Main categories of neurological diseases

• Cerebrovascular diseases
• Trauma
• Neoplastic diseases
• Epilepsy
• Headache
• Infectious diseases
• Inflammatory diseases
• Degenerative neurological diseases
• Developmental diseases
• Spinal cord diseases
• Peripheral nerve diseases
• Muscle & neuromuscular diseases
 6% of the
population had
a neurological
disorder
sometimes
during the life:
3.6 million in
Italy
625
neurological
disorders were
observed per
100,000
population
annually:
375,000 in Italy
MacDonald et al , Brain 2000
 Initial approach to neurological patients
1. Watch the patient as he enter the ambulatory or ER
2. Watch the age and the apparent general condition
3. Watch if he is alone or accompanied by someone
4. Watch how he is wakeful, alert and move
5. Watch if he has any unusual movement or posture
6. Ask for the problem leading the patient to the visit
7. Ask for the duration and progression of symptoms
8. Ask for symptoms that may be associated to patient’s disturbance
9. Ask for antecedent diseases or surgery
10. Ask for currently used therapy
11. Ask for the use of potentially toxic agents or drugs
12. Ask for the sexual attitude of the patient
13. Ask for his work
14. Ask for his mood
15. Ask for previously performed tests, visit or diagnosis
16. Ask for neurological and non-neurological disease in the relatives
Instruments for neurological examination
Electrophysiological abnormalities
 Slowing of nerve conduction

 Conduction block along the nerve

 Denervation

 Reinnervation
Lumbar puncture

Muscle and Nerve Biopsy

Genetic tests
Cerebral angiography
Nuclear Magnetic Resonance
PET for Amyloid in Alzheimer’s disease:
11-C Pittsburgh compound B
Brain tractography
Examination of Cranial Nerves
 Smell: olfactory nerve (I° cranial nerve)
Symptoms:
1. anosmia
2. hyposmia
3. dysosmia
4. cacosmia
Testing:
coffee with closed eyes
Causes of lesions:
 head injury
 chronic basilar meningitis
 brain tumor (frontal menin-
gioma, Foster-Kennedy S.)
Other causes of loss of
smell: cold, allergic rhinitis,
aging, smoking, cockayne
 Vision disturbances

1. Loss of visual acuity

2. Loss of visual field

3. Loss of visual alignment

The visual system: Optic pathways
Visual field examination: confrontational test
Visual charted perimetry
Visual field loss

Left amaurosis

Bitemporal
hemianopia

Right homonymous
hemianopia

Right homonymous
superior
quadrantanopia

Right homonymous
hemianopia with
macular sparing

Scotoma
 Category of
causes of optic
neuropathy
Papilledema Normal

Papillitis Optic atrophy

 Causes of visual
field impairment
Bitemporal Hemianopia:
Pituitary Adenoma
Craniofaringioma
Empty sella
Lateral Homonymous
Hemianopia:
Brain infarct
Brain hemorrhage
Brain trauma
Brain tumor
Demyelinating disease (MS)
 Control of voluntary eye movement
3° (oculomotor nerve), 4° (troclear nerve), 6° (abducens nerve)
 Eye inspection

- Palpebrae rim (ptosis, eyes protrusion, enophtalmos

- Pupils diameter and symmetry
- Ocular alignment
- Conjunctive injection
 Ocular movement
examination

Superior rectus Inferior Superior rectus

oblique

Lateral rectus Medial Lateral rectus

rectus

Inferior rectus Superior Inferior rectus

oblique

+ Accommodation & convergence reflex and pupillary light reflex

Symptoms and signs associated with
ocular malalignment
Strabismus: convergent, divergent
Diplopia: horizontal, vertical
3° Cranial nerve palsy 6° cranial nerve palsy
 Main causes of diplopia
 Oculomotor nerve palsy (3°, 4°, 6°):
 ischemic: diabetes, hypertension: vasculitis (PAN,
temporal arteritis),
 thrombosis/aneurism/fistula/inflammation of cavernous sinus
 Orbital Inflammation (pseutotumor orbitis)
 compression (brain hernia, tumor, cranial hyper/hypotension)
 aneurism of Willis circle or SAE
 infiltration (carcinomatous meningitis, lymphoma)
 inflammatory neuritis (GBS, CIDP)
 Brainstem lesions
 Brainstem Stroke (hemorrhage, infarct)
 Brainstem tumor
 brainstem encephalitis
 Multiple sclerosis
 Muscle and Neuromuscular junction diseases
 Hyperthyroidism, Ocular dystrophy, Myasthenia gravis,
 3° Nerve

