diseases of cranial nerves 5th, 7th and 8th

PRESENTER

DR. A T M HASIBUL HASANMD (Neurology) Thesis part studentDepartment of Neurology

CRANIAL NERVES (V, VII, VIII)

Applied

TOPICS FOR DISCUSSION

• Case stories

• Radiological anatomy of cranial nerves

• Individual cranial nerve pathology

• Some cranial neuropathies

Case-1

• A 45 year old professor, was experiencing sudden severe episodes of brief pain involving left side of her face and was shooting through her jaw. Pain was elicited mostly on brushing, talking or while taking food.

• Her dentist was unable to give any relief even after extracting several teeth.

• She was later consulted and treated by a neurologist and after some investigations was referred to a neurosurgeon.

Case-2

• On a fine morning a 40 year old marketing manager had difficulties in shaving his left side of the face. Due to unusual dryness in mouth he ordered soup at lunch. But the soup was dribbling out the left corner of his mouth.

• At the doctors office, he was unable to close his left eye completely and the angel of the mouth was deviating to the right with smile.

• Six weeks later he noticed that every time he smiled his left eye would get closed.

Case-3

• A 34 year old call centre worker, noticed progressive difficulty in hearing on his right ear and occasionally with buzzing in the same ear. He later experienced waves of vertiginous episode with loss of balance during walking.

• His physician noticed abnormal eye movement during examination and the tuning fork was ringing louder on the left ear.

RADIOLOGIC ANATOMY

CRANIAL NERVES:- V, VII, VIII

Trigeminal

MRI- FIESTA sequence

CN- VII & VIII

MRI- FIESTA sequence

CN-VIII

INDIVIDUAL CRANIAL NERVE PