Dr.

Ali’s Uworld Notes For Step 2 CK

Neurology
Important Dermatomes -

Nipple- T4

Xiphoid- T6

Umblicus- t10

Brown Sequard Syndrome - Damage to the lateral spinothalamic tracts causes contralateral loss
of pain and temperature sensation beginning two levels below the level of the lesion.

Craniopharyngiomas - They are benign tumors arising from Rathke's pouch and are more
common in children. Although they are infrequently seen in adults, Craniopharyngiomas
actually have a bimodal age distribution - children and 55-65 years age group.The tumor is
located above the sellaturcica. and consists of multiple cysts. which are filled with an oily fluid.
The tumor grows gradually over a period of years. and usually presents with hypopituitarism. In
children retarded growth is the most prominent feature (due to growth hormone and thyroid
hormone deficiency) whereas sexual dysfunction is more prominent in adults. Women can
present with amenorrhea. Since the tumor compresses the optic chiasm,bitemporal blindness is
a classic sign of the disease. Headaches occur due to an increased intracranial pressure. The
diagnosis is made with an MRI or CT scan and treatment is with surgery and/or radiotherapy.

Spontaneous Intracranial Hemorrhage - Hypertension is a common cause of spontaneous

intracranial hemorrhage with the most common sites being the basal ganglia, cerebellum,
thalamus and pons.
Hypothyroidism & Memory - Hypothyroidism is an important cause of reversible changes in
memory and mentation. It will be accompanied by systemic changes such as weight gain,
fatigue and constipation.

Pseudodementia is a presentation of major depression in the elderly. Depressed patients may

have memory changes and other symptoms concerning for dementia.

Burning mouth syndrome is a rare cause of facial pain. It is caused by a virus and the individual
has reddened mucosa and has significant pain. The condition is aggravated by dryness. It is
treated by supportive care.

Carotidynia is a neurological condition caused by inflammation of the carotids and the vagus.
The pain is sharp and localized to the carotid artery distribution in the neck. Usually, the
intensity of pain is much less. The diagnosis is clinical, although an MRI is sometimes required.

Thalamic stroke (Dejerine-Roussy syndrome) - This condition is caused by a stroke involving

ventral postero-lateral (VPL) nucleus of the thalamus which transmits sensory informationfrom
the contralateral side of the body. The classic presentation involves contralateral
hemianesthesia that can be accompanied by transient hemiparesis,athetosis or
ballisticmovements. Dysesthesia& Hyperesthesiaof the area affected by the sensory loss is
characteristic and is called thalamic pain phenomenon.

Cerebral emboli are one of the most dreaded consequences of endocarditis. They occur when
pieces of infected valvular vegetations break off and enter the CNS circulation. Treatment is
with antibiotics. Many aspects of a patient's history suggest that he has endocarditis. First, he is
an intravenous drug user who has been suffering from chills for the last week. Additionally, he
has evidence of cardiac disease on ECG. Finally, his urinalysis shows proteinuria, most likely
from immune complex disease or septic emboli. Although IV drug abusers can get right-sided
endocarditis, left-sided endocarditis is also very common! The next steps in the management
of this patient are initiation of broad-spectrum antibiotics and echocardiogram to evaluate for
valvular vegetations.

Essential tremor is characterized by a tremor that is suppressed at rest and exacerbated toward
the end of a goal-directed movement. Affected patients typically have difficulty holding a
newspaper, writing legibly, drinking a cup of coffee, or feeding themselves. It is diagnosed by
the presence of an action tremor in the absence of additional neurologic signs. In contrast to
an essential tremor, the tremor associated with Parkinson disease is a resting tremor, which is
worse during rest and improves with intention.

First line treatment for essential tremor is propranolol, especially if the patient is also
hypertensive. Alternative medications include primidone ortopiramate.
Primidone is an anticonvulsant that gets converted into phenylethylmalonamide and
phenobarbital. The administration of primidone may precipitate acute intermittent porphyria
which manifests as abdominal pain, neurologic and psychiatric abnormalities.

Vertigo is a sensation of motion when no motion is present or an exaggerated sense of motion

for a given bodily movement. It must be distinguished from other sensations that are often
described by patients using similar terminology, such as imbalance, light-headedness, and
syncope. Vertigo resulting from vestibular dysfunction tends to be of sudden onset, interfere
with walking, and cause nausea and vomiting. Typical causes of vestibulopathy include
Meniere's disease, perilymphatic fistulas, benign positional vertigo, labyrinthitis, and acoustic
neuromas.

Acute Labirynthitis aka Vestibular Neuritis - Acute labyrinthine dysfunction usually causes
severe vertigo. In many instances, the etiology is not found and the terms "acute labyrinthitis"
or "vestibular neuritis" are often used.

Benign paroxysmal positional vertigo (BPPV) - Another important cause of vertigo caused by
labyrinth dysfunction is called benign paroxysmal positional vertigo (BPPV) where the
symptoms are precipitated by recumbent head position. Approximately 30% of cases of BPPV
occur after head trauma but typically no precipitating causes are found.

Vestibular Dysfunction due to Drugs–Patients present with gait abnormality and vertigo. They
may have the visual illusion of room spinning around them or the environment moving to & fro.
Ototoxicity and nephrotoxicity are potential adverse effects of aminoglycosides. Of the
aminoglycosides, gentamicin is especially vestibulotoxic. The mechanism underlying
aminoglycoside-induced ototoxicity involves damage to the motion-sensitive hair cells in the
inner ear. Gentamicin-associated ataxia and vertigo can be either transient or permanent. Look
for a patient being treated with Aminoglycosides.

Meniere's disease is a disorder of the inner ear that presents with the triad of dizziness,
hearingloss, and tinnitus.

Transient ischemic attacks of the posterior circulation (i.e., supplying the vestibular nuclei and
labyrinth) are associated with vertigo. The patients have other neurological deficits depending
on the area of ischemia. Vertigo is not typically related to recumbent head positioning

Patients with cardiovascular and dehydration problems present with Orthostatic HTN which is
totally different thing. Look for a decrease in systolic BP of greater that 20 mmHg when moving
from Supine to standing position.
Restless Leg Syndrome (RLS) - The four cardinal symptoms of RLS are:

(1) an uncomfortable sensation or urge to move the legs

(2) discomfort which worsens in the evening or during sleep
(3) discomfort which worsens at rest. and
(4) discomfort alleviated by movement of the affected limb(s)

The lower extremity discomfort may also get better after massage or warming. RLS is more
common in middle aged and older patients. as well as in those with chronic kidney disease
and/or iron deficiency anemia. It can have a profoundly negative impact on the sleep of
patients and their sleeping partners. The mechanism of primary RLS is unclear but it may
involve abnormalities of dopaminergic transmission in the central nervous system.
Dopaminergic agonists (e.g. pramipexole and ropinerole) or levodopacan be effective in
reducing symptoms and improving sleep quality.

Trigeminal Neuralgia & Corneal Anasthesia- The trigeminal nerve (cranial nerve V) contains
both motor and sensory fibers in its three branches. The first branch, the ophthalmic nerve
(V1)carries sensory fibers to the scalp,forehead, upper eyelid, conjunctiva, cornea, nose and
frontal sinuses. Damage to V1 results in corneal anesthesia; thus, a lesion on the eye may not
be sensed if a V1 lesion is present.

Huntington's Disease - It is an autosomal dominant neurodegenerativedisorder. Patients

usually present in their forties or fifties with chorea and/or behavioral disturbance. Chorea
is characterized by sudden, jerky and irregular movements of the extremities. Impaired
judgment,Executivefunction, awareness and attention occur at an early stage. Memory
impairment is a late finding. Depressionirritability and social withdrawal are also common.
Atrophy of the caudate nucleus is a characteristic featureand is seen on neuroimaging as
enlargement of lateral ventricles.

Diffuse atrophy of the cerebral cortex is a feature of Alzheimer's disease.

Atrophy of the lenticular nucleus is most marked in patients with Wilson's disease.

