Multiple Cranial Nerve Palsy Surat Tanprawate Tanprawate,, MD, FRCP(T) Northern Neuroscience Center Chiangmai University
Needed Skills • Approach skills
• Neurological
Approached skill
Approach Patient presents with suspected multiple CN palsy
examination skills Multiple CN palsy mimickers
Localized and identified cause of lesion
Patient suspected multi le CN als
• Presentation • Motor cranial nerve symptoms/signs • Sensory symptoms symptoms/signs
Multiple Cranial Nerve Palsy Mimickers UMN control
LMN control 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 Pseudo bulbar palsy
Bulbar
Pseudobulbar
Weakness
Ipsilateral lesion
Contralateral lesion
Special weak
CN 7
CN 7
Reflex
Hyporeflexia
Hyperreflexia
Muscle
Hypotonia, atrophy, fasciculation
Hypertonia
Associated corticospinal tract
Alter lterna nati ting ng hem hemipar ipares esis is
Hem Hemipar ipares esis is:: same same sid side
Bulbar = Bulb = Medullar
Bulbar palsy = LMN Cranial nucleus, and nerve Pseudobulbar palsy = UMN Cortex or corticobulbar 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
Mimicker # IV
• Associated with proximal muscle weakness • Special test for myasthenia gravis • ex. enhancing ptosis, sustain upward gaze
Muscle diseases
with cranial nerve lesion
test
Muscle diseases • Opthalmoplegia myopathy(CPEO) • Mitochondrial myopathy(CPEO) • Thyroid opthalmopathy • Opthalmoplegia+dysphagia • Oculopharyngeal muscular dystrophy • Ptosis+dysphagia+dysarthria • Myotonic dystrophy
Cranial Nerve Pathway Intramedullary •Cranial nuclei •Cranial fascicle
Localized Multiple Cranial Nerve Lesion
Intramedullary •Cranial nuclei •Cranial fascicle
Long tract signs Short tract signs Cranial nucleus involved
Lie on Brain stem
Adjacent cranial nerve
Subarachnoid space
Signs of meningeal irritation Non-pattern distribution Asymmetrical
Extramedullary Lie on Brain stem Subarachnoid space
Cranial nerve •Sinus •Fossa, foramen
Cranial nerve •Sinus •Fossa, foramen
Intramedullary •Cranial nuclei •Cranial fascicle
Brain stem syndrome
Lie on Brain stem
CP angle lesion
Subarachnoid space Cranial nerve •Sinus •Fossa, foramen
Meningitis
Syndrome of opthalmoplegia Syndrome of Lower CN involvement
Neighborhood signs Associated signs
Brain Stem Syndrome
Cranial nucleus in Brain stem
Major Components of Brainstem 1. Cranial nerve nuclei & related structures 2. Long fiber tracts ascending and descending 3. Cerebellar paths and circuits 4. Reticular formation & related structures
Isolated brain stem stroke syndrome syndrome Rostral VS caudal Medial VS VS lateral Dorsal VS ventral
Functional Divisions 1. Cranial nerve abnormalities 2. Long tract deficits (motor & sensory) 3. Ataxia (cerebellar origin) 4. Impaired level of consciousness (RF)
Combination pattern Medial plus lateral Rostral plus caudal !"
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Common Brain Stem Stroke Syndrome •
Midbrain: Brain stem syndrome of CN III palsy
• •
Pontine: Brain stem syndrome of CN VI and VII
• •
Benedict’s syndrome and Weber’s syndrome
Millard-Gubler syndrome, Foville’s syndrome
Medullar: Brain stem syndrome of lower CN
•
Wallenberg’s syndrome
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Cerebellopontine angle(CPA) •
Cranial nerve
Cerebellopontine angle lesion •Vestibular schwannomas(acoustic neuroma) 85% •Meningioma •Primary cholesteatoma
Triangle bounded by
•
under surface of the cerebellar hemisphere
•
lateral aspect of the pons
•
superior surface of the inner third of the petrous ridge
Cerebellopontine angle lesion • •
ipsilateral CN VII palsy
•
eventually pontine compression(nystagmus, gaze palsy)
•
eventually cerebellar hemisphere/peduncle compression(ipsilateral ataxia)
•
May be CN V and VI involvement
ipsilateral CN VIII palsy(tinnitus, deafness, vertigo)
Meningitis and Multiple CN involvement
Meningitis
• Meningeal’s signs positive • Distribution: asymmetrical and non-
and Multiple CN involvement
adjacent CN involvement
• May associate with polyradiculopathy
Cavernous Sinus
Cavernous Sinus •
Vertically
• • •
•
Cavernous Sinus Syndrome Opthal moplegia(CN ia(CN III, IV, IV, VI) • Opthalmopleg • Chemosis • Proptosis Horner’s syndrome • Horner’s • Trigeminal sensor y loss
CN III CN IV V 1 and 2 branch of CN V
Horizontally
•
ICA(and sympathetic plexus)
•
CN VI
Causes of Cavernous Sinus Syndrome
• Inflammatory/
Infectious causes
• Inflammation:
Tolosa-Hunt syndrome, Wegener granulomatosis
• Infection:
• Non-infectious causes • Tumor • Vascular: C-C fistular, Cavernous sinus aneurysm, Cavernous sinus thrombosis
Cavernous Sinus Tumors
Tolosa-Hunt Syndrome • • • •
Painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure. Severe Severe retro-orbital or periorbital pain of acute onset. Diplopia related to ophthalmoparesis follows the onset of pain May be visual loss
Meningioma, • Primary tumors: Meningioma, Neurofibroma
Nasopharyngeal, • Local spread tumors: Nasopharyngeal, Pituitary, craniopharyngioma
• Metastatic tumors: lung, breast, prostate
Paresthesias Paresthesias along first division of the trigeminal nerve
Superior Orbital Fissure Syndrome
Superior Orbital Fissure Syndrome • • •
Involve CN 3, 4, 6 and V1 CN 5 distribution +/oculosympathetic paresis without anhydrosis
Superior Orbital Fissure Syndrome
Gradenigo’s syndrome •
• •
May exopthalmos due to blockade of the opthalmic veins Blindness due to extension of the pathologic process to involve the optic canal
Diagnostic criteria of Gradenigo's syndrome
• •
Suppurative otitis media Pain in the distribution of the trigeminal nerve Abducens nerve palsy
Aetiology of Gradenigo's syndrome
•
Apical petrosis secondary to suppurative otitis media
Syndrome of
Extra-axial
Lower CN involvement
Foramen and Fossa Anterior cranial fossa
Syndrome
Nerve affected
Location of lesion
Jugular foramen( Vernet)
CN 9, 10, 11
Jugular foramen
Intercondylar space(Collet-Sicard)
CN 9, 10, 11, 12
Retroparotid space usually; lesion may intra or extracranial
Retropharyngeal space(Villalet)
CN 9, 10, 11, 12 plus sympathetic Retroparotid or chain, occasionally CN 7 retropharyngeal space
Schmidt
CN 10,11
Usually intracranial before nerve fiber leave skull; occasionally inferior margin of jugular foramen
Jackson
CN 10, 11, 12
May be intraparenchyma(medulla); usually intracranial before nerve fiber leave skull
Tapia
CN 10, 12(occasionally CN 11 and sympathetic chain)
Usually high in neck
Garcin (hemibase syndrome)
All CN on one side( often incomplete)
Often infiltrative; arising from base of skull( esp. NP carcinoma)
Cribiform plate of ethmoid-olfactory Middle Cranial Fossa
Optic foramen-CN2 SOF- CN 2,4,6, V1, SOV Foramen rotundum-V2 Foramen ovale-V3 Foramen lacerum-sympathetic plexus Foramen spinosum-middle meningeal a., V. Posterior Cranial Fossa
Internal acoustic meatus-CN 7, 8 Jugular foramen-CN 9, 10, 11, Jugular v. Hypoglossal canal-CN 12 Foramen magnum-CN 11, spinal cord
Neurological examination skill
Neurological examination Focus neurological exam. “Test it intensively” General neurological exam. “Test for screening” General physical exam. “Test it generally”
Step Examination
• • CN II, III, IV, VI VI I, VIII VIII • CN V,VII, • CN IX, X, XI, XII CN I
Peripheral Vision Visual Field: Confrontation Confrontation test Test retina function
CN II: Optic Nerve Vision: Central and Peripheral vision
Central Vision Visual Acuity: test macular function
Monocular confrontation visual field test
Binocular confrontation visual field test
CN III, IV, VI
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CN V: Trigeminal Nerve
Onion skin distribution of the trigeminal nerve
CN VII: Facial Nerve
CN V: Trigeminal Nerve “Sensory Testing Testing of Face”
CN IX, X
CN XI
END
CN XII