4° Nerve
6° Nerve
 Internuclear
Ophtalmoplegia
A lesion in right mlf would take place
here and cause right INO,
Which would be present as righ eye
Most common causes:
not being capable of adduction
And left eye jerk nystagmus

Multiple Scleoris
Brainsten infarct
Trauma, Infection, vasculitis
Intrinsic Ocular Movement

3° Nerve Palsy

Claude Bernard Horner Syndrome

 Vth Cranial Nerve:
trigeminal nerve

+ Corneal Reflex
 Trigeninal Neuralgia
Definition of the International Association for
the Study of Pain:
a sudden, usually unilateral, severe brief
stabbing recurrent pain in the distribution of
one or more branches of the fifth cranial nerve

Diagnostic criteria of International Headache Society

A Paroxysmal attacks of pain lasting from a fraction of a second to
two minutes, affecting one or more divisions of the trigeminal
nerve and fulfilling criteria B and C;
B Pain has at least one of the following characteristics:
(1) Intense, sharp, superficial or stabbing;
(2) Precipitated from trigger areas or by trigger factors;
C Attacks are stereotyped in the individual patient;
D There is no clinically evident neurological deficit;
E Not attributed to another disorder.
Causes of trigeminal neuralgia

1) Compression of the trigeminal

nerve root at or near the dorsal
root entry zone by a blood vessel
(conflitto neurovascolare)
2) Infection by Herpes Zoster (Zoster The pain of
ophtalmicus) idiopathic TN is
3) Multiple sclerosis indistinguishable
4) Infiltration or compression of the nerve from that caused by
root, trigeminal ganglion or nerve by a a demonstrable
tumour or amyloid, structural lesion
other than vascular
5) Small infarcts or angiomas in the pons or
medulla compression, ma
non si associa a
6) Undetermined deficit oggettivi
Therapy of of trigeminal neuralgia
1) Carbamazepine superior to placebo
in 4 RCT studies. A Cochrane review
deduced a number needed to treat of
2.5. CBZ lost effect or was poorly
tolerated in half the patients after 10
years
2) Lamotrigine slightly more effective Carbamazepine
than placebo as add on therapy to remains the most
CBZ or DHT in one randomized effective therapy
controlled trial. for trigeminal
neuralgia
3) Clonazepam, gabapentin, phenytoin,
oxcarbazepine & sodium valproate: Wiffen et al, 2007
little evidence supports their Cochrane Review
beneficial role in TN.
7th° Cranial nerve: facial nerve
Facial Nerve examination
 Causes of peripheral nerve
facial palsy
1)Idiopathic palsy (Bell’s palsy,
frigor palsy)
2) Infection: Herpes Zoster (zona
genicolatus, Ramsay Hunt syndrome:
vesicles meatus auricularis)
Causes of Bell’s
3) Acoustic Neurinoma or other palsy:
pontocerebellar angle tumors (+
vertigo, tinnitus, hearing loss) Nerve Inflammation/
infection (HSV, VZV,
4) Brainstem lesions (Millard-Gubler Influenza B, echovirus,
syndrome: + 6° ipsilateral palsy and Borrelia, HIV, ??)
opposite hemiparesis): ischemic, causing nerve oedema
hemorrhage, inflammation, tumor) and compression within
the facial channel
Treatment started within 72 hrs from onset x 10 days
Prednisolone: 25 mg x 2/die; Acyclovir: 400 mg x5/die

551 pazienti
Hearing: 8th nerve: cochleovestibular nerve
Symptoms & Signs
1. Hypoacusia (reduced
hearing)
2. Anacusia (deafness)
3. Tinnitus (disturbing noise
like whistle, buzzing,
pulsing)
Hearing 8th nerve: cochleovestibular nerve
Test
1. Test of Rinne (mast-meato)
2. Test of Weber (vertex)

3. Audiogram
Equilibrium: 8th nerve: cochleovestibular nerve
Symptoms and Signs
1. Vertigo (rotation sense)
2. Nausea & vomit
3. Nystagmus
4. Loss of equilibrium
5. Trunc deviation
6. Gait (& tandem) deviation
7. Lateralized Romberg
8. Index deviation

9. Otovestibular test
 Nystagmus:
Involuntary to-and-fro oscillations of the eyes with
a slow (pathological) and a fast (recovery) phase
(jerky nystagmus), or pendular

Orizzontal Rotatory
Vertical

•1° when present in the direction of the fast phase

•2° when present with the eyes in primary position
•3° when present in the direction of the slowt phase
The direction of nystagmus is that of the fast phase
Main causes of 8th cranial nerve lesion
• Cerebellopontine angle tumor
(Acoustic neuroma, dermoid
cistis, meningioma)
• Pontomedullary lesion
(multiple sclerosis)
9° cranial nerve: glossofaringeal 10° cranial nerve: vagus

Brachial motor, sensory, visceral motor

IX° (glossofaringeal) & X° (vagus) cranial nerves:
1. Dysathria: V°, VII° (labials), IX° e X° (gutturals), XII°
(lingual), V°, VII°, IX°, X° (sibilants), V, XII (dental/
fricatives)
2. Dysphagia: V°-mastication, VII°-mouth closure, IX°,
X°, XII°-pulling bolus back
3. Dysphonia: X°
4. Dysgeusia: VII°, IX°, X°
1. Voluntary
orofaringeal motility
2. Reflex orofaringeal
motility (gag reflex)
Anatomy of taste/gustation:
VII°, IX°, X° cranial nerve
1. Ageusy-Ipogeusy
2. Disgeusy-parageusy
3. Basic taste:
1. Sweet: sugar
2. Bitter: coffee
3. Salty: salt
4. Sour: lemon
5. Umami: glutammate
6. Grease
11° cranial nerve: spinal accessory
XIIth cranial nerve: ipoglossal nerve

Symptoms & signs: dys-

lalia, dysartria, dysphagia,
atrophy, fasciculation
Causes: vasculitis, tumor,
ALS, motor neuropathy
Motor pathways

Pyramidal system
 Motor Examination:
Symptoms:
1. Weakness
2. Fatigue & and fatigability
3. Wasting
4. Cramps
5. Tremor
6. Muscle twitching
7. Involuntary movement
Inspection:
1. Patient’s posture (head & arms)
2. Walking and standing
3. Trophy: hypo/hypertrophy
4. Involuntary movements: Tremor,
fasciculation, cramps, clones, chorea,
athetosis, hemiballism, tics, dystonia &
dyskinesia
Motor Examination:
Testing:
1. Strength: anti-gravity and
segmental strength: paresis,
paralysis/ plegia
2. Muscle tone: hypo tonus,
hyper tonus/ spasticity;
3. Deep tendon reflexes (DTR):
hypo-hyper- areflexia
4. Pyramidal signs: Babinski,
Rossolimo, Hoffmann,
clonus, abdominal reflexes
Distribution:
1. Emi-, para-, diparesis/plegia,
2. stock and glove weakness
3. Proximal weakness
 Movement disorders:
Involuntary Movements:
• Tremor: voluntary, resting,
. action/intentional
• Fasciculation, cramps,
(fibrillation)
• Clones
• Chorea
• Athetosis
• Hemiballism
• Tics
• Dystonia e dyskinesia
Bradi-, a-, hyper-kinesia
 Ataxia: disturbance of
coordination and equilibrium
Vestibular, cerebellar, sensory ataxia
Standing ataxia Walking ataxia

Dismetry

Romberg sign
Sensory pathways
 Temperature

touch in movement

Vibration/touch fast
pain/Temp. Stable pressure
Slow deformation/touch
prolonged
 Sensory disturbances
Positive symptoms: Paraesthesias (tingling, burning,
pressure, coldness, tightening…), pain,
dysesthesia, painful hyperesthesia,
allodynia, numbness
Negative symptoms: Hypo-/anesthesia (superficial, deep,
pain, temperature, ataxia)
 Symptoms and signs of
meningeal involvement
Symptoms:
• Acute headache frontoccipital,
fotofobia, nausea, vomit, impaired
consciousness -> coma
Signs: Rigor
• Nucal rigidity, Brudzinski and Kernig
signs
Brudzinski Kernig
Disturbance of high cognitive functions:
memory -> amnesia
ST orientation -> disoriented
judgment -> hypo -a criticism
language -> aphasia
use of movement -> apraxia
identification -> agnosia
calculation -> acalculia
writing -> agraphia
lecture -> alexia
 Mechanism of speech