Atrophy of the frontal and/or temporal lobes is a feature of Pick's disease which presentswith
dementia and personality changes

Tick Borne Paralysis Vs GuillianBarre Paralysis - Tick-borne paralysis is characterized by rapidly

progressive ascending paralysis, absence of fever, absence of sensory abnormalities and normal
CSF examination. Patients usually present with progressive ascending paralysis over hours to
days. Fever is typically not present; hence. a history of fever or prodromal illness makes the
diagnosis unlikely. Sensation is usually normal. The CSF examination is typically normal.
Meticulous search and removal of the tick usually result in improvement within an hour and
complete recoveryafter several days.
Guillain-Barre syndrome presents with an ascending paralysis over days to weeks. Sensation is
either mildly or grossly abnormal. Approximately two-thirds of patients give a history of an
antecedent respiratory tract or gastrointestinal (especially Campylobacter) infection. CSF
analysis reveals an elevated protein level with normal glucose levels, WBC, and RBC counts.
This is known as albumino-cytologic dissociation (high protein with few cells). The treatment
includes IV immunoglobulin or plasmapheresis.

GBS can lead to respiratory muscle weakness and may occasionally result in respiratory failure.
The best way to monitor respiratory function in such cases is through serial measurements of
bedside vital capacity.

Delerium - Delirium is an acute confusional state characterized by a reduced or fluctuating level

of consciousness and impaired attention. Patients may present with anxiety, irritability,
impaired recall, amnesia, insomnia, disorientation, and/or visual hallucinations. The possible
causes of delirium are numerous. The most common cause in hospitalized patients overall is
substance abuse or withdrawal. Other potential causes include medication adverse effects,
infection, metabolic/electrolyte derangements, vascular disease affecting cerebral perfusion,
and psychiatric illness. In the elderly, urinary tract infection, pneumonia and metabolic
disturbances are all common precipitating factors. For this reason, urinalysis and measurement
of serum electrolytes should always be included in the initial work-up of acute delirium in the
elderly.

It may be frequently be superimposed on underlying dementia.

Fronto – Temporal Dementia - The characteristic features of this condition are personality
changes (e.g., euphoria, disinhibition, apathy), compulsive behaviors (e.g., peculiar eating
habits, hyperorality), and impaired memory. New onset Promiscuity &Coprolalia
observed.Visuospatial functions usually remain intact. A positive family history may be present.
20 to 40% of patients have family history of frontotemporal dementia.

Dementia with Lewy bodies (DLB) is characterized by fluctuating cognitive impairment. Bizarre
visual hallucinations may occur."Lewy bodies" or eosinophilicintracytoplasmic inclusions
representing accumulations of alpha-synuclein protein may be seen in neurons of the
substantianigra, locus ceruleus, dorsal rapheand substantiainnominata. Treatmentof motor and
psychiatric symptoms includes acetylcholinesterase inhibitors like rivastigmine. For
hallucinations refractory to acetylcholinesterase inhibitors atypical antipsychotics may be of
benefit.

Alzheimer's disease is a progressive dementia. Its associated risk factors are: age, female
gender, positive family history, head trauma, and Down's syndrome. The typical first symptoms
are subtlememory loss, language difficulties and apraxia, followed by impaired judgment and
personality changes.

Alzheimer's disease is the most common cause of dementia in the United States. Early signs
include visuospatial deficits (getting lost in one's own neighborhood), problems with
anterograde memory formation (old memories tend to be preserved), personality and
behavioral changes (apathy, agitation) and apraxia (difficulty using a comb),Hallucinations,
changes in alertness, gait impairment and urinary incontinence may occur late in the disease.
The diagnosis is madeclinically by assessing the mini-mental status exam (MMSE),
neuropsychological testing and the following clinical criteria.

• Two or more areas of cognitive deficits

• Progressively worsening memory and other cognitive function
• No disturbance of consciousness
• Onset between 40 and 90 years
• Absence of other systemic or neurologic disorder causing progressive cognitive defects

Parkinson’s disease (PO)– It is a neurodegenerative disorder caused by accumulation of alpha-

synuclein within the neurons of the substantianigra pars compacta, which ultimately
leads to the death of these neurons. The three cardinal signs of PO are rest tremor, rigidity and
bradykinesia.The presence of two of these three signs suggests a clinical diagnosis of PO, which
can be further confirmed by physical examination. To date, there are no imaging or laboratory
tests that can be used to confirm this diagnosis with any greater accuracy than physical
examination. The most common presenting sign of PO is an asymmetric resting tremor in the
distal part of an upper extremity. To summarize, physical examination findings that contribute
to a clinical diagnosis of PO include:

Tremor:
• A resting 4 to 6 Hz tremor with a "pill rolling"quality
• Frequently first manifests in one hand, and may then slowly generalize to involve the other
side of the body and the lower extremities

Rigidity:
• Baseline increased resistance to passive movement about a joint which may be uniform (lead
pipe) or oscillating (cogwheel rigidity)

Bradykinesia:
• Difficulty initiating movements, as when starting to walk or rising from a chair
• Narrow-based, shuffling gait with short strides and without arm swing (festinating gait)
• Micrographia (small handwriting)
• Hypomimia (decreased facial expression)
• Hypophonia (soft speech)
Postural instability:
• Flexed axial posture
• Loss of balance during turning or stopping
• Loss of balance when pushed slightly from a stationary bipedal stance
• Frequent falls

The presence of at least two of these signs on physical examination is grounds for a clinical
diagnosis of this disease.

The most effective symptomatic therapy for parkinsonism is levodopa/carbidopa. The most
common early side effects are hallucinations, dizziness, headache and agitation. After
severalyears of therapy,Involuntary movements are more likely to occur.
Trihexyphenidyl is an anticholinergic medication sometimes used in the treatment of
Parkinson's disease. generally in younger patients where tremor is the primary symptom.

Anticholinergic excess – This occurs with use of trihexyphenidyl, a medication used for
Parkinson's disease and drug-induced extrapyramidal symptoms. Benztropine is another agent
in this class. The classic anticholinergic symptoms can be remembered with the phrase "red as
a beet, dry as a bone, hot as a hare, blind as a bat, mad as a hatter, and full as a flask,"
describing flushing, anhidrosis/dry mouth, hyperthermia, mydriasis/vision changes,
delirium/confusion, and urinary retention/constipation, respectively. Additional anticholinergic
symptoms include: headache, dizziness, and tachycardia. They can also precipitate acute
glaucoma which presents as headache &retro orbital pain.

Multiple System Atrophy (Shy-Drager syndrome) – It is a degenerative disease characterized by

the following:

1. Parkinsonism

2. Autonomic dysfunction (postural hypotension, abnormal sweating, disturbance of

bowel or bladdercontrol, abnormal salivation or lacrimation, impotence, gastroparesis,
etc.)

3. Widespread neurological signs (cerebellar, pyramidal or lower motor neuron)

Always consider Shy-Drager syndrome when a patient with Parkinsonism experiences

orthostatichypotension (causing patient to fall), impotence, incontinence, or other autonomic
symptoms (dry mouth & dry skin). The accompanying bulbar dysfunctionand laryngeal stridor
may be fatal. Anti-Parkinsonism drugs are generally ineffective, and treatment isaimed at
intravascular volume expansion with fludrocortisone, salt supplementation, alpha-
adrenergicagonists, and application of constrictive garments to the lower body.

Multi-infarct dementia accounts for 15-20% of dementia cases. The characteristic feature is
cognitive dysfunction with motor and sensory neurological dysfunction. The associated risk
factors are: older age, male sex, black race, cigarette smoking, hypertension, diabetes, and
vasculitis. The treatment is generally directed towards the risk factors.

NeurosyphilisDementia - There are four types. The general paresis type is characterized by
general involvement of the cerebral cortex, decrease in concentrating power,memory loss,
dysarthria, tremors of finger and lips, irritability and mild headaches. In addition, there is
acharacteristic personality change, with the patient becoming slovenly, irresponsible, confused
and psychotic.
HIV Dementia - This presents with anorexia, weight loss, fatigue, mild/borderline cognitive
impairment and history of drug abuse should be tested for HIV infection. Undiagnosed or
advanced HIV infection can be associated with dementia. Look for IVDU + Dementia. No Focal
Neurlogical deficits present.

Pseudodementia – It is Major Depressive Disorder + Dementia in an Elderly person. Treat with

antidepressants like SSRI.

Creutzfeldt-Jakob - The typical features include rapidly progressive dementia, myoclonus and
sharp, triphasic, synchronous discharges on EEG. Creutzfeldt-Jakob disease is a spongiform
encephalopathy caused by a prion. Most patients die within one year of symptom onset.

Acute Agitation in Dementia - Irrespective of the etiology of dementia, all patients with severe
agitation need to treatedand the treatment of choice for agitation in the elderly is low-dose
haloperidol. Atypical antipsychotics like quetiapine and risperidone may also be used.

Normal Ageing Vs Dementia - Normal age-related cognitive changes include

tiredness,Occasional forgetfulness, occasional word finding difficulty and trouble falling asleep.
Dementia cannot be diagnosed unless there are functional impairments.
Impairment of daily functioning is essential in distinguishing between dementia and normal
changes of aging. Patients with dementia have functional impairments.

Normal Pressure Hydrocephalus – Triad of abnormal gait, incontinence and dementia (Wet,
Wacky & Wobbly).NPH is a clinical syndrome accompanied by increased ventricular size
without persistent elevations in intracranial pressure. Symptoms result from distortion of the
periventricular brain matter. Affected patients typically have a slow, broad-based, shuffling gait.
Urinary incontinence is classic but fecal incontinence may develop at advanced stages. The
dementia in NPH is characterized by memory loss but no focal neurologic changes.

The prevailing theory for the pathogenesis of NPH is that patients have a transient increase in
intracranial pressure that causes ventricular enlargement. After the ventricles enlarge, the
pressure returns to normal. The initial increase in ventricular size may be due to either
diminished CSF absorption at the arachnoidvilli or obstructive hydrocephalus.

The diagnosis is made by demonstrating enlarged ventricles on CT or MRI plus normal opening
pressure on lumbar puncture. Treatment consists of sequential CSF removal. This is initially
done by performing serial large volume lumbar punctures. A ventriculoperitonealshunt may be
placed if the patient's symptoms respond to lumbar punctures.

Gait impairment is the most prominent clinical features of NPH and appears early in its
disease course.

Alzheimer’s VS NPH - When you see a patient with gait impairment, Dementia (memory
problems) & Urinary Incontinence, It could be Alzheimer’s or NPH.

If gait impairment develops 1st, it NPH.

If Memory impairment develops 1t, it’sAlzheimer’s.

Intracranial Hypertension (ICH) - ICH is defined as intracranial pressure more than20 mm Hg.
There are a number of potential etiologies, including trauma, space-occupying lesions,
hydrocephalus, and impaired CNS venous outflow. Typical symptoms of ICH include diffuse
headaches that tend to be worse in the morning, nausea and vomiting early in the day, vision
changes like blurry vision that becomes worse when leaning forward, papilledema, cranial
nerve deficits, somnolence, confusion, unsteadiness, and Cushing's reflex (hypertension and
bradycardia). These patient's signs and symptoms require imaging via CT or MRI.

Benign Intracranial Hypertension aka Pseudotumor Cerebri – It is of unknown etiology. It

presents as an acute, subacute or chronic headache with associated blurry vision or visual loss,
papilledema, pulsatile tinnitus, abducens nerve Palsy, nausea and vomiting.The pathology
involves impaired absorption of CSF by the arachnoidvilli. The Patients with IIH are typically
young obese women. Certain medications including vitamin A and oral contraceptive pills can
also lead to IIH.The initial symptom is usually a headache that is pulsatile in nature, awakens
the patient from sleep and is associated with pulsatile tinnitus (i.e .."whooshing" sound in the
ears). The symptoms gradually evolve and patients can develop clinical signs of increased
intracranial pressure (e.g .. blurry vision, nausea, vomiting and transient visual obscurations).
Because of the increased intracranial pressure, patients typically complain that the headaches
become worse with lying flat and better with standing up. Pertinent physical
examinationfindings include visual loss, sluggish pupillary reflexes to light, papilledema on
funduscopy and abducens nerve palsy.

Suspect benign intracranial hypertension (pseudotumor cerebri) in a young obese female with a
headache that is suggestive of a brain tumor, but with normal neuroimaging and elevated CSF
pressure.

Neuroimaging is always performed first to rule out a space-occupying lesion. A percentage of

patients with pseudotumor cerebri have an empty sella seen on neuroimaging which is
probably caused by the downward herniation of arachnocele due to the high cerebrospinal fluid
( CSF) pressure and slit-like ventricles. Magnetic resonance venography is also done to rule out
cerebral venous sinus thrombosis which can present in the same way as IIH.(MRI followed by
MRV).

Once neuroimaging excludes a space-occupying lesion, a lumbar puncture (LP) is indicated.

Papilledema is not a contraindication to an LP unless the patient has evidence of obstructive
or noncommunicating hydrocephalus and/or a space-occupying lesion with/without mass
effect or midline shift. Pseudotumor cerebri causes a communicating hydrocephalus (i.e ..
pressures in the ventricular and subarachnoid spaces are equilibratedwith the lumbar cistern);
therefore an LP is considered safe. In patients with pseudotumor cerebri, the LP reveals normal
CSF findings except for an increased opening pressure (>20 em of water). Sufficient CSF should
be removed during the LP to achieve a closing pressure of <20 em of water. The LP should also
be used to rule out meningitis, encephalitis and subarachnoid hemorrhage. The following
criteria are used for the diagnosis of IIH:

1. Presence of features of increased intracranial pressure in an alert patient

2. Absence of focal neurologic signs except for sixth nerve palsy
3. Normal CSF examination except for increased CSF opening pressure
4. Absence of any ventricular abnormality other than reduction in ventricular size (slit-like
ventricles) and other causes of increased intracranial pressure.

The treatment includes weight reduction and acetazolamide (if weight reduction fails). When
medical measures fail or visual field defects are progressive, shunting or optic nerve sheath
fenestration is done to prevent blindness, which is the most significant complication of this
otherwise benign disorder.Repeated lumbar punctures are usually not helpful in the
management of benign intracranial hypertension.

Pronator Drift - You ask the patient to stretch out his arms with the palms facing up and close
his eyes. Five seconds later you observe the right palm turning inward and downward.

The upper extremity supinator muscles are naturally weaker than the pronators, a difference
that is exaggerated in patients with an upper motor neuron lesion. As a result, when these
patients close their eyesand extend their arms with the palms up, the affected arm will tend to
pronate. However, when people arefeigning upper extremity weakness they typically will drop
the "affected" arm without pronating.

Amaurosisfugax is defined as painless loss of vision from emboli. It looks like a curtain drawing
in front of the eye and occurs intermittently. Cholesterol particles (Hollenhorst bodies) may be
seen in the eye. This event is a warning sign of an impending stroke. An underlying embolic
disease is almost always present. Most emboli occur from the carotid bifurcation; hence, a
duplex ultrasound of the neck is often done to identify any plaques which may be present.

Lacunar Stroke - Lacunar infarcts occur secondary to occlusion of a single, deep penetrating
artery in the brain. The underlying pathology is thought to be a combination of microatheroma
and lipohyalinosis. Lacunar infarcts are rarely embolic. Because of their small size, lacunes are
often not appreciated on non-contrast CT scans obtained during or shortly after the
event.Hypertension and diabetes are major risk factors.The area of ischemic damage in the
brain tissue is relatively small, thus explaining why the neurologic deficit is typically not
prominent.

Always suspect lacunar stroke if a patient presents with a limited neurologic deficit& NO LOSS
OF CORTICAL HIGHER FUNCTIONS!!!.

The most common site for a lacunar infarct is the posterior internal capsule, producing a pure
motor stroke (the posterior internal capsule carries corticospinal and corticobulbar motor
fibers). Other common lacunar stroke syndromes are ataxic hemiparesis, pure sensory stroke,
and mixed sensory-motor stroke.

Know each type well!

A lesion in the posterior limb of the internal capsule is characterized by unilateral motor
hemiparesis of the face, arm and leg without any higher cortical dysfunction and visual field
abnormalities like speech & praxis.

