bank Criteria (See Steps
below). No imaging technology can give a definitive diagnosis, however NICE recommend the use of
magnetic resonance imaging (MRI) for investigating the cause of parkinsonism, and single photon
emission computed tomography (SPECT) to distinguish essential from parkinsonian tremor. A definite
diagnosis of PD can only be made after brain autopsy.
STEP 1: Diagnosis of Parkinsonism
Bradykinesia and at least one of the following:
Muscular rigidity
46 Hz resting tremor
Postural instability not caused by primary visual, vestibular, cerebellar or Proprioceptive dysfunction

STEP 2: Features tending to exclude Parkinsons disease as the cause of Parkinsonism
History of repeated strokes with stepwise progression of parkinsonian features
History of repeated head injury
History of definite encephalitis
Neuroleptic treatment at onset of symptoms
>1 affected relatives
Sustained remission
Strictly unilateral features after 3 years
Supranuclear gaze palsy
Cerebellar signs
Early severe autonomic involvement
Early severe dementia with disturbances of memory, language and praxis
Babinski's sign
Presence of a cerebral tumor or communicating hydrocephalus on CT scan
Negative response to large doses of levodopa (if malabsorption excluded)
MPTP exposure

STEP 3: Features that support a diagnosis of Parkinsons disease (three or more required for
diagnosis of definite Parkinsons disease)
Unilateral onset
Tremor at rest present
Progressive disorder
Persistent asymmetry affecting the side of onset most
Excellent (70100%) response to levodopa
Severe levodopa-induced chorea
Levodopa response for 5 years
Clinical course of 10 years
(Adapted from
Differential diagnosis
Essential tremor
Classically, essential tremor is a postural tremor (in contrast with the resting tremor of parkinsonism). It
often improves on alcohol consumption and responds well to the -blocker propanolol. However
occasionally it is difficult to differentiate from a parkinsonian tremor, which is when SPECT scan can be

Drug-induced parkinsonism
A complete drug history should always be obtained from patients, asking specifically about
antipsychotics and metoclopramide. The parkinsonian symptoms induced by medications are more
symmetrical than those of primary parkinsonism.

Wilsons disease
Wilson's disease should be especially considered in young patients, and investigated by examining for
kayser-fleischer rings, checking ceruloplasmin levels, and performing a liver biopsy.

Multiple-system atrophy (MSA)
Characterized by cerebellar disturbance, preservation of cognitive function, poor response to L-dopa,
and severe autonomic disturbance such as postural hypotension, urinary and rectal incontinence and
impotence (5).

Progressive supranuclear palsy (PSP)
Earlier onset of falls, symmetrical bradykinesia, failure of downward saccadic vision, severe dysarthria,
severe swallowing difficulties, and lack of response to L-dopa are features that favor the diagnosis of
PSP rather than PD (6).

Lewy-body dementia
Lewy-body dementia is characterized by fluctuating cognitive function, visual hallucinations,
parkinsonism, and severe sensitivity to antipsychotic medications

No cure is currently available for PD. Pharmacological therapy improves symptoms by correcting the
neurochemical imbalance in the basal ganglia. Several therapy strategies are available; the choice of
drugs should be based on the patients condition and preferences.

First Line treatment for motor symptoms
Levodopa (L-dopa)
L-dopa is a precursor of dopamine. It is a commonly used drug in both early and late PD to replenish
dopamine levels in the basal ganglia. Short term side effects of L-dopa include GI upset (treated with
domperidone), agitation, insomnia, impulsive behavior, and poor BP control. L-dopa is commonly
combined with carbidopa (Sinemet) or benserazide (Madopar, Prolopa). These are drugs that inhibit L-
dopa conversion to dopamine outside the central nervous system by the enzyme dopa-decarboxylase,
reducing peripheral side effects.
Long term pharmacological therapy with L-dopa is limited in several ways. Firstly, with time, symptoms
become less responsive to the medications. Secondly, high concentrations of dopamine can lead to
abnormal involuntary movements (dyskinesias) such as chorea and tics. Due to a narrowing of the
therapeutic index with time, fluctuations in dopamine concentration produce on-off phenomena, where
high dopamine levels lead to dyskinesia (on), while low dopamine levels lead to bradykinesia and rigidity
(off). This is also known as motor fluctuation. It is common practice to avoid early initiation of L-dopa
therapy in order to delay the onset of these common complications.
Dopamine agonists
Synthetic dopamine agonists such as ropinirole, pramiprexole, pergolide, and bromocriptine can be used
instead of or alongside L-dopa to manage motor symptoms of PD. However, adverse effects including
nausea, vomiting, dizziness, postural hypotension, insomnia, prepulmonary fibrosis (especially with
pergolide), hallucinations and delusions may limit the use of these drugs. Apomorphine is a
subcutaneously administered dopamine agonist that is used to manage motor fluctuations in late PD.
Monoamine oxidase type B (MAO-B) inhibitors
Rasagiline and selegiline are examples of MAO-B inhibitors, a group of medications that inhibit the
breakdown of dopamine, hence increasing its concentration. Their use has been validated for early PD.
Catechol-O-methyl transferase (COMT) inhibitors
Drugs such as entacapone and tolcapone inhibit the metabolism of L-dopa. They are used in late PD to
manage motor fluctuations by reducing L-dopa concentration variations. Combination preparations of L-
dopa, carbidopa, and entacapone (Stalevo) are available.

Other Medications
Amantidine use in PD is a result of an accidental discovery, and is a weak dopamine antagonist.
However, insufficient evidence is available regarding its efficacy and safety, therefore it is only used as
second line for motor symptoms in early PD.
Drugs such as trihexyphenidyl and benztropine restore neurochemical balance by antagonizing ACh. As
with amantidine, anticholinergics are only recommended as second line therapy for motor symptoms.
Their use is further limited by their side effects. These include dry eyes, dry mouth, drowsiness,
clumsiness, and psychiatric disturbance such as hallucinations, delusions, and confusion.
Depression is common in PD, and doctors should have a lower threshold for diagnosis mood disorders.
Typical antipsychotics are contraindicated in PD as they worsen motor symptoms, however clozapine
has been approved for use in PD. Agranulocytosis is a serious adverse effect of this drug, and so regular
blood count monitoring is essential.

Deep brain stimulation of the subthalamic nucleus or globus pallidus interna is used in patients with
severe disease refractory to pharmacological therapy.

Deep brain stimulation

(1) Von Campenhausen S, Bornschein B, Wick R, Botzel K, Sampaio C, Poewe W, Oertel W, Siebert U,
Berger K, Dodel R. Prevalence and incidence of Parkinson's disease in Europe. Eur
Neuropsychopharmacol. 2005;15(4):473490.
(2) Fahn S. Description of Parkinsons disease as a clinical syndrome. Ann N Y Acad Sci 2003;991:114
(3) Hernan MA, Zhang SM, Rueda-deCastro AM, Colditz GA, Speizer FE, Ascherio A. Cigarette smoking
and the incidence of Parkinsons disease in two prospective studies. Ann Neurol 2001; 50:78086.
(4) De Lau LM, Breteler MM. Epidemiology of Parkinsons disease. Lancet Neurol 2006; 5: 52535.
(5) Rehman HU. Multiple system atrophy. Postgrad Med J 2001;77:379-382
(6) Rehman HU. Progressive supranuclear palsy. Postgrad Med 2000;76:3336