Respiration: Lung in expiration

Phonation: larynx -> dysphonia
Articulation: oropharyngeal cave ->
dysartria
Formulation and comprehension: brain
-> aphasia
 Aphasia:
Acquired disturbance of the language including expression,
comprehension, repetition , reading and writing
 Main speech abnormalities
anomies
verbal circumlocution
paraphasias semantic, verbal and
phonemic
agrammatical
neologisms
neologistic speech
 Classification of Aphasia
•1) Aphasia non fluent:
– Aphasia Broca
– Aphasia global
– Aphasia transcortical motor
•2) Aphasia fluent
– Aphasia Wernicke
– Aphasia conduction
– Aphasia transcortical sensory
– Aphasia amnestic
•3) Pure Forms
– Anartria
– Pure word deafness +/- auditory agnosia
– Alexias e agraphy
 Apraxia
Disturbance of motor function due to the incapacity to use the
movement for an intentional purpose in the absence of
palsy, tremor ataxia, akynesia or movement disorders
Types of Apraxias
Limb apraxia (left hemisphere)
– Ideational (unable to make a motor project that may lead
to an action: how to prepare a sandwich or make pasta)
– Conceptual (unable to think how to use instrument for a
purposeful movement, what to use as a screwdriver)
– Ideomotor (unable to move the arm to make an
intentional movement like how to use a comb, scissor -
transitive-, or to make and hand gesture, intransitive)
– Kinetic (loss of digital dexterity: torn coins,show fingers)
Orofacial apraxia (left emisphere, frontal lobe):
Constructional apraxia (right hemisphere, parietal lobe)
Dressing apraxia (right hemisphere, parietal lobe)
 Agnosia
Definition:
Disturbance in the ability to identify familial stimuli and to
recognize them not due to a loss of sensation, attention or
alertness
Types of Agnosia
Visual agnosia
– aperceptional: inability to identify the object
– associative: inability to recognize the objects
– prosopagnosia (face)
Auditory agnosia
Tactile agnosia:
- anilognosia (materials)
- astereo(morfo)gnosia (shape)
Disturbance of body scheme or body
representation in the space
Definition:
Disturbance in the capacity of recognizing own body, to
localize it in the space and to explore the external space
that is not caused by a sensorial loss
Digital agnosia
Allokiria
Emisomatagnosia
Anosognosia
Hemineglect
Disturbance of memory (amnesia)
Definition: disturbance in the capacity of
registering, fixing and evoke memory
Areas and type of Memory:

1) episodic 2) semantic; 3) procedural

4) working (attention, concentration):
a) phonologic
b) spatial
 Memory disturbance (amnesia)

Types of Amnesia

• Anterograde or retrograde amnesia

• Transient global amnesia
• Benign amnesia in the elderly
• Amnesia in cognitive impairment
• Post-traumatic or post-critical amnesia
 Mini Mental State Examination
1) Orientamento
I. Temporale: Anno, stagione, mese, giorno settimana, data: 5
II. Spaziale: stato, regione, città, luogo, piano: 5
2) Memoria:
I. Casa, pane gatto: 3
3) Attenzione Calcolo
I. 100-7-7-7-7-7 5
II. Oppure: Carne al contrario (ENRAC): (5)
4) Rievocazione:
I. Casa, pane gatto 3
5) Linguaggio:
I. Orologio e penna 2
II. Non c’è né se nè ma che tenga 1
III. Ordine in tre tempi 3
IV. Ordine scritto 1
V. Scrivere una frase 1
6) Prassia Costruttiva
I. Copiare un disegno /(due pentagoni intersecati) 1
TOTALE …/30
 Sindrome del lobo frontale
• Emiplegia controlaterale
• Segni di liberazione corticale (grasping, suzione)
• Afasia non fluente (emisfero sinistro)
• Aprassia motoria e buccofacciale (emisfero sinistro)
• Alterazioni cognitive, di personalità e comporta-
mento legate alla funzione di supervisione ed avvio
delle attività cerebrali:
– Perdita di iniziativa e spontaneità, indifferenza, apatia, abulia
– Perdita di autocontrollo, inappropriato, disinibito
– Perdita della capacità di programmazione
– Perdita di attenzione e concentrazione con labilità di pensiero
. ma anche rigidità mentale e vischiosità di pensiero con
. impersistenza ma anche perseverazioni
Brain tractography