Cortical Lesions –

Right Parietal Cortex Lesion - Patients may shave only the right side of their face.
comb the right side of their hair, and ignore the subject located in the left side of a space.
Anosognosia may also be present. Asking a patient to fill in the numbers of a clock is a typical
test that is used to detect hemi-neglect syndrome. This syndrome is caused by the lesion of the
right (non-dominant) parietal lobe which is responsible for spatial organization

Frontal cortex lesion results in hemiparesis (with motor aphasia if the dominant lobe is
involved).

Left temporal lobe involvement leads to receptive aphasia.

Occipital lobe damage results in visual disturbances.

Anterior cerebral artery occlusion manifests as contralateral weakness that predominantly

affects the lower extremity. Associated findings include abulia, akinetic mutism, emotional
disturbances, deviation of the head and eyes toward the lesion, and sphincter incontinence.

Middle cerebral artery occlusion, especially if the stem of the artery is affected, results in an
extensive infarction with contralateral hemiplegia, conjugate eye deviation toward the side of
the infarct, hemianesthesia, and homonymous hemianopia. There is associated aphasia if the
dominant hemisphere is involved or hemineglect if it involves non-dominant hemisphere
lesions.

Posterior cerebral artery stroke is characterized by homonymous hemianopia, alexia without

agraphia (dominant hemisphere), visual hallucinations (calcarine cortex), sensory symptoms
(thalamus), third nerve palsy with pareses of vertical eye movement, and motor deficits
(cerebral peduncle, midbrain)

Lesions in the vertebrobasilar system that supply the brainstem are characterized by
'alternate' syndromes, with contralateral hemiplegia and ipsilateral cranial nerve
involvement.

Brain Stem Lesions - Brainstem lesions typically involve the cranial nerves and sensory loss of
one half of the face and the contralateral half of the body. Lesions of the thalamus or cortex are
associated with sensory loss of one half of the face and the same half of the body. Cortical
lesions are associated with findings specific to the cerebral cortex (eg, aphasia, neglect,
abnormal graphesthesia or stereognosis).

Medial Medullary Syndrome is typically caused by an occlusion of the vertebral artery or one of
its branches. As a result, the patient will demonstrate contralateral paralysis of the arm and leg,
contralateral loss of tactile, vibratory, and position sense, and tongue deviation to the injured
side.
Lateral Medullary Syndrome – This is also called as Wallenberg syndrome. This syndrome
typically involves an ipsilateralHorner syndrome; loss of pain and temperature sensation of the
face; weakness of the palate, pharynx, and vocal cords; and cerebellar ataxia. There is also loss
of pain and temperature sensation on the contralateral side of the body.

Lateral Pontine Syndrome, which results in impaired sensory and motor function of CN V (the
trigeminal nerve) with accompanying limb ataxia.

Midbrain Strokescan cause a number of different syndromes, including Weber'ssyndrome,

Benedikt's syndrome, Claude's syndrome, Nothnagel's syndrome, and Parinaud's
syndrome.Oculomotor paresis and other abnormalities of CN Ill function are common to all of
these syndromes, andcerebellar ataxia and contralateral hemiplegia may be noted as well.

Multiple Sclerosis - In patients with multiple sclerosis (MS), CSF examination shows normal
pressure, normal total cell count and normal total protein concentration. The predominant
cell type is T lymphocytes. Although the total protein concentration is normal,
immunoglobulin levels are high relative to other protein components. The predominant
immunoglobulin type is lgG although lgM and lgA are also increased. The lgG index measures
the increase in immunoglobulin G level relative to other proteins and is elevated in 90% of
patients with MS. Oligoclonal bands are present in 85 - 90% of cases of MS.

Suspect multiple sclerosis in a young female with bilateral trigeminal neuralgia.

Relapsing-Remitting form of multiple sclerosis (MS) is characterized by well-defined acute

episodes with no significant disease progression between the episodes. Several well-designed
clinical trials have proven that interieron-beta decreases the frequency of relapse and reduces
disability in patients with the relapsing-remitting form of MS.

Acute exacerbations of multiple sclerosis are generally treated with IV steroids. Steroids are
the most widely used drugs in the treatment of acute exacerbations because these can hasten
the recovery of such patients. High-dose intravenous steroids (i.e ..methylprednisone) is used
when patients present with disabling symptoms. Mild sensory symptoms generally do not
require steroids. Long-term steroid therapy provides no benefit, and does not prevent future
relapses. To reduce the frequency of acute exacerbations, interferon,plasmapheresis,
cyclophosphamide, intravenous immunoglobulins and glatiramer acetate may be used.

Cauda Equina Syndrome (CES) – It is a serious disorder of the spinal nerve roots. CES can result
from any disorder that compresses the nerves of the cauda equina. These include
trauma,lumbar disk disease, malignancy and abscesses. The cauda equina is particularly
sensitive to damage because the nerves have a poorly developed epineurium.

Symptoms of CES include low back pain, bowel and bladder dysfunction,saddle
anesthesia,sciatica and lower extremity sensory and motor loss. Exam will revealperineal
anesthesia, poor rectal tone, lower extremity muscle weakness, reflex abnormalities and an
elevatedpost-void residual volume. An emergent MRI of the spine must be obtained when
entertaining a diagnosis ofCES.Cauda equina is an acute compression syndrome presenting with
acute motor and sensory loss, loss of rectal tone and urinary retention. This is a surgical
emergency.

The presence of upper motor neuron findings (such as hyperreflexia, hypertonia,

spasticity and positive Babinski sign) would indicate that the lesion was in the brain or spinal
cord (Cord Compression). CES involves spinal nerve roots which carry lower rather than upper
motor neuron fibers. Hence the Babinski sign is Negative.

Spinal Cord Compression –It’s a tetrad or Lower extremity muscle weakness, Decreased
sensation, hyperreflexia and positive Babinski sign. The presence of upper motor neuron
symptoms indicates involvement of the central nervous system. Because the symptoms are
bilateral and present only in the lower extremities, the lesion can be localized to the spinal
cord. Possible etiologies for this patient's spinal cord compression are disk herniation,
abscessand malignancy. An epidural abscess (even without fever) is of particular concern in this
IV drug abuser. Spinal cord compression is a medical emergency and must be evaluated
immediately with an MRI of the spine.

SCC vs Diabetic Neuropathy - Diabetic neuropathy is a common cause of peripheral sensory

problems in patients with diabetes. The nerves that control proprioception can be involved.
causing a feeling of imbalance. Numbness and tingling are commonly experienced as well.
Physical examination can help confirm a suspicion of diabetic neuropathy. However, these
symptoms can also be seen in cord compression syndrome. Motor symptoms are usually a late
manifestation of severe diabetic neuropathy. So decreased muscle strength in the legs is
somewhat atypical for diabetic neuropathy. Hyperreflexia&Positive Babinski sign suggest an
upper moton neuron lesion (in contrastto diabetic neuropathy. which strictly affects the lower
motor neurons). The Diabetic patient should be questioned about recent fevers as diabetic
patients are prone to development of epidural abscesses. A spine MRI should be performed to
evaluate for cord compression.

Steroid-induced myopathyis the most common cause of drug-induced myopathy and can occur
weeks to months after starting steroid therapy. The mechanism of steroid myopathy is thought
to be due to decreased protein synthesis,Mitochondrial alterations, increased protein
degradation and electrolyte and carbohydrate metabolism disturbances. Acute steroid
myopathy occurs within 1 week of drug initiation (relatively uncommon) and is characterized by
diffuse muscle weakness and rhabdomyolysis. The chronic form is more common and has an
insidious onset after prolonged steroid use. Patients typically present with proximal muscle
weakness (lower extremity before upper extremity) without significant pain and difficulty
getting up from a chair, climbing stairs,or brushing their hair. For both acute and chronic forms,
the diagnosis is clinical with no definitive diagnostic test. Muscle power improves after
discontinuation of the steroids. But the improvement can take weeks tomonths.

Exertional Heat Stroke It occurs in otherwise healthy individuals undergoing conditioning in

extreme heat. Heat stroke exists on the same spectrum as heat exhaustion but is distinguished
by a body temperature >40 .5 C (105 F). The body's main mechanism of heat dissipation is via
the evaporation of sweat. In the case of heat stroke, this thermoregulatory mechanism
becomes overwhelmed and cannot compensate for ambient heat. Other signs and symptoms of
heat stroke may include altered mental status, Headache, dizziness, dry skin, hypotension,
tachycardia and tachypnea. Hyperthermia may also induce systemic damage including
rhabdomyolysis, acute renal failure and DIC. Mortality from heat stroke is approximately 20%.
The treatment of heat stroke involves institution of rapid cooling best achieved by spraying the
naked patient with lukewarm water and running fans to circulate air. This induces evaporation
cooling of the patient.

Non-exertional heat stroke typically affects individuals at the extremes of age incapable of
obtaining adequate fluids and removing themselves from a hot environment.

Brain Strokes–

Hypertension THE BIGGEST risk factor for Brain Strokes - Hypertension has the
strongestassociation with strokes. Hypertension increases the risk of all types of stroke.
Furthermore, multipleobservational studies have demonstrated that patients with hypertension
have approximately four times therisk of stroke when compared to non-hypertensive subjects.

Strokes can be subdivided intoSubarachnoid hemorrhage, ischemic stroke and intracerebral

hemorrhage.

Subarachnoid hemorrhage is usually accompanied by a sudden, dramatic onset of a severe

headache.

Ischemic strokes are usually accompanied by a history of previous transient ischemic attacks.
Patients with ischemic stroke usually don't have headache and impaired consciousness. If there
is evidence of ischemic stroke, carotid Doppler and TEE are performed to evaluate the possible
source of embolism.
Hemorrhagic stroke is characterized by focal neurological signs that develop suddenly and
gradually worsen over minutes or hours. Patients tend to present initially with focal symptoms
but can rapidly progress to signs of elevated intracranial pressure.

In cases of hemorrhage due to Aspirin & Warfarin Ill management, aspirin warfarinand therapy
should be stopped and the patient should be given fresh frozen plasma and vitamin K.

Stroke with slurred speech – Brocas area involved

Stroke with minimal neurological deficits – Lacunar Infarcts.
Stroke with NO loss of cortical Functions – Lacunar Infarcts.
BabinskiSign Positive? – look for a lesion in Brain or Spinal cord
Hypertensive intracerebral hemorrhage generally evolves over a course of minutes to hours.
Patients initially present with focal neurologic symptoms (eg, hemiplegia, hemiparesis,
hemisensory disturbances). These are quickly followed by findings suggestive of increased
intracranial pressure (eg, vomiting, headache, bradycardia, decreased alertness).

In contrast, patients with subarachnoid hemorrhage complain of severe headache atthe onset
and usually do not have focal neurologic findings. Brain computed tomography imaging
confirms intracranial hemorrhage (both intracerebral and subarachnoid) and excludes other
causes.

The onset of symptoms is not abrupt as in subarachnoid hemorrhage or embolic stroke.

Symptoms usually start during normal activity or may be precipitated by sex or strenuous
activity. As the hemorrhage expands, headache, vomiting and altered mental status develop.
Hypertension is the most important risk factor for hemorrhagic stroke.

The first step in all patients suspected with a stroke is CT scan without contrast. This
distinguishes between the three subtypes of stroke. CT scan without contrast can confirm the
presence, size and location of a hemorrhage. Ischemic changes may not appear in the CT scan
in the first 24 hours; however, the absence of a hemorrhage in such cases still makes CT very
valuable for the diagnosis.

Intraparenchymal Hemorrhagic Stroke - Hypertension is the most important risk factor for this
entity. Focal neurological signs suddenly develop and gradually worsen over minutes or hours.
The onset of symptoms is not abrupt as in subarachnoid hemorrhage or embolic stroke.
Symptomsusually start during normal activity or may be precipitated by sex or strenuous
activity. As the hemorrhage expands, headache, vomiting and altered mental status develop.
The most common site of hypertensive hemorrhage is the putamen (35%). The internal
capsule that lies adjacent to the putamen is almost always involved, thereby leading to
hemiparesis. The other neurologicalsigns include hemi-sensory loss, homonymous
hemianopsia, stupor and coma. The eyes are deviated away from the paralytic side.

Cerebellar hemorrhage accounts for 16% of cases of hypertensive intraparenchymal

Hemorrhages. Patients present with ataxia, vomiting, occipital headache, gaze palsy, and
facial weakness. There is NO hemiparesis. Early diagnosis is essential since urgent
decompression may be life-saving. If left untreated, stupor or coma may ensue due to
brainstem compression.

Pontine hemorrhage accounts for 5-12% of cases of hypertensive intraparenchymal

hemorrhages. Patients present with a deep coma and paraplegia that developed within a few
minutes. The pupils are pinpoint and reactive to light. There is decerebrate rigidity. There are
no horizontal eyemovements.
Subarachnoid Hemorrhage Complications- Vasospasm is the major cause of morbidity and
mortality in patients with subarachnoid hemorrhage (SAH). Calcium channel blockers (e.g.,
nimodipine) are used to prevent vasospasm in patients with SAH.

Cerebral salt-wasting syndrome - This may occur in patients with subarachnoid hemorrhage.
The pathology involves:

1.An inappropriate secretion of vasopressin, which causes water retention, and

2.An increased secretion of atrial/brain natriuretic peptide, which causes cerebral salt-
wasting.

These changes result in hyponatremia, which usually resolves within 1-2 weeks. SIADH is also
commonly seen in patients with intracranial hemorrhage. SIADH also results in hyponatremia,
for which water restriction is the initial treatment of choice.

Stroke Management - Stroke is the third most common cause of death in the United States. CT
scan without contrast should be performed in all patients who present with signs and
symptoms of stroke. In the first 24-hours the role of CT scan is essentially to rule out any
hemorrhage in which case aspirin and tPA are contraindicated. If a patient presents within 4.5
hours after the onset of an ischemic stroke, thrombolytic therapy with tissue plasminogen
activator (tPA) should be given. Many trials have demonstrated improved neurological
outcomes with this approach in management.

Antiplatelet therapy with aspirin is indicated in all victims of ischemic stroke who are not
candidates for fibrinolytic therapy. Aspirin has proven benefits for both primary and secondary
strokeprevention. However, it should not be used as a substitute for fibrinolytic therapy as it
does not confer the same benefits for neurologic recovery. In patients who do receive
fibrinolytic therapy, aspirin should be held for 24 hours.

A transient ischemic attack (TIA) is defined by focal neurologic symptoms lasting less than 24
hours due to cerebral ischemia. A head CT should always be obtained in patients with stroke
symptoms to distinguish ischemic from hemorrhagic etiologies. In patients with strokes or TIAs
due to ischemia, if a thrombotic cause is suspected, an antiplatelet agent such as aspirin is used
to prevent future events. Medications such as warfarin are used to prevent future ischemic
events due to cardiogenic emboli.
Although hypertension (HTN) is most common in the early acute period of stroke, aggressive
blood pressure control is not advised as this may impair the cerebral autoregulation and
worsen theneurological outcome. Treatment is indicated when the systolic blood pressure is
greater than 220 mmHg or diastolic blood pressure is greater than 130 mm Hg. HTN should be
reduced over a period of several hours. The preferred drugs are the calcium channel blockers
(e.g ..nicardipine) and ACE inhibitors. Intravenous nitroglycerine and nitroprusside are not
indicated.

The use of streptokinase in stroke patients has not shown any benefits. The drug actually
increases the risk of bleeding. The FDA currently recommends only tPA in the management of
stroke patients.

Intraparenchymal Hemorrhage due to Warfarin - Anticoagulation therapy is the most common

bleeding disorder responsible for brain hemorrhage. For this reason, patients on anticoagulants
(e.g., warfarin) are standardly monitored with regular INR measurement. The risk of bleeding
increases with an increase in the INR. Correction of excess anticoagulation is dependent upon
the INR value and the presence of clinically significant bleeding

Patients with serious intracranial bleeding (such as in this case) should have rapid correction of
excess anticoagulation, with a target INR of less than 1 .5. Fresh-frozen plasma (FFP) reverses
the action of warfarin, works immediately, and lasts for a few hours; therefore, FFP should be
administered emergently.

Broca’s Aphasia - Dominant hemisphere is responsible for speech, language and calculation.
Motor cortex and premotor cortex are present in the frontal lobe and damage to these areas
result in contralateral spastic paresis. Posterior part of dominant frontal lobe contains motor
speech area of Broca and damage to this area results in expressive aphasia in which patient
can't articulate speech or write normally but CAN comprehend written and spoken
language.The patient has word-finding difficulties but intact language comprehension.

Hypokalemia Manifestations - Hypokalemia is a common electrolyte abnormality that causes

weakness. fatigue. and muscle cramps. When severe it can lead to paralysis and arrhythmia.
The ECG may show U waves, flat and broad T waves and premature ventricular beats. Look for
a patient with weakness in all muscle groups who is also taking a potassium wasting diuretic.

Diabetic Neuropathy - Diabetes mellitus causes a number of complications with neuropathy

being very common. Diabetics most commonly have (symmetric) peripheral
neuropathy,mononeuropathy and autonomic neuropathy. Mononeuropathies are divided into
cranial and somatic-among cranial nerves. CN Ill is affected the most often. In diabetes,
oculomotor nerve (CN Ill) neuropathy is ischemic. Somatic and parasympathetic fibers of CN Ill
have separate blood supplies. For this reason, diabetic CN Ill neuropathy affects only somatic
fibers Leaving parasympathetic fibers intact. Symptoms are ptosis and a "down and out" gaze.

Accommodation and the pupil's response to light remain intact. (Alternatively, if nerve
compression causes CN Ill neuropathy,Both somatic and parasympathetic fibers will be
affected. Patients will have ptosis a "down and out" gaze, a fixed, dilated pupil and no
accommodation reflex.) CN Ill (oculomotor nerve) has the following functions:

1. Its somatic component innervates the inferior. superior. and medial rectus. inferior
oblique and levatorpalpebrae muscles. Ptosis occurs due to paralysis of the
levatorpalpebrae; whereas the unopposed action of the lateral rectus ( CN VI) and
superior oblique ( CN IV) muscles lead to a "down and out" gaze.
2. Parasympathetic fibers of CN Ill innervate the sphincter of the iris and the ciliary muscle.
Paralysis of parasympathetic fibers causes a fixed, dilated pupil and causes an inability
of the lens to change shape (loss of accommodation).

7th Cranial Nerve Central vs Peripheral Palsy - The absence of forehead furrows indicates Bell's
palsy (peripheral seventh nerve palsy) and rules out the diagnosis of a central facial paresis.
Patients with central lesions still have forehead furrows because the contralateral motor
innervation of the forehead remains intact.

Gait Types –

High Steppage Gait - Patient have a high-stepping, or "steppage," gait due to right foot drop.
Foot drop results from an inability to dorsiflex the foot. To compensate, patients must overly
flex the hip and knee to bring the foot forward with each step. The toes of the affected foot
may also drag on the ground with this type of gait. Foot drop is most commonly caused by
peripheral neuropathy. Foot drop also classically results from trauma to the common peroneal
nerve or radiculopathy to any of the spinal roots that contribute to the common peroneal nerve
(L4- S2). Finally, foot drop may also be congenital, such as in Charcot-Marie-Tooth disease.

Antalgic Gait - Tarsal tunnel syndrome results from entrapment of the posterior tibial nerve as
it passes underneath the flexor retinaculum on the medial aspect of the ankle. Symptoms
include dysesthesia and numbness plantar foot. Patients with this condition may walk with an
antalgic gait, where the affected foot is favored and as little weight as possible is placed on it.

Broad Based Gait aka Sensory Ataxia – This is seen in Alcoholics, B12 Deficiency & NPH.

In chronic alcoholism, there is cerebellar atrophy and cerebellar damage secondary to chronic
alcohol abuse. The cerebellum helps coordinate movement; thus, people with cerebellar
damage exhibit movement abnormalities. Common signs of cerebellar dysfunction are
ataxia,broad-based gait, dysmetria, intention tremor, difficulty with rapid alternating
movements and nystagmus.

Narrow Based Gait aka Hypokinetic Gait aka Shuffling Gait aka festinating Gait –This is seen
in Parkinson’s Disease. It is characterized by small shuffling steps and a general slowness of
movement.

Ataxic Gait - Cerebellar ataxia is usually ipsilateral (i.e., the patient tends to fall towards the
side of the lesion). Other features of Cerebellar disorders include nystagmus, hypotonia,
dysarthria, loss of coordination, and the inability to perform rapid, alternating movements.

Waddling Gait - Muscular dystrophy results in a waddling gait owing to weakness of the gluteal
muscles. The "waddling" is due to the weakness of the proximal muscles of the pelvic girdle.
The patient uses circumduction to compensate for gluteal weakness.

Spastic Gait – This is seen with lesions of the upper motor neuron (e.g., spinal cord injury or
cerebral palsy). The movements of the affected extremities are slow, stiff, and effortful.

Vestibular ataxia– It results in an en-bloc gait, with minimal movements of the head during
walking. Itis typically a staggering gait, and is accompanied by vertigo and nystagmus.

Dystonic gait– It is characterized by involuntary, sustained, twisting movements of the limbs

and trunk.

Head Aches

Symptoms (e.g., nausea, vomiting, headaches that worsen with changes in position, coughing
and sneezing) and physical findings (e.g .. papilledema) are indicative of increased intracranial
pressure, which is most likely due to a space-occupying lesion.

Migraines are generally unilateral and have a pulsating quality. Attacks can last from 4 to 72
hours. Photophobia is commonly associated. Migraines are more common in younger female
patients. In some cases there is an aura of neurologic symptoms that precedes the onset of the
headache. In cases where the diagnosis is straightforward and there are noconcerning features
such as fever, meningeal signs, or focal neurologic symptoms, further work-up is generally not
required. Nausea can be very severe.Migraine headaches DO NOT cause eye redness or a
dilated, non-reactive pupil& Papilledema.
Intravenous antiemetics (chlorpromazine, prochlorperazine or metoclopramide) can be used
as monotherapy or as adjuvant therapy in combination with NSAIDs or triptans for the
treatment of acute migraine headaches. Prochlorperazine is an effective acute treatment for
migraine and can be given in intravenous form, which is often necessary given these patients'
tendency towards vomiting.

Migraine Treatment – The drug of choice is Triptans. Contraindications of Triptans are as follows:

1.Familial hemiplegic migraine

2. Uncontrolled hypertension
3. Coronary artery disease(make sure patient meets CAD Risk Factors)
4. Prinzmetal angina
5. Pregnancy
6. Ischemic stroke
7. Basilar migraine

Cluster headache is characterized by recurrent episodes of unilateral, periorbital or

retroorbital headache that is stabbing like in quality &accompanied by ipsilateral rhinorrhea,
lacrimation, red eye, and Horner's syndrome. This is the only headache syndrome that is more
common in men than in women. The pathophysiology is related to alterations in the circadian
pacemaker, which may be due to hypothalamic dysfunction. Repetitive episodes of headache
typically occur for weeks to months, followed by a period of remission.The attacks often occur
at the same time of the day, and frequently awaken the patient from sleep. The headache is
always unilateral, sudden in onset, severe, deep, excruciating, and continuous or explosive in
quality. It generally starts as retro-orbital pain and later spreads to the hemicranium. It is NOT
associated with nausea or vomiting, but is frequently associated with ipsilateral lacrimation,
rhinorrhea, red eye, stuffy nose, pallor or even Horner's syndrome (look for Ptosis&Myosis).
Both abortive and prophylactic regimens are needed for an episode of cluster headache. Of all
the regimens available, 100% oxygen is the most rapid-acting and effective in aborting the
pain during an acute attack.Patients DO HAVE a non-reactive, dilated pupil.