8. Stroke
Written by: Cheryl Coulter from Newcastle University,
Every year, over 150,000 people in the UK suffer a Stroke or Transient Ischaemic Attack (TIA).
Stroke is the third most common cause of death in developed countries.
Uncommon below the age of 40.
More common in males.
There are over one million stroke survivors in the UK; around half of those are left dependent on
others for help with everyday activites.
"Clinical syndrome of rapid onset of cerebral deficit (usually focal), lasting more than 24 hours or leading
to death, with no apparent cause other than a vascular one." (Kumar and Clark, 2008)

Minor Stroke:
As above for Stroke but additionally "Patients recover without significant deficit, usually within a week."
(Kumar and Clark,2008)

Transient Ischaemic Attack (TIA):
"A focal deficit, such as weak limb, aphasia, loss of vision lasting from a few seconds to 24 hours, there
is COMPLETE recovery." (Kumar and Clark, 2008)
Risk Factors
Diabetes Mellitus
Heart Disease (Valvular / Ischaemic / Atrial Fibrillation)
Peripheral Vascular Disease
Past TIA
Carotid Bruit
The Oral Contraceptive Pill
Raised Clotting
Increased Age
Afro-Caribbean Origin
Pathophysiology and Clinical Features
A stroke is caused either by ischaemia and infarction of the brain or an intracranial haemorrhage,
both resulting in damage to the brain tissue, causing altered functioning. The clinical presentation
therefore is very variable, as it depends on the site affected and to what extent.

Intrcranial haemorrhage
Intracranial haemorrhage, is a bleed within the cranial cavity, and can be subdivided into:
Intracerebral Haemorrhage
Cerebellar Haemorrhage
Subarachnoid Haemorrhage
Subdural Haemorrhage
Extradural Haemorrhage

1. Intracerebral Haemorrhage
Haemorrhage within the cerebral hemispheres accounts for around 10% of strokes. The underlying
cause of the haemorrhage is mainly due to rupture of micro aneurysms, and degeneration of small
penetrating arteries. Usually these haemorrhages are massive and often fatal. These strokes tend to be
more dramatic in presentation and with an associated severe headache, than those of ischaemic origin.

2. Cerebellar Haemorrhage
Haemorrhages within the cerebellum usually have the same underlying cause as intracerebral
haemorrhages. Patients can present with headache, and can have rapid detoriation in their GCS. Due to
the close proximity with the brainstem, they can also present with brainstem signs such as nystagmus
and ocular palsies, and their gaze may deviate TOWARDS the haemorrhage.

3. Subarachnoid Haemorrhage
Subarachnoid haemorrhages (SAH), account for 5% of strokes, with an annual incidence of 6 per 100
000. SAH is when spontaneous arterial bleeding occurs in the subarachnoid space. Usually it has a
dramatic onset, with a sudden very severe (thunderclap) headache, usually in the occipital region,
followed by vomiting and often by coma.
The most common underlying cause of SAH include Saccular (Berry) aneurysms (accounting for around
70% of SAH), and Ateriovenous malformations (accounting for around 10% of SAH). Rarer causes
include bleeding disorders, acute bacterial meningitis, brain tumours, arteritis and polycystic kidneys.
However, it is unusual to find any contributing disease.

4. Subdural Haemorrhage
Subdural haemorrhage occurs when there is an accumulation of blood within the subdural space
following rupture of a vein. Often it follows a head injury, which maybe trivial, and presentation may not
be for days, weeks or even months after the injury. The elderly and alcoholics are more likely to present
with chronic, unsuspected or spontaneous haemorrhages.
Clinical presentation is headache, drowsiness and confusion, symptoms of which often fluctuate.
Additionally focal deficits such as hemiparesis or sensory loss may develop. Chronic subdural
haemorrhage should always be considered in an elderly patient with new or worsening confusion.

5. Extradural Haemorrhage
Extradural haemorrhage is when a branch of the middle meningeal artery tears. This occurs through a
linear skull fracture and leads to the accumulation of blood in the extradural space. Often a patient will
suffer a head trauma, followed by a brief period of unconsciousness followed by a luccid interval of
recovery. Next the patient can go on to develop progressive hemiparesis and stupor. Additionally
transtentorial coning may occur, leading causing an ipsilateral dilated pupil, which may progress to
bilateral fixed dilated pupils, tetraplegia and respiratory arrest.

Ischaemia and Infarction
Brain infarcts, in both cerebral hemispheres and brainstem, are caused by local interruption of blood flow
to the brain and are the commonest cause of strokes, accounting for 70% of all strokes. Cerebral
atherosclerosis is the most common cause of brain infarcts, with the most severe atherosclerotic lesions
typically encountered within the larger vessels such as the internal carotid arteries, proximal middle
cerebral arteries and basilar arteries. Additionally patients with atherosclerosis can have an occlusion
due to a thrombosis of an atherosclerotic arterial segment, occuring most commonly near the carotid
bifrucation or basilar artery.
Finally other causes of occulsion include emboli, mainly originating from the heart (as in chronic AF) or
from atherosclerotic plaques. Vasculitis and trauma are less common causes of occlusion. Assessment
of individuals presenting with embolic stroke should involve searching for their source (ECG, echo,
carotid dopplers)
Clinical presentation will vary depending on site and extent of infarction, but generally present as:
Sudden onset or stepwise progression over hours, rarely days.

1. Cerebral Infarction (50% of Infarcts - most common)
Contralateral Hemiplegia (initially flaccid then become spastic)
Contralateral Sensory Loss
Homonymous Hemianopia
Visuo-Spatial Deficit (depending on site)

2. Brainstem Infarction (25% of Infarcts)
Causes complex signs depending on the relationship of the infarct to the cranial nerve nuclei, long tracts
and brainstem connections.
Disturbances of Gaze and Vision
Locked in Syndrome

3. Lacunar Infarction (25% of Infarcts)
Small infarcts around basal ganglia, internal capsule, thalamus and pons.
Pure Motor Signs
Pure Sensory Signs
Mixed Motor and Sensory Signs
Sudden Dysarthria with a clumsy hand

Example of CT Scan showing Cerebral Haemorrhage [6]

Example of CT Scan showing Infarction [7]

Classification of Strokes - Bamford Classification
The Bamford classification system, is a simple bedside method of classifying acute ischaemic strokes. It
uses the patients' symptoms to classify which region of the brain has been affected, and once classified
it allows for prediction of a patient's prognosis.

Total Anterior Circulation Stroke (TACS)
Higher Cerebral Dysfunction & Homonymous Visual Field Defect & Ipsilateral Motor +/- Sensory Deficit
Mortality: 39% at 1 month, 60% at 1 year, Recurrence: Low Risk

Partial Anterior circulation stroke (PACS)
2 out of 3 of TACS Symptoms OR Higher Cerebral Dysfunction Alone OR Monoparesis
Mortality: 4% at 1 month, 16% at 1 year, Recurrence: Very High Risk

Lacunar stroke (LACS)
Motor Stroke OR Sensory Stroke OR Sensori-motor Stroke OR Ataxic Hemiparesis
Mortality: 2% at 1 month, 11% at 1 year, Reccurrence: Low Risk

Posterior circulation stroke (POCS)
Ipsilateral Cranial Nerve Palsy with Contralateral Motor Deficit OR Bilateral Deficit OR Disorder of
Conjugate Eye Movement OR Cerebellar Dysfunction OR Isolated Homonymous Hemianopia
Mortality: 7% at 1 month, 19% at 1 year, Recurrence: High Risk
The purpose of investigations is to confirm the clinical diagnosis and to distinguish between strokes
caused by infarction or haemorrage. Additionally, it is important to identify if there is an underlying cause
of the stroke and to use the investigations to assist in directing the managment of the patient.
Initial Investigations:
U & Es
CT Head
Carotid Doppler


As previously discussed, when an ischaemic stroke occurs there is an interuption of the blood supply to
part of the brain, this has affects in two ways.
Firstly to the brain tissue in the immediate area of the ischaemia, otherwise known as the 'Umbra'. The
lack of oxygen causes the brain tissue to cease functioning. If this is prolonged and hasn't been restored
after approximately 3 hours, the tissue will suffer irreversible injury, possibly leading to death of the
Secondly, the tissue area surrounding the ischaemic event can also be affected, it is tissue which is
potentially destined for infarction but not yet irreversibly injured, this is known as the 'Penumbra'. Since
the blood supply to the adajacent brain tisue is now restricted, the lack of oxygen delivery into metabolic
pathways is affected. So, to improve this situation, the brain resorts to using anaerobic respiration in
these areas. This form of respiration produces the by product lactic acid, which is an irritant and alters
the acid-base balance of the brain.
Additionally, as oxygen and glucose depletion in these areas continue, the production of ATP fails. The
knock on effect is failure of energy-dependent processes (i.e. ion pumps) necessary for tissue survival,
setting off a cascade of events resulting in cellular injury and death. This cascade is known as the
'Ischaemic Cascade' and occurs in other tissues, but the brain differs to other tissue by having little
respiratory reserve and being far more dependent on aerobic respiration.
So, to protect the 'Penumbra' region of the brain, it is vital to restore blood flow as quickly as possible.
This is where thrombolysis can play a key role.
Thrombolysis, is the break down of a thrombus by pharmacological means. The pharmacological agent
most commonly used today is 'Alteplase'. The pathway is described in the diagram below.
Age 18 years or over
Clinical Diagnosis of Acute Ischaemic Stroke
Assessed by Experience Team
Measurable Neurological Deficit
Timing of Symptom onset well established
CT or MRI and Blood Test Available and consistent with diagnosis
Treatment could begin within 180 mintues of symptom onset

Exclusion Criteria
Symptoms minor or improving
Haemorrhage on pretreatment scan
Suspected Subarachnoid Haemorrhage
Active Bleeding from any site
GI or Urinary Tract Haemorrhage in last 21 days
Platelet Count <100 x 10(9)/litre
Recent Treatment with Heparin and activated Partial Thromboplastin time above normal
Recent treatment with warfarin and INR elevated
Major Surgery or Trauma in last 14 days
Recent post MI pericarditis
Neurosurgery, serious head trauma or stroke in last 3 months
History of Intracranial Haemorrhage
Known Arteriovenous Malformation or Aneurysm
Recent Arterial Puncture at Non- Compressible Site
Recent Lumbar Puncture
Blood Pressure consistently >185 systolic or >110 diastolic
Abnormal blood glucose
Suspected or known Pregnancy
Active Pancreatitis
Epileptic Seizure at Stroke Onset
(Provided by Kumar and Clarke, 2002 - Pg 1214)

1. Kumar P, Clark M. Clinical Medicine , 6th Edition. Elsevier Saunders, 2008. Pg 1209 - 1219.
2. Kumar V, Cotran RS, Robbins SL. Robbins Basic Pathology, 7th Edition. Elsevier, India, 2004. Pg 814
- 815.
3. Longmore M, Wilkinson I, Turmezei T, Cheung CK. Oxford Handbook of Clinical Medicine, 7th Edition.
Oxford University Press, Oxford, 2008. Pg 462.
4. NHS Direct Website [homepage on Internet]. Stroke Section. Available
from: Accessed on 23rd January 2011.
5. The Stroke Association [homepage on Internet]. The Stroke Association; Information; Our Publications
Section; Leaflets; What is a stroke. Available
from: Accessed on 23rd
January 2011.
6. A. Taranath & C. Kah Fook Wong : Pulmonary And Central Nervous System Involvement In Juvenile
Dermatomyositis: Vasculopathy And/Or Steroids As Causative Agents - A Case Report . The Internet
Journal of Radiology. 2005 Volume 4 Number 1. Available
from: Accessed on 01
June 2011.
7. The Internet Journal of Neurology [homepage on
e/my_husband_s_eye_looks_funny_doctor.html. Accessed on 01 June 2011.


9.Subarachnoid Haemorrhage
Written by: Milan Samarage from Imperial,
Bleeding into the physiological subarachnoid space between the pia mater overlying the brain and the
subarachnoid membrane is known as subarachnoid haemorrhage (SAH) and is considered a form of
stroke. SAH is a medical emergency with an average case fatality rate of 51% and a mortality rate of 10-
15% in those not reaching a hospital. SAH is treated by prompt neurosurgical and radiological

SAH makes up 3% of all strokes and accounts for 5% of all stroke mortalities.
Incidence: 10.5 per 100,000 / year (range 6-21)
Peak age of presentation in 50s to 60s.
More common in women, blacks, Japanese and Finnish peoples.

Risk factors
Common risk factors for SAH are as follows:

-First degree relative with SAH
-Combined oral contraceptive pill usage

-Substance abuse;
Cocaine abuse
Alcohol consumption

-Medical conditions associated with SAH;
Autosomal dominant polycystic kidney disease (ADPKD)
Fibromuscular dysplasia (FMD)

-Connective tissue disorders
Ehlers-Danlos syndrome type IV
Marfans syndrome
Pseudoxanthoma elasticum
1. Trauma
2. Spontaneous

Ruptured intracranial aneurysm
Cerebral AV malformations
Cerebral vasculitides
Cerebral artery dissection

Clinical features
Thunderclap headache (Classically described as the worst ever headache or kicked in the back of the
Meningismus (neck pain)
Syncope / Coma
Sudden death
Focal cranial nerve deficits (from aneurismal compression)
Low back pain (irritation of lumbar nerve roots following pooling of arachnoid blood)

Meningismus (Kernigs sign, Brudzinskis sign)
Ocular haemorrhage (caused by defective ocular venous drainage secondary to ICP);
Subhyaloid haemorrhage
Intra-retinal haemorrhage
Terson's syndrome (Blood within the vitreous humour)
Generally the following sequence is employed in the clinical evaluation of patients with suspected SAH;
1. Preliminary tests to diagnose SAH (non-contrast CT scan)
2. Lumbar puncture (LP) if CT is negative
3. Cerebral angiography in confirmed SAH or in high level of suspicion of SAH
4. Adjunctive tests if angiography is negative including repeat angiography, spinal angiography, CTA,
MRA and surgical exploration.
CT scan
Non-contrast CT scans with thin slices (<3mm) obtained within 12h of presentation have a sensitivity of
1. Hydrocephalus (enlarged ventricles)
2. Haematoma
3. Infarction (secondary to vasospasm)
4. Subarachnoid blood (present within the basal cisterns and fissures)
5. Aneurysms
Lumbar puncture (LP)
LP should be performed with a thin needle (22G) 12h or more after initial presentation.
1. Elevated opening pressure
2. Xanthochromic appearance
3. Presence of bilirubin
CT Angiography (CTA)

MR Angiography (MRA)

Cerebral Angiography / Digital Subtraction Angiography (DSA)
Gold standard investigation for aneurysms and vascular malformations.
Useful for demonstrating vasospasm radiographically.
Grading SAH
World federation of Neurologic Surgeons (WFNS) classification

Grade 0 Intact aneurysm
Grade 1 GCS 15
Grade 2 GCS 13-14
Grade 3 GCS 13-14 with major focal deficit
Grade 4 GCS 7-12 with/without major focal deficit
Grade 5 GCS 3-6 with/without major focal deficit
Hunt & Hess grading system

Grade 1 - Asymptomatic or mild headache, slight nuchal rigidity
Grade 2 - Moderate-severe headache, nuchal rigidity, cranial nerve deficit only
Grade 3 - Mild focal deficit, drowsiness, confusion
Grade 4 - Stupor, hemiparesis, early decerebration
Grade 5 - Coma, decerebrate rigidity, moribund

Patients with SAH are managed in monitored beds in Neuro ITUs.
Patients should be given bed rest, kept euvolaemic and administered oral nimodipine 60mg/4h.
Treatment is based on treating the underlying pathology causing SAH (e.g. surgical clipping / vascular
bypass or endovascular coiling/stenting of cerebral aneurysms, endovascular embolisation of
arterovenous malformations, etc.)

Immediate complications
Rebleeding (greatest risk within first 24h)
Vasospasm (greatest risk on days 7-10)
Acute hydrocephalus - due to blockage of the arachnoid granulations preventing reabsorption of
Delayed complications
Hyponatraemia (secondary to cerebral salt wasting and/or SIADH)
Cardiac problems (arrhythmias & ECG changes)
Hyperdynamic or Triple H therapy.
Aimed at controlling vasospasm medically. Different regional protocols are in place centred around
1. Hypertension
2. Hypervolaemia
3. Haemodilution
Calcium channel blockers (e.g. nimodipine)
1. Van Gijn J, Rinkel GJE: Subarachnoid haemorrhage - diagnosis, causes and management. Brain, 124
2. Samandouras G eds: The Neurosurgeon's Handbook OUP, 2010.
3. Greenberg MS eds: Handbook of Neurosurgery 6th edition, Thieme, 2006.

10. Extrapyramidal Signs
Written by: Ania Crawshaw from Manchester University,
Extrapyramidal signs and symptoms occur as a result of pathology in the basal ganglia. They should
be differentiated from pyramidal signs, which occur following an upper motor neurone lesion.
Some of the dyskinesias (abnormal movements) which occur following extrapyramidal pathology or
treatment with antiparkinsonian medications are discussed in more detail in the 'Other movement
disorders' article. More detailed descriptions of progressive supranuclear palsy (PSP) and Multiple
system atrophy (MSA), referred to in this article, can also be found there.
This article focusses on examination of a patient with suspected parkinsonism and some associated
signs and symptoms.

General inspection
Look for:
1. Walking aids
2. Hypomimia (reduced facial expression)
3. Stooped posture (in PD)
4. Resting tremor. This is often described as "pill-rolling" and is frequently exacerbated by high
emotions. InParkinson's disease, the tremor usually starts asymmetrically, commonly in thehands
and/or feet. Later in the disease, tremor may spread to all four limbs, and sometimes the face (eg. the
lips or chin). Patients with drug-induced parkinsonism are more likely to experience a symmetrical
5. Choreoathetosis. These abnormal movements are often caused byParkinson's medications. For a
more detailed description of this and other dyskinesias, see 'Other movement disorders'.

Focussed cranial nerve exam
Olfactory nerve
Sense of smell may be one of the first functional losses evident in Parkinson's disease.
Oculomotor, trochlear and abducent nerves
Examine eye movements for presence of a vertical gaze palsy. This is a common feature of the
parkinsonian syndrome Progressive Supranuclear Palsy (PSP). Patients may have difficulty looking
down voluntarily. However oculogyric downward gaze is preserved. This can be demonstrated if
you ask the patient to fix their gaze on a point straight ahead while you move their head back (extending
the neck).
Trigeminal and facial nerves
The (primitive) glabellar reflex is often present and prolonged in parkinsonian patients. It is elicited by
repeatedly tapping the forehead (between the eyebrows), from outside the field of vision. Blinking after
the initial 2-3 taps is normal. However failure to supress the reflex after subsequent taps is indicative of
Pseudobulbar palsy
This can be a feature of PSP. It is caused by lesions in the upper motor neurons which synapse on the
cranial nerves in the medulla (bulb) of the brainstem (hence pseudo-bulbar, as damage is not to the
cranial nerves themselves).
Signs include:
Dysarthria - slow, thick, indistinct 'Donald duck' speech
Tongue spasticity - it will appear stiff with reduced movement
Brisk jaw jerk - increased activity of the trigeminal nerve following UMN lesion.
Abnormal, labile affect - crying or laughing inappropriately, and alternating between the two

Test for evidence of 'lead-pipe' rigidity. The joints will resist movement in all directions. When rigidity is
combined with tremor, the effect is sometimes called 'cogwheeling'. This is best elicited at the wrist.
Hold the patient's hand (as if in greeting), moving it in a circular motion while steadying their wrist with
your other hand. In the presence of cogwheeling, the motion will be jerky. Cogwheeling can be brought
out more by asking the to patient to simultaneously move their other arm up and down (synkinesis).
Ask the patient to touch their thumb to each of the fingers in that hand, and to make the movements as
big as possible. Parkinsonian patients generally perform this slowly, with progressively smaller amplitude
of movement as they complete the task.
Ask the patient to write their name. Look for evidence of small spidery handwriting (micrographia),
particularly writing which gets smaller across the page as the patient writes

Cerebellar function
The cerebellum may be affected in Multiple System Atrophy (a Parkinson-plus syndrome). Test for past-
pointing and dysdiadochokinesis:
Finger-nose test for dysmetria- ask the patient to alternately touch their nose and your index finger.
As the arm is outstretched to touch your finger, the patient's finger might veer from side-to side
(intention tremor) or overshoot (past-pointing).
To test for dysdiadochokinesis, ask the patient to alternately pronate and supinate their dominant
hand on the back of their non-dominant hand. Test the non-dominant hand in a similar way. Rapidly
alternating these movements will be difficult in patients with cerebellar dysfunction.
Autonomic function
Another feature of MSA may be autonomic dysfunction:
1. Test the patient for postural hypotension by taking their sitting and standing blood pressure.
2. Examine the mouth for evidence of reduced salivation, or simply ask the patient if their mouth feels
3. Ask about bladder problems
4. Ask about constipation
5. Ask about excessive or reduced sweating

Postural instability
With the patient facing away from you and having explained what you are about to do, pull back hard on
their shoulder so as to unbalance them. Normally, people will regain their balance by taking 2-3 steps
backwards and will not fall. Parkinsonian patients often need to take more steps than this, or will fall
Stand close behind the patient while performing this manoevre and keep your hands by their
shoulders in order to prevent the patient falling to the ground.

Ask the patient to walk a short distance, then turn and walk back. Several features are characteristic of a
parkinsonian gait:
1. Difficulty initiating movement (hesitancy)
2. Small, shuffling steps
3. Festination, where the patient speeds up as they walk
4. Reduced arm swing
5. Turning en bloc

Extra questions

Dementia with Lewy-Bodies is commonly present in patients with parkinsonian symptoms. Ask
about visual hallucinations as these are present in 75%, often featuring animals or people. Many
sufferers do not find these hallucinations threatening, and may have good insight as to their origin.
AnMMSE is a useful initial screening tool for neuropsychological changes.

