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Multiple Cranial

Nerve Palsy Approached


Surat Tanprawate, MD, FRCP(T)
skill
Northern Neuroscience Center
Chiangmai University

Approach
Needed Skills Patient presents with
suspected multiple CN palsy
• Approach skills • Neurological
examination skills

Multiple CN palsy mimickers

Localized and identified cause


of lesion

Multiple Cranial Nerve Palsy


Patient suspected Mimickers
multiple CN palsy
UMN control

• Presentation
• Motor cranial nerve symptoms/signs LMN control

• Sensory symptoms symptoms/signs Cranial nucleus


and nerve

NMJ
Muscle
UMN control of motor
function

Mimicker # 1
UMN Lesion
Corticobulbar pathway

UMN control of
motor function

Homunculus
Latin = "little human",
plural "homunculi"
homo = "human"

Sensory and motor homunculi Photograph taken at the London Science Museum.

Bulbar VS Bulbar Pseudobulbar


Pseudo bulbar palsy Weakness Ipsilateral lesion Contralateral lesion

Special
CN 7 CN 7
weak
Bulbar = Bulb = Medullar
Reflex Hyporeflexia Hyperreflexia

Bulbar palsy = LMN Muscle


Hypotonia, atrophy,
Hypertonia
Cranial nucleus, and nerve fasciculation

Pseudobulbar palsy = UMN Associated


corticospinal Alternating hemiparesis Hemiparesis: same side
Cortex or corticobulbar tract
pathway
Ocular motility

Mimicker # II
Internuclear control of
ocular motility

Internuclear control of horizontal gaze

Mimicker # III
NMJ disorder

Neuromuscular
junction disorder
Clinical clues
• Fluctuation of symptoms
• Distribution of weakness: don’t consistent
with cranial nerve lesion
Mimicker # IV
• Associated with proximal muscle weakness
• Special test for myasthenia gravis Muscle diseases
• ex. enhancing ptosis, sustain upward gaze
test
Muscle diseases
• Opthalmoplegia
• Mitochondrial myopathy(CPEO) Localized Multiple
• Thyroid opthalmopathy Cranial Nerve
• Opthalmoplegia+dysphagia Lesion
• Oculopharyngeal muscular dystrophy
• Ptosis+dysphagia+dysarthria
• Myotonic dystrophy

Cranial Nerve Pathway Intramedullary Long tract signs


•Cranial nuclei Short tract signs
•Cranial fascicle Cranial nucleus involved
Intramedullary
•Cranial nuclei
•Cranial fascicle
Lie on Brain
Adjacent cranial nerve
stem
Extramedullary
Signs of meningeal
Lie on Brain stem Subarachnoid irritation
space Non-pattern distribution
Subarachnoid
Asymmetrical
space
Cranial nerve
Cranial nerve Neighborhood signs
•Sinus
•Sinus
•Fossa, foramen •Fossa, foramen Associated signs

Intramedullary
•Cranial nuclei Brain stem syndrome
•Cranial fascicle

Lie on Brain
stem
CP angle lesion
Brain Stem
Subarachnoid
space
Meningitis Syndrome
Cranial nerve Syndrome of opthalmoplegia
•Sinus Syndrome of Lower CN
•Fossa, foramen involvement
Cranial nucleus in Brain stem

Major Components of Brainstem


1. Cranial nerve nuclei & related structures Isolated brain stem stroke syndrome
2. Long fiber tracts ascending and descending
Rostral VS caudal
3. Cerebellar paths and circuits
Medial VS lateral
4. Reticular formation & related structures
Dorsal VS ventral
Functional Divisions
1. Cranial nerve abnormalities
Combination pattern
2. Long tract deficits (motor & sensory) Medial plus lateral
3. Ataxia (cerebellar origin) Rostral plus caudal
4. Impaired level of consciousness (RF)

!"

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Common Brain Stem
Stroke Syndrome
• Midbrain: Brain stem syndrome of CN III palsy

• Benedict’s syndrome and Weber’s syndrome

• Pontine: Brain stem syndrome of CN VI and VII

• Millard-Gubler syndrome, Foville’s syndrome

• Medullar: Brain stem syndrome of lower CN

• Wallenberg’s syndrome

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Brain Stem
AH#N
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Cerebellopontine
angle(CPA)
• Triangle bounded by

• under surface of the


cerebellar
hemisphere

• lateral aspect of the


pons

Cranial nerve • superior surface of


the inner third of
the petrous ridge

Cerebellopontine
Cerebellopontine angle lesion
angle lesion
• ipsilateral CN VII palsy

•Vestibular
• ipsilateral CN VIII palsy(tinnitus, deafness,
vertigo)
schwannomas(acoustic
neuroma) 85% • eventually pontine compression(nystagmus,
gaze palsy)
•Meningioma
•Primary cholesteatoma • eventually cerebellar hemisphere/peduncle
compression(ipsilateral ataxia)
• May be CN V and VI involvement
Meningitis and Multiple
Meningitis CN involvement

and • Meningeal’s signs positive


• Distribution: asymmetrical and non-
Multiple CN adjacent CN involvement
• May associate with polyradiculopathy
involvement

Cavernous Sinus Cavernous Sinus

• Vertically
• CN III

• CN IV

• V 1 and 2 branch of CN V

• Horizontally
• ICA(and sympathetic
plexus)

• CN VI

Cavernous Sinus Causes of Cavernous


Syndrome Sinus Syndrome
• Inflammatory/ • Non-infectious causes
• Opthalmoplegia(CN III, IV,VI) Infectious causes

• Chemosis • Inflammation: • Tumor


Tolosa-Hunt
• Proptosis syndrome,
• Vascular: C-C
fistular, Cavernous
• Horner’s syndrome Wegener
granulomatosis
sinus aneurysm,
Cavernous sinus
• Trigeminal sensory loss thrombosis
• Infection:
Tolosa-Hunt Syndrome
Cavernous Sinus
Tumors
• Painful ophthalmoplegia caused by nonspecific
inflammation of the cavernous sinus or superior
orbital fissure.
• Primary tumors: Meningioma,
Neurofibroma
• Severe retro-orbital or periorbital pain of acute
• Local spread tumors: Nasopharyngeal,
onset. Diplopia related to ophthalmoparesis
follows the onset of pain Pituitary, craniopharyngioma
• May be visual loss • Metastatic tumors: lung, breast,
prostate
• Paresthesias along first division of the trigeminal
nerve

Superior Orbital Fissure Superior Orbital Fissure


Syndrome Syndrome

Superior Orbital Fissure


Syndrome Gradenigo’s syndrome
• Involve CN 3, 4, 6 and V1 • Diagnostic criteria of Gradenigo's syndrome
CN 5 distribution +/-
oculosympathetic paresis
without anhydrosis
• Suppurative otitis media

• Pain in the distribution of the trigeminal


• May exopthalmos due to nerve
blockade of the opthalmic
veins • Abducens nerve palsy

• Blindness due to • Aetiology of Gradenigo's syndrome


extension of the
pathologic process to
• Apical petrosis secondary to suppurative
otitis media
involve the optic canal
Syndrome of
Extra-axial
Lower CN
involvement

Foramen and
Syndrome Nerve affected Location of lesion
Fossa
Anterior cranial fossa Jugular foramen( Vernet) CN 9, 10, 11 Jugular foramen
Cribiform plate of ethmoid-olfactory
Middle Cranial Fossa Intercondylar Retroparotid space usually;
CN 9, 10, 11, 12
space(Collet-Sicard) lesion may intra or extracranial
Optic foramen-CN2
SOF- CN 2,4,6, V1, SOV Retropharyngeal CN 9, 10, 11, 12 plus sympathetic Retroparotid or
Foramen rotundum-V2 space(Villalet) chain, occasionally CN 7 retropharyngeal space

Foramen ovale-V3 Usually intracranial before


Foramen lacerum-sympathetic plexus nerve fiber leave skull;
Schmidt CN 10,11
occasionally inferior margin of
Foramen spinosum-middle meningeal jugular foramen
a., V. May be
intraparenchyma(medulla);
Posterior Cranial Fossa Jackson CN 10, 11, 12
usually intracranial before
Internal acoustic meatus-CN 7, 8 nerve fiber leave skull
Jugular foramen-CN 9, 10, 11, Tapia
CN 10, 12(occasionally CN 11
Usually high in neck
and sympathetic chain)
Jugular v.
Often infiltrative; arising from
Hypoglossal canal-CN 12 Garcin (hemibase All CN on one side( often
base of skull( esp. NP
syndrome) incomplete)
Foramen magnum-CN 11, spinal cord carcinoma)

Neurological
examination
Neurological Focus neurological exam.
“Test it intensively”
examination General neurological exam.

skill “Test for screening”

General physical exam.


“Test it generally”
Step Examination CN II: Optic Nerve

Vision: Central and


• CN I Peripheral vision
• CN II, III, IV,VI
• CN V,VII,VIII Central Vision

• CN IX, X, XI, XII Visual Acuity: test macular


function

Peripheral Vision
Visual Field: Confrontation test
Test retina function Monocular
confrontation
visual field test

Binocular
confrontation
visual field test

CN III, IV, VI C1*)-.% ='+>(';% 5.)"#2(';% E.'?(';%

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CN V: Trigeminal Nerve

Onion skin distribution of the


trigeminal nerve CN V: Trigeminal Nerve
“Sensory Testing of Face”

CN VII: Facial Nerve CN IX, X


CN XI CN XII

END