Acute angle closure glaucoma occurs when a preexisting narrow anterior chamber angle is
closed in response to pupillary dilation from medications or another stimulus. It is characterized
by the rapid onset of severe pain and vision loss. Patients may complain of seeing halos around
lights. The affected eye will appear injected and the pupil will be dilated and poorly responsive
to light. Tearing may be present and nausea and vomiting may develop as intraocular pressure
increases. A precipitating factor is sometimes identified, such as dim light, an anticholinergic
medication, or a sympathomimetic medication. If untreated, this condition results in severe and
permanent vision loss 2-5 hours after symptom onset. Patients DO HAVE a non-reactive,
dilated right pupil& red eye.The best diagnostic test is tonometry. Drugs that dilate the pupil
can precipitate glaucoma.
Cavernous sinus thrombosis (CST) - OccursBecause thefacial/ophthalmic venous systemis
valveless.Uncontrolled infection of the skin, sinuses and orbit canspread to the cavernous sinus.
Inflammation of the cavernous sinus subsequently results in life-threateningCST and
intracranial hypertension. Headache is the most common symptom and can become
intolerable. Alow-grade fever and periorbital edema usually occur several days later secondary
to impaired venous flow inthe orbital veins. Vomiting is also a result of intracranial
hypertension and fundoscopy may revealpapilledema.

Cranial nerves Ill, IV, V, and V1 pass through the cavernous sinus, which has anastomoses
crossing midline. As a result, unilateral symptoms (eg, headache, binocular palsies, periorbital
edema, hypoesthesia, or hyperesthesia in V1 N2 distribution) can rapidly become bilateral.
Magnetic resonance imaging with magnetic resonance venography is the imaging modality of
choice for diagnosis of CST. Treatment includes broad-spectrum intravenous antibiotics and
prevention or reversal of cerebral herniation.

Orbital cellulitis is often difficult to distinguish from cavernous sinus thrombosis. Fever,
proptosis, ophthalmoplegia, and visual deficits can be seen in orbital cellulitis. Howeve the
presence of headache, bilateral periorbital edema and bilateral cranial nerve
finding(paralysis of bilateral extraocularmuscles ) suggest infectious cavernous sinus
thrombosis.

Brain Abscess – The patient presents with headaches, a focal neurologic deficit, a solitary ring-
enhancing lesion on brain CT scan. The most likely explanation for these findings is
development of a brain abscess. In 25% of cases, brain abscesses arise from distant infections,
usually lung infections (bronchiectasis, empyema, lung abscess), but teeth, heart (endocarditis),
Gl tract (e.g. diverticulitis), and bone infections are also potential causes. In another 25% of
cases, brain abscesses arise due to contiguous spread of infection from the middle ear,
mastoids, or paranasal sinuses. Aerobic and anaerobic streptococci (60-70%) and Bacteroides
species (anaerobes) (20-40%) are the organisms most commonly responsible.

HSV Encephalitis - Herpes encephalitis accounts for 1 0-20% of all viral encephalitis, and is the
most common cause of fatal sporadic encephalitis in the United States. HSV-1 is the etiological
agent in almost all cases of herpes encephalitis beyond the neonatal period. Herpes mainly
affects the temporal lobe of the brain, and presents with an acute onset ( < 1 week duration) of
focal neurological findings such as altered mentation, focal cranial nerve deficits, hemiparesis,
dysphasia,aphasia, ataxia, or focal seizures. Fever is present in approximately 90% of patients.
Behavioral syndromes such as hypomania, Kluver-Bucy syndrome, and amnesia have also been
reported.
CSF analysis, MRI, and EEG are useful for the early diagnosis and therapy of suspected HSV
encephalitis. CSF examination characteristically reveals lymphocytic pleocytosis,
increasednumber of erythrocytes (hemorrhagic destruction of temporal lobes), and elevated
protein levels; low CSF glucose levels are generally not seen. Brain imaging shows temporal
lobe lesions. MRI is generally preferred since it is more sensitive and specific than CT.

Previously, brain biopsy was considered the only definitive test for the diagnosis of herpes
encephalitis; however, polymerase chain reaction (PCR) analysis of HSV DNA in the spinal fluid
has assumed its role as the gold standard, and is currently used to avoid such an invasive
procedure. HSV PCR has an extremely high sensitivity(> 98%) and specificity (94-1 DO%).
Intravenous acyclovir is the treatment of choice.

Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection seen in

immunocompromised patients. It is caused by the JC virus, a human polyomavirus. The exact
mode of transmission is unknown. PML predominantly involves the cortical white matter, but
the brainstem and cerebellum may also be involved. The lesions typically do not produce a
mass effect, and the onset of symptoms is usually gradual. The most common presenting
symptoms are hemiparesis and disturbances in speech, vision and gait. Cranial nerve deficits
may occasionally develop.

An immunocompromised patient with focal neurological deficits should raise the suspicion for
PML, and this diagnosis is best confirmed with MRI. Classic MRI findings in PML consist of
multiple demyelinating, non-enhancing lesions in the WHITE MATTER with no mass effects.
There is no effective treatment for PML, and the mean duration of survival from the time of
diagnosis is six months.

Cerebral toxoplasmosis is the most common ring-enhancing mass lesion in HIV-infected

patients. MRI reveals lesions that are usually multiple, spherical, and located in the
basalganglia. This is unlikely to occur if the patient is receiving trimethoprim-
sulfamethoxazole. A positive Toxoplasmaserology is quite common in normal subjects in the
United States, and is therefore not specific for this condition.

Primary CNS lymphoma is the second most common cause of mass lesions in HIV-infected
patients. It also presents as a ring-enhancing lesion on MRI, but is usually solitary, weakly
enhancing and periventricular. The presence of EBV DNA in CSF is quite specific for the
diagnosis.

In patients with AIDS dementia complex imaging studies reveal cortical and subcortical
atrophyand secondary ventricular enlargement. No focal neurological deficits.
Metoclopramide-induced dystonic reaction - Metoclopramide is a dopamine receptor
antagonist used to treat nausea, vomiting, and gastroparesis. It has significant prokinetic
properties, which include promoting increased peristalsis, increased strength of gastric
contractions, and relaxation of thepyloric sphincter. Common side effects include agitation and
loose stools. Extrapyramidal symptoms (tardivedyskinesia, dystonic reactions and
(Parkinsonism) occur less frequently. Rarely, neuroleptic malignant syndrome may occur.
Treatment for a dystonic reaction involves discontinuation of the medication and
administration of benztropine or diphenhydramine.

Ulnar Nerve Entrapment – Presents with decreased sensations over the 4th and 5th
fingers and weak grip due to involvement of interosseous muscles of the hand are
characteristic. The most common site of ulnar nerve entrapment is the elbow where the
ulnarnerve lies at the medial epicondylar groove. Prolonged, inadvertent compression of the
nerve by leaning on the elbows while working at a desk or table is the typical scenario.

Carpel Tunnel Syndrome - This patient presents with signs and symptoms characteristic for
median nerve involvement numbness and occasional pain in the right palm and
thenareminence atrophy. The most common location of median nerve entrapment is at the
wrist while it passes through carpal tunnel. Carpal tunnel is a fibro-osseous structure formed by
the carpal bones and covered by the transverse carpal ligaments. The median nerve passes
through this tunnel along with the tendons of flexor digitorumsuperficialis.
flexordigitorumprofundus. And flexor pollicislongus muscles. The median nerve is vulnerable to
compression within this channel. Certain conditions predispose to carpal tunnel syndrome (e.g
.. it develops in 7% of patients with hypothyroidism).

Myasthenia Gravis –It is a disease of the neuromuscular junction (NMJ). The clinical
presentation involves fluctuating weakness in voluntary muscles, thereby causing
diplopia,ptosis, and extremity weakness. Extraocular weakness is the presenting symptom in
over half of patients.

There are three treatment options available for the treatment of myasthenia gravis. These
includeacetylcholinesterase inhibitors (anticholinesterases), immunosuppressive agents and
thymectomy.Anticholinesterases provide symptomatic benefit, but do not induce remission.
Immunosuppressive agents and thymectomy may induce remission. The choice of treatment
depends on the patient's age and the clinical scenario.

Oral anticholinesterase (Pyridostigmine or neostigmine) are usually the initial treatment of

choice for myasthenia gravis.
Immunosuppressive agents that are used in the treatment of myasthenia gravis include
prednisone, azathioprine and cyclosporine.

Plasmapheresis removes acetylcholine receptor antibodies from the circulation. It is used in

seriously ill patients when other treatments are not effective or contraindicated. Its effect is
transient and cannot be used on a long-term basis. Other indications of plasmapheresis include
( 1) stabilization of the patient before thymectomy, and (2) myasthenia crisis.