REM-sleep behaviour disorder is another common problem in parkinsonian patients, often appearing
many years before the onset of other symptoms. Patients act out their dreams while asleep, and may
kick or thrash out, potentially harming themselves or their partners. The patient will not be aware of the
movements at the time, so a collateral history from a sleeping partner is helpful.
11. Reduced Consciousness
Written by: Diana Fitzrol from ,
'Mr H has become drowsier' - this is one of the common calls a junior doctor will receive. This happens
not only in the neurology or neurosurgery department, but in other specialties as well. This is also a
common scenario to be tested for in clinical exams. So what is consciousness? It is a state of being
aware of one's thoughts and surroundings. Clinically, this can be assessed by using the Glasgow Coma
Scale (GCS) which consists of the patient's eye, verbal and motor responses. In a more acute situation,
for example during the primary survey for a trauma case, the AVPU (Alert, Voice, Pain, Unresponsive)
scoring system is more convenient to use (see below). Reduced consciousness can vary in severity from
mildly drowsy to obtundation, stupor and coma which is the most severe form.

Tools used to assess conscious level:

Pathophysiology of reduced consciousness
Consciousness is divided into 2 components:

1. Content and cognition
2. Arousal and wakefulness

The content and cognition component of consciousness depends on the cortical function of the cerebral
hemispheres whereas arousal and wakefulness of an individual depends on the interaction between
intact cerebral hemispheres and reticular activating system (RAS) within the brainstem. Lesions which
can cause impaired consciousness include lesions that:

Affect the cortical function diffusely - E.g. metabolic or endocrine disorders. Focal damage to the
cortex does not contribute to disturbed consciousness.
Cause direct pressure on the brainstem - E.g. increased intracranial pressure leading to tentorial
Damage the RAS system within the brainstem itself - E.g. brainstem infarction.
Causes of reduced consciousness
Before managing a patient with reduced consciousness, it is essential to have a grasp on the common
possible conditions that could have caused this (Figure 1). It is important to remember other non-
neurological causes and to look out for these especially the ones which can be reversed rapidly.

Management of reduced consciousness
The definite treatment for a patient with reduced consciousness depends on the underlying aetiology.
However the immediate management for any of these patients irrespective of the cause includes
thorough clinical assessment consisting of history and clinical examination.

History is important in guiding towards a set of likely causes. If the patient is unable to provide an
adequate history, information must be sought by those available such as family, friends and nursing
staffs. Information as listed below should be elicited as accurately as possible:

Preceding events (e.g. recent or previous head trauma suggestive of intracranial haemorrhage )
Onset and duration of reduced consciousness
Progression (e.g. slow progression may be due to metabolic causes, rapid progression can be
secondary to intracranial haemorrhage.)
Pattern (constant or intermittent)
Other signs & symptoms (e.g. limb twitching & incontinence suggestive of possible epilepsy)
Past medical history (e.g. diabetes predisposing to hypoglycaemia)
Drug history
Social history ?Lives alone, occupation, next of kin (in situations where consent is required e.g.
surgical intervention), history of recreational and alcohol abuse

In an acute situation, a concise history is sufficient to help rule out or diagnose immediate life threatening
situations. However, this must be re-visited in detail after making sure that the patient is not in immediate

When assessing a patient with reduced consciousness, it is essential to assess the patient going through
the ABCDE steps (Airway, Breathing, Circulation, Disability, Exposure/everything else):

Establish the patency of the patients airway
Is he/she able to maintain it? Generally if the GCS is less than 8, the patient will require
intubation and an anaesthetist must be informed immediately.

Respiratory effort.
Respiratory rate.
Oxygen saturations.
Administer high flow oxygen via non-rebreather mask Important to maintain well oxygenated
blood and protect the brain from hypoxic injury.
Oxygen therapy in patients with COPD should be titrated as appropriate however in the event of
an acute life-threatening situation, it is essential to remember that hypoxia will cause serious
acute harm to the patient compared to the gradual onset of expected hypercapnea.
Chest examination chest expansion, mediastinal shift, percussion and auscultation of chest.

Blood pressure (BP) and pulse If the BP readings are not recordable ensure to check this
again with a manual sphygmomanometer. If the carotid pulse is impalpable, it is important to call
the cardiac arrest team immediately.
Listen for heart sounds.
Establish venous access Ideally 2 large-bore cannula inserted.
Blood samples These need to be sent urgently to the lab for tests necessary to find the
possible causes of reduced consciousness. Blood tests required are full blood count,
electrolytes, inflammatory markers and renal function. Depending on clinical suspicions,
additional blood test such as toxicology screen, drug levels or blood cultures should be
Arterial blood gas Gives an accurate reading of the patients oxygen and carbon dioxide
partial pressures and provides a rapid picture of the patients metabolic state (acidotic/alkalotic).
Intravenous fluids IV fluids should be commenced especially if there are signs of shock such
as hypotension and tachycardia. If the patient is hypotensive, it is advisable to give a fluid
challenge to assess the responsiveness. Be wary with fluid boluses if the patient has a
significant cardiac history e.g. heart failure or recent myocardial infarction. Normally 500ml of
0.9% sodium chloride is used however in patients with cardiac problems, it is recommended to
start with a lower volume of 250ml of 0.9% sodium chloride instead. Gelofusin can also be used.
There is no evidence currently to support the use of colloids over crystalloids however colloids
are more expansive and do carry the risk of anaphylaxis.
Urine output Patient should be catheterised if not already. Monitor the patients hourly urine
output. The kidneys are one of the earliest organs to show signs of shock. Fluid resuscitation
should aim to produce a minimum urine output of 0.5ml/kg/hr.

GCS It is important to establish the patients current GCS (Figure 1) and always compare it to
the patients baseline. Note that GCS ranges from a minimum of 3 to a maximum of 15. Scores
for eye response ranges from 1 to 4, verbal response is 1 to 5 and motor response is 1 to 6.
Pupils Pupils size and reaction can point towards presence of intracranial pathology or
systemic disorder such as pin point pupils in opiate toxicity. A unilateral fixed and dilated pupil
points towards likelihood of an intracranial pathology.
Glucose levels Hypoglycaemia is essential to rule out as has detrimental effects to the brain
and is rapidly reversible.
Drugs - Always check the kardex for any causative/contributing medications eg. opioids or other
sedating drugs.

Exposure/Everything else
A full examination should be carried out looking for signs of trauma, bleeding, sepsis, drug/
alcohol abuse, seizures and other possible causes.
Relevant investigations arranged urgently - e.g. CT head scan for a patient with recent head

It is important to reassess the patient after each step so that response to any treatment given
can be evaluated and guide further management.

It is useful to know the immediate medical treatment for some common conditions as listed below which
can be commenced as appropriate after assessing the patient in the steps mentioned above (ABCDE).
Different hospitals have different local guidelines for these so it is worth familiarising yourself with them.
Management of patients with acute surgical problems must include input from the relevant surgical
teams for example the general surgical team must be informed regarding a patient with intra-abdominal

10% dextrose IV with close monitoring of blood glucose levels.
Encourage oral diet when glucose levels are back to normal.

Assess fluid status.
Obtain measurement of the sodium levels and osmolality of both the plasma and urine.
Management depends on the underlying cause and be classified into hypervolaemic,
euvolaemic and hypovolaemic hyponatraemia.

Perform septic screen to locate source of infection.
Start broad spectrum antibiotics promptly after blood cultures and urine culture are being sent.
Refer to hospital guidelines for antibiotic therapy.
Provide analgesia if patient is in pain and antipyretic if patient is pyrexial.
Seek microbiologist advice regarding antibiotics choice when receive culture results.

Status epilepticus
Slow IV bolus of 2-4mg lorazepam over ~30 seconds.
If no response within 2 minutes another dose can be given. Alternatives include buccal
midazolam, 10mg IV diazepam or per rectal diazepam.
If seizures do not stop after 2 doses of benzodiazepine, commence IV phenytoin, 18mg/kg at a
rate <50mg/min. At this point patient should have a cardiac monitor on.
If seizures continue despite phenytoin infusion, contact an anaesthetist as patient will need ITU

Diabetic ketoacidosis (DKA)
Dehydration is more lethal in DKA than hyperglycaemia so aggressive fluid resuscitation is
Insulin infusion of 6units/hr should be started with IV fluids. 0.9% sodium chloride with 20mmol
of potassium chloride (KCl) per litre is advised.
Generally the regime used for fluid replacement is 1L in the first hour followed by another litre in
2 hours, 4 hours and 6 hours.
Blood glucose and potassium levels should be monitored closely. Potassium levels should be
monitored 2 hourly during aggressive fluid therapy.
As blood glucose levels decrease to 10-12mmol/L, fluid therapy should be changed to 1L of
10% dextrose with 20mmol of KCl per litre running at a rate of 6 hours with insulin infusion
adjusted according to blood glucose.
The underlying cause for DKA to develop must be sought e.g. infection, non-compliance to
insulin therapy.
Seek advice from the diabetic team to achieve an appropriate insulin regime for the patient.

Reduced consciousness can vary in severity and has many causes which include life-threatening
emergencies. Hence it is essential to have an adequate history, assess the patient promptly in an
ABCDE stepwise and carry out appropriate investigations urgently as these direct towards the likely
diagnosis which then dictates the appropriate management.

1. Lindsay, Kenneth W., Ian Frcp Bone and Geraint Fuller. Neurology and Neurosurgery Illustrated. 5th
ed. Edinburgh: Churchill Livingstone, 2010.
2. Liebenberg, W. Adriaan and Reuben Johnson. Neurosurgery for Basic Surgical Trainees. 2nd ed. ed.
Carnforth: Hippocrates Books, 2010.
3. Loftus, Christopher M. Neurosurgical Emergencies. 2nd ed. ed. New York: Thieme ; Rolling Meadows,
Ill. : American Association of Neurosurgeons, 2008.
4. Longmore, J. M. Oxford Handbook of Clinical Medicine. 8th ed. / Murray Longmore ... [et al.]. ed.
Oxford: Oxford University Press, 2010.
5. Kumar, Parveen J. and Michael L. Clark. Kumar & Clark's Clinical Medicine. 7th ed. ed. Edinburgh:
Saunders Elsevier, 2009.
6. Perel, P. and I. Roberts. "Colloids Versus Crystalloids for Fluid Resuscitation in Critically Ill Patients."
Cochrane Database Syst Rev 6, (2012): CD000567.
11. Upper Limb Examination
Written by: Dhruv Panchal from Oxford Deanery,
Upper Limb Neurological Examination

Firstly prepare yourself and the patient for the examination. An often used mnemonic for this sequence
is WIPER: wash your hands, introduce yourself, permission, expose the patient, re-position the patient.
The arms should be fully exposed including the shoulders, neck and upper back.

Things to look for include:
Posture of the upper limbs: examples include flexed elbow and hyperpronated forearm (indicating
pyramidal weakness); clawing of the fingers or hands (ulnar nerve palsy, T1 lesion, non-neurogenic
causes e.g. Volkmanns contracture).
Wasting: a sign of lower motor neurone lesions but also present in long standing upper motor
neurone disease due to disuse atrophy. Is the wasting generalised or focal? If focal; is it the thenar
eminence only (median nerve palsy e.g. Fig 1), hypothenar and first dorsal interosseus with clawing
of the medial two fingers(ulnar nerve palsy e.g. Fig 2) or generalised hand wasting (T1 lesion)?

Fasciculations: a sign of lower motor neurone lesions. This is involuntary twitching of muscle fibres
due to spontaneous depolarisation of lower motor neurones causing contractions.
Scars: posterior midline neck scars for surgical decompression of symptomatic cervical spondylosis,
traumatic scars from injury to the arm or axilla, ulnar nerve transposition scars around the elbow,
carpal tunnel decompression scars at the wrist, breast surgery scars (potential injury to
intercostobrachial nerve, long thoracic nerve or thoracodorsal nerve).
Abnormal movement: tremors, choreoathetosis, dystonia.

Finally, a very useful manoeuvre to begin with is to ask the patient to reach forward with their
outstretched hands with their palms facing upwards. Ask the patient to close their eyes and briefly
observe the fingers to look for writhing movements (pseudoathetosis) caused by severe proprioceptive
disease. Also observe for pronator drift. This is positive if the arm starts to fall with the forearm drifting
into pronation. This is a sign of pyramidal weakness. Then you can ask the patient to keep their arms in
place whilst you gently tap each arm downwards. An exaggerated, pendular swingingof the arm around
the original position may suggest cerebellar disease.

Assess Tone:
Before moving the arms, always ask if the patient is in pain or discomfort. Tone is assessed by passively
moving the elbows through extension and flexion, moving the forearm through pronation and supination,
moving the wrist through flexion, extension and circumduction.
You must decide if there is hypertonia (increased tone) or hypotonia (reduced tone) or normal tone.
There are several types of hypertonia and understanding the differences between them will allow you to
properly examine for them:
1. Spasticity: this is velocity dependent and often called clasp knife spasticity and is a sign
ofpyramidal disease. Practically, it is elicited by comparing slow extension with quick extension of the
elbow and slow supination with fast supination of the forearm. A catch during the quick movement
indicates spasticity.
2. Rigidity: this is not velocity dependent and there is increased tone throughout the range of
movement; it is sometimes called lead pipe rigidity. This is a sign of extrapyramidal disease.There
is sometimes an associated tremor which can be elicited by distraction techniques (e.g. flapping the
contralateral arm or asking the patient to count backwards). Cogwheeling is rigidity plus tremor and is
best felt at the wrist.
3. Paratonia: hypertonia with an involuntary variable resistance during passive movement. Resistance
increases with movement speed, but is not limited to a certain direction. It may be seen in dementia.
Increased tone usually suggests an upper motor neurone lesion; hypotonia suggests a lower motor
neurone lesion or cerebellar lesion. Patients with acute stroke may sometimes have reduced tone but
will later develop spasticity. Shoulder tone can rarely be attributed to neurological disease and its
assessment is of limited use.