Myasthenia crisis is a life-threatening condition that is characterized by weakness of the

respiratory and pharyngeal muscles. The treatment of all patients includes endotracheal
intubation and withdrawal of anticholinesterases for several days. The most common cause of
myasthenic crisis is an intercurrent infection, and in such cases antibiotics are an important part
of management. All patients with suspected myasthenic crisis should have bedside pulmonary
function tests monitored, such as vital capacity and tidal volume. After intubation,
Plasmapheresis or IVIG should be given. They hasten the recovery of patients with myasthenia
crisis, but these are not the first steps in management.1st step is to intubate which is followed
by Plasmapharesis.

Nonsense and frameshift mutations are typically more severe than missense mutations. Silent
(same sense) mutations do not affect the structure of the protein.

Unprovoked 1st Seizure - Computed tomography (CT) scan of the brain without contrast is the
initial imaging study of choice in patients with unprovoked first seizure to exclude acute
neurologic problems ( eg. intracranial or subarachnoid bleed) that might require urgent
intervention. Magnetic resonance imaging is more sensitive than CT in identifying most
structural causes of epilepsy and is the neuroimaging modality of choice in elective situations.

Pain Management in Opoid Addicts - Patients suffering acute pain should receive the same
standard of pain management regardless of addiction history. Undertreatment of pain is a
common occurrence. It is more likely in patients with current or past drug addiction due to fear
on the part of hospital staff (and even the patient) of exacerbating addiction. However,
undertreatment of pain causes patient dissatisfaction and longer hospitalizations. By increasing
patient anxiety and pain, undertreatment may inevitably lead to higher risk of relapse. The best
option for relief of acute, severe pain is IV morphine,which has a faster onset of action than oral
analgesics. While the use of IV morphine is appropriate in the management of current and
former opioid addicts with acute pain, these situations do present further responsibilities for
the physician. For instance, in current addicts, higher than ordinary doses are needed to
overcome tolerance. In both current and former addicts, outpatient follow-up is appropriate to
monitor pain relief and minimize risk of relapse.Physicians should never undertreat pain, even
if there is a risk of abuse.
Neurofibromatosis - Suspect neurofibromatosis type II in a young patient with acoustic
neuroma and multiple cafe-au-lait spots. MRI with gadolinium enhancement is the best
method to diagnose acoustic neuromas.

Pressure Ulcers - Continued pressure on a bony prominence for a prolonged period of time can
result in ischemic necrosis of overlying muscle, subcutaneous tissue, and skin leading to
formation of a pressure (decubitus) ulcer. Caregivers should reposition vulnerable patients
regularly to reduce the incidence of decubitus ulcers.

Diabetic foot ulcers - They result from chronic unnoticed trauma due to advanced peripheral
neuropathy and poor wound healing from microvascular insufficiency. It is believed that
peripheral neuropathy plays a bigger role in pathogenesis. These wounds occur most
commonly on the soles of the feet over the metatarsal heads and the top of the toes with
Charcot deformity.

Venous stasis ulcers – They occur in the setting of chronic lower extremity edema and occur on
thepretibial area of the lower leg or above the medial malleolus in the ankle. They often begin
insidiously on a background of stasis dermatitis.

Arterial ulcers– They are due to arterial occlusive disease that blocks blood supply and causes
tissuenecrosis. The usually occur in the most distal parts of the body where blood supply is
lowest such as tip of the toes.

Extremity embolization – This presents with a sudden tingling sensation in the involved
extremity. The extremity becomes COLD and there is ABSENT Pulse. Speech and consciousness
are not impaired. Patients characteristically have a normal neurological exam with persistent
signs of ongoing ischemia (e.g., paresthesias, change in temperature, and non-detectable radial
arterial pulsations). Immediate anticoagulationwith heparin and surgical intervention (i.e.,
embolectomy) are crucial, because this condition can lead to tissue death and amputation if not
effectively treated within hours.

Brain Death - Brain death is defined as irreversible loss of function of the whole brain, including
the brain stem. Once a patient has been declared brain dead they are legally dead and artificial
life support does not need to be continued. There are several prerequisites for declaring brain
death. Clinical and/or radiologic evidence of a CNS catastrophe consistent with brain death
must be present, other medical causes that may mimic brain death must be absent, there must
be no evidence of drug or alcohol intoxication or poisoning, and the coretemperature must be
greater than 32•c. Physical exam findings consistent with brain death include an absent corneal
reflex, absent gag reflex, and absent oculovestibular reflex. The pupils will be fixed and
dilated and the patient will not breathe spontaneously when the ventilator is turned off for 10
minutes. Importantly, brain death must be confirmed by at least two physicians. Therefore,
the most appropriate step in this case is to confirm brain death with another physician.
The spinal cord may still be functioning; therefore, deep tendon reflexes may be present. The
heart rate fails to accelerate after atropine injection because vagal control of the heart is lost,
and the heart rate becomes invariant. An apnea test shows no spontaneous respiration at Pco2
values of 50 mmHg and more, thereby demonstrating no functioning of the lower brainstem
centers.

Wernicke &Korsakoff’s Encephalopathy–This occurs in chronic alcoholics due to Thiamine

deficiency. It is a triad of

1. Encephalopathy – Altered Mental Status

2. Oculomotor dysfunction – Nystagmus & Conjugate Gaze Palsy &
3. Gait ataxia – Frequent falls.

This condition may be induced iatrogenically in susceptible patients by administration of

glucose without thiamine. Chronic thiamine deficiency can also cause Korsakoff's syndrome,
characterized by irreversible amnesia, confabulation, and apathy.

Suspect this when you see an alcoholic with a history of frequent falls.

Consider Wernicke's encephalopathy in all patients who present with acute delirium or acute
ataxia, especially malnourished and alcoholic patients. Importantly, all alcoholic and/or
malnourished patients should receive thiamine before any dextrose, as sugars can worsen or
precipitate Wernicke's.

Cerebellar tumor - These typically presents with ipsilateral ataxia, especially if it is

located within the hemisphere. The patienttends to fall towards the side of the lesion. When
asked to stand with his feettogether, the patient also tends to sway to the affected side, and
may exhibittitubation, which is a forward and backward movement of the trunk. Other
characteristic features of cerebellar tumors include nystagmus, intention tremor, ipsilateral
muscular hypotonia, and marked difficulty in coordination and performing rapid, alternating
movements. Obstruction of the CSF flow by the tumor results in increased intracranial pressure,
which may present as headaches, nausea, vomiting and papilledema.

Seizures & Brain Damage - These patients presents with status epilepticus in the setting of
seizure disorder and noncompliance with antiepileptic therapy. Approximately 30% of patients
with epilepsy will have status epilepticus, especially those who are noncompliant with medical
therapy. Status epilepticus has been defined historically as a single seizure lasting >30 minutes.
However, recent studies suggest that a brain that has seized for >5 minutes is at increased risk
of developing permanent injury due to excitatory cytotoxicity.
Cortical laminar necrosis is the hallmark of prolonged seizures and can lead to persistent
neurologic deficits and recurrent seizures. Magnetic resonance imaging of the brain will
generally show evidence of cortical hyperintensity on diffusion-weighted imaging suggesting
infarction. The definition of status epilepticus has therefore been amended to be any single
seizure lasting >5 minutes or a cluster of seizures with the patient not recovering a normal
mental status in between. The exact duration of this patient's seizures is unknown. However,
given that they began at home and continue in the emergency department, it can be assumed
that he has been seizing for >5 minutes, consistent with status epilepticus.

Torticollis is a common form of focal dystonia involving the sternocleidomastoid muscle. a focal
dystonia of the sternocleidomastoid muscle. Dystonia is defined as sustained muscle
contraction resulting in twisting, repetitive movements or abnormal postures. It may befocal
(affecting only one muscle) or diffuse. Torticollis can be congenital, idiopathic, secondary to
trauma or local inflammation or drug-induced. Medications commonly responsible include the
typical antipsychotics. metoclopramide. andprochlorperazine

Eaton-Lambert syndrome is associated with small cell carcinoma of the lung. and results from
autoantibodies directed against the voltage-gated calcium channels in the presynaptic motor
nerve terminal. Look for a patient with Lung Mass + Muscle weakness. Treatment consists of
plasmapheresis and immunosuppressive drug therapy.