Muscle power is graded using the MRC scale, in which different degrees of power are denoted a
0. No movement
1. Flicker of muscle contraction
2. Movement of muscle with gravity eliminated
3. Movement of muscle against gravity
4. Movement of muscle against some resistance
5. Normal power

The customary sequence of examination is to test proximal to distal but depending on clinical context,
the examination of individual muscles may also be required. The muscle belly or tendon should also be
palpated during contraction. A common sequence of movements which are tested, along with muscle
name, root, and peripheral nerve is shown in table 1:

Table 1: Movements of the upper limb

There are a few common pitfalls in technique students (and doctors!) fall into during the power
1) Resisting movement of the muscle too early. For example, whilst testing shoulder abduction
examiners often begin resisting the movement before the patient is able to abduct their shoulders to 90
degrees. This makes it difficult to know if there is 4/5 power or only 2/5 power. The correct technique
would be to let the patient perform the movement against gravity, then resist. If the patient cannot move
the muscle against gravity, then remove its effect by asking the patient to perform the movement along
the bed.
2) Not isolating the muscle. This occurs frequently during elbow movements where the patient is
asked to push the examiner away and uses her whole body weight with shoulder flexion to add power.
Isolate each muscle by stabilising the relevant joint e.g. elbow during flexion and extension, wrist during
wrist extension.
3) Elbow flexion is performed by two main muscles: biceps and brachioradialis. Putting the forearm in
full supination, elbow flexion tests biceps. Putting the forearm in the neutral position, midway between
supination and pronation, elbow flexion tests brachioradialis. This is important to understand as biceps
is supplied by the musculocutaneous nerve, whereas brachioradialis is supplied by the radial nerve.
4) Thumb abduction is controlled by two different nerves but is often used as a test of median nerve
motor function. Thumb abduction moves the thumb perpendicular to the plane of the palm. Patients often
find it difficult to accurately perform this move. One tip is to ask the patient to flatten their hand on their
lap, palm upwards. The examiner places his finger a thumbs length above the middle of the patients
palm and simply asks the patient to touch his finger with their thumb. Once the patient does this, ask the
patient to keep their thumb still. Attempt to adduct the thumb and palpate the belly of abductor pollicis
brevis, located in the thenar eminence. Palpation of this muscle belly is important because thumb
abduction is also controlled by abductor pollicis longus which is supplied by the radial nerve. Therefore
using thumb abduction for median nerve motor function may be confounded by poor technique.
Once power is examined and weakness is elicited you should consider which pattern weakness it falls
into. Common patterns include:
1) Proximal weakness or distal or generalised weakness?
2) Weakness in a nerve distribution?
3) Weakness in a myotome distribution?
4) Is there fatigueability? (This suggests neuromuscular disease e.g. myasthenia gravis and can be
tested by eliciting if there is increased weakness after repetitive muscle movement).

Coordination is looking for evidence of cerebellar lesions. The two main tests include finger-nose testing
and dysdiadochokinesia.
1) Finger-nose testing is performed by asking the patient to touch the tip of their nose and then the
tip of your finger alternately. Make sure the patient has to fully use her outstretched hand to reach your
finger. This will exaggerate intention tremor which is seen as an increasing amplitude tremor the closer
the patient gets to the target. If present there is often dysmetria; misjudging the distance of your finger
and therefore missing it. If the patient is clearly dysmetric, ask them to touch their chin instead, this will
prevent them from injuring their eye.
2) Dysdiadochokinesia is the inability to perform rapidly alternate moves. Ask the patient to supinate
and pronate the forearm whilst tapping the palmar or dorsal aspect of their hand onto the contralateral

Deep tendon reflexes
The main reflexes tested in the upper limb are the biceps, supinator and triceps. Here are a few points
on technique for those finding eliciting reflexes difficult:
1. Get the patient to completely relax. Ask the patient to let you take the complete weight of their arm.
2. Tendon hammer technique: grasp the tendon hammer from the end and use the weight of the hammer
to provide the force. As you strike the tendon, inspect the muscle belly to look for contraction. Always
test reflexes in pairs to compare.
3. If you cannot elicit the reflex use reinforcement: usually after two attempts if you are unable to elicit
the reflex, you should use reinforcement techniques. This is called Jendrassiks manoeuvre. For the
upper limb, ask the patient to clench their teeth on the count of three, and strike the tendon at the
same time. Reflexes are often denoted by a variable number of + symbols, referred to as plusses.
- = absent reflexes even with reinforcement
+ = reflexes present with reinforcement
++ = normal reflexes
+++ = hypereflexia without clonus
++++ = hypereflexia with clonus
4. For the biceps reflex: flex the elbow to approximately 130 degrees, rest the arm and forearm onto the
patients chest. Palpate the biceps tendon, place a finger onto it and strike your finger. Inspect for
biceps contraction as well as palpating for a contraction under your finger. The biceps reflex
tests C5,6 root and the musculocutaneous nerve.
5. For the supinator reflex: this is actually testing brachioradialis and is best tested by striking a finger
placed on the radial side of the forearm with it in the neutral position. Inspect for brachioradialis
contraction in the lateral antecubital fossa. This tests C5,6 root and the radial nerve.
6. For the triceps reflex: bring the patients arm across the chest and strike the bare tendon just proximal
to the olecranon. Observe for triceps contraction. This tests C7,8 root and the radial nerve.
Hyperreflexia may indicate an upper motor neurone lesion; absent reflexes may indicate a lower motor

Two modalities of sensation are tested representing different spinal cord tracts. Proprioception and light
touch is predominantly carried by the dorsal column whereas pain and temperature is carried by the
spinothalamic tract.
Testing proprioception
Proprioception is the ability to sense joint position. This is important to coordinate movement, as well as
to prevent joint damage. To test proprioception, grasp the distal phalanx of the index finger by the sides
and isolate the distal interphalangeal joint. Demonstrate to the patient extension is denoted as up and
flexion is down. Ask the patient to close their eyes and move the distal phalanx a few millimetres and
ask the patient which way you are moving it. If the patient is unable to correctly identify the direction of
movement, there is proprioceptive pathology. To identify how proximally the patient is affected, move
either to the metacarpophalangeal joint or wrist and continue testing proprioception. Keep moving
proximally to the elbow and then shoulder until the patient has normal joint proprioception. Remember to
test both sides.
Testing light touch and pain
Use a wisp of cotton wool and a sharp point, often found on the end of neurotips, for pain sensation.
Touch the patients sternum and explain this is the reference point the patient should mentally compare
limb sensation with. Ask the patient to rest his arms in the anatomical position, close their eyes and then
proceed to test the areas supplied by the different dermatomes in the upper limb:
1. C5 mid point of the lateral arm
2. C6 tip of the thumb
3. C7 - tip of the middle finger
4. C8 ulnar border of the little finger
5. T1 mid point of the medial arm
If a peripheral nerve problem is suspected, individual nerves can be tested by eliciting sensation in the
following areas:
1. Radial nerve dorsal hand, webspace between thumb and first finger
2. Median nerve radial border of the first finger
3. Ulnar nerve ulnar border of the little finger
4. Musculocutaneous nerve mid point of the lateral forearm
5. Axillary nerve regimental area found around the insertion of the deltoid muscle
It is important to ask the patient if the sensation is the same as the sternum each time. If there are
bilateral changes, ask how they compare to each other. Note that only asking if sensation is the same on
both sides can miss bilaterally reduced sensation.
Testing temperature:
This is not often done but in principle it would be tested in the same manner as light touch and pain but
with objects of different temperature e.g. cold side of a tuning fork.

Additional examination points for suspected median nerve pathology
Patients often appear as short cases for PACES cases and indeed in clinical practice with a history
suggestive of median nerve neuropathy. This most commonly manifests as carpal tunnel syndrome a
compressive median nerve neuropathy within the carpal tunnel of the wrist. Symptoms are numbness
and tingling in the thumb, index, middle finger and lateral half of the ring finger. This is often exacerbated
at night, and can be relieved by shaking.
When examining the median nerve, both motor and sensory function should be tested. Specific tests for
identifying potential locations of nerve compression should also be done.
Testing the motor function of the median nerve
Thenar eminence wasting is highly suggestive of median nerve neuropathy. Thumb abduction is often
used as a motor screening movement for median nerve disease. The median nerve supplies most of the
flexor muscles in the forearm and the 'LOAF' muscles of the thenar eminence:
1. Lateral 2 lumbricals
2. Opponens pollicis
3. Abductor pollicis brevis
4. Flexor pollicis brevis
Testing the sensory function of the median nerve
As suggested above, this is tested on the radial border of the index finger. The median nerve however
supplies the entire lateral palm from the wrist, to the tips of the first, second and half if the ring finger
including the thumb. On the dorsum, only the tips of the index, middle and ring fingers to about the distal
interphalangeal joint is supplied by the median nerve. Carpal tunnel syndrome does NOT usually affect
the palm as the palmar branch of the median nerve supplying this area branches proximal to the carpal
tunnel and does not run within it.
Examining for the location of compression
Carpal tunnel syndrome is caused by compression within this space. A number of clinical tests are
described to try to reproduce these symptoms:
1. Durkans test direct pressure onto the flexor retinaculum, the roof of the carpal tunnel
2. Tinels test tapping the flexor retinaculum
3. Phalens test asking the patient to hyperflex the wrists, or reverse Phalens where the patient is
asked to extend the wrists.
Other anatomical regions apart from the wrist where the median nerve can be damaged include:
1. Around the humerus e.g. secondary to supracondylar fracture therefore examine for cubitus varus (the
so called gunsling deformity).
2. In the cubital fossa: the median nerve can get compressed inbetween the heads of pronator teres and
can mimic carpal tunnel syndrome; sometimes even needlessly putting patients through carpal tunnel
decompression surgery! Test for this by asking the patient to resist supination. A positive test is
reproduction of symptoms.
3. In the forearm: the median nerve gives off a branch called the anterior interosseous nerve (AIN) which
supplies part of flexor digitorum profundus, flexor pollicis longus and pronator quadratus. A simple way
to test the AIN is to ask the patient to perform the ok sign. The patient should be able to flex the
distal interphalangeal joint of the index and interphalangeal joint of the thumb to correctly shape the
ok sign. Alternatively ask the patient to make a closed fist and bury their fingers into their hand. AIN
palsy will result in the thumb, index and middle finger being still visible (fig 3).

Additional examination points for suspected ulnar nerve palsy
The ulnar nerve supplies all of the intrinsic muscles of the hands except those in the thenar eminence. It
also provides branches in the forearm to flexor carpi ulnaris and the medial two flexor digitorum profundi.
The most common location for ulnar damage is the elbow where it winds around the medial epicondyle
of the humerus and is prone to damage. Damage to the ulnar nerve can lead to a claw hand. This is
where there is extension of the metacarpophalangeal joints (MCPJ) of the ring and little fingers, with
flexion of their interphalangeal joints (IPJ). The clawed posture is due to denervation of the medial two
lumbricals (whose action is to flex the fingers at the MCPJ and extend at the IPJs) and medial two flexor
digitorum pronfundus muscles (whose action is to flex the ring and little fingers at the distal IPJs).
The ulnar paradox is the description of increased deformity the more distal the lesion. This is because
distal lesions preserve flexor digitorum profundus innervation which acts to flex the fingers more and
cause more of a deformity.
For motor testing, wasting of the hypothenar eminence and the intrinsic muscles of the hand is highly
suggestive of ulnar neuropathy. Palpating the belly of the first dorsal interosseous muscle on index finger
abduction is also a good area to check for intrinsic muscle wasting. An often used test is
eliciting Froments sign (fig 4). This is where the examiner places a piece of paper between the
patients thumb and index finger, and asks them to stop it being pulled out of their hand. Adductor pollicis
is supplied by the ulnar nerve. Patients with ulnar nerve palsy will flex the interphalangeal joint of the
thumb to keep the paper from slipping; this is supplied by the median nerve. This is a positive Froments
sign. For testing sensation, the ulnar border of the little finger is used.

Apart from the medial epicondyle, the ulnar nerve can also get trapped in other places:
1. Arcade of Struthers: this is a musculofascial band approximately 8cm proximal to the medial
epicondyle arching from the medial head of triceps to the medial intermuscular septum.
2. Near the cubital fossa: the ulnar nerve can sometimes get trapped in between the heads of flexor
carpi ulnaris. This is called cubital tunnel syndrome.
3. Guyons canal: this is a bony canal between the pisiform and the hook of the hamate bone in the
carpal tunnel. Bike riders and people operating pneumatic drills are often prone to compression here.
Additional examination points for suspected radial nerve pathology
The radial nerve is a long and complicated nerve with innervations to many muscles. It supplies the
entire posterior compartments of the arm and forearm. Radial nerve palsy is sometimes synonymous
with Saturday night palsy. A classical history is a young patient who has spent the night slumped on his
arm only to wake up with a wrist drop (ironically probably on Sunday morning). This is due to
compression of the radial nerve around the humerus and most often self-resolves. However, all radial
nerve palsies do not simply have wrist drop! When examining the radial nerve, it is important to consider
the locations it can get damaged:
1. Axilla historically from old style crutches, causing a complete radial nerve palsy including loss of
triceps power and reflex
2. Humerus depending on where along the humerus the radial nerve is damaged, the clinical picture
will differ slightly:
1. Nerve damaged at upper third: complete paralysis including triceps
2. Nerve damaged at middle third: this is Saturday night palsy and preserves triceps power and reflex
but brachioradialis function (and therefore supinator reflex) is lost.
3. Nerve damaged at lower third: here triceps and brachioradialis (and both triceps and supinator
reflexes) are preserved. Everything distal in the posterior forearm compartment is affected.
4. Elbow here the radial nerve gives off the deep branch of the radial nerve to extensor carpi radialis
longus. This muscle alone can extend the wrist and therefore wrist drop will not be a feature.
Instead, the patient would not be able to extend the fingers at the MCPJs causing finger drop.
5. Forearm here the superficial radial nerve, which is entirely sensory to the web space on the dorsal
hand is compressed. Also known as Wartenberg syndrome.

Finally, understanding the mechanics of movement can help reduce unnecessary work up of simple
radial nerve palsies. If a patient were to present with wrist drop, the other nerves should of course be
examined to exclude mononeuritis multiplex or brachial plexopathy. When wrist drop is present, poor
technique can provide falsely positive signs resulting in needless investigations and anxiety for both
patient and doctor. When testing for finger abduction (spread your fingers), a patient with wrist and
finger drop will have weak abduction if the hand is left in the dropped position. This is simply because
finger abduction is strongest with the fingers in the plane of the palm, due to the anatomy of the muscles.
This is not due to a concomitant ulnar nerve palsy! Likewise, power grip is often used as a crude test of
median nerve function. Power grip, however, requires the radial nerve to bolster the wrist in firm
extension to counteract the powerful finger flexors supplied by the median nerve. A person with wrist
drop will suddenly have normal power grip if the wrist is manually hyperextended for them, thus avoiding
a false diagnosis of concomitant median nerve neuropathy!

The upper limb neurological examination should begin with a proper introduction. Then proceed to
inspect for signs of neurologic disease. A recommended starting manoeuvre is to test for pronator drift
but then move onto to formal testing of tone, power, reflexes, coordination and sensation. Decide if the
lesion is an upper or lower motor neurone lesion. If there is a lower motor neurone lesion, decide if this is
in a myotome, peripheral nerve, neuromuscular junction or muscle. Brachial plexus lesions can give
mixed peripheral nerve type pictures so be aware of this diagnosis. Finally, complete the examination by
offering to examine the lower limbs and cranial nerves. Report your findings with a view to delineate
where the lesion is in the neuroanatomical axis, then proceed to suggest aetiologies for the clinical
syndrome the patient has.
1) Fig 1:
2) Fig 2:
3) Fig 3: Postgrad Med J 2002;78:625 doi:10.1136/pmj.78.924.625
4) Fig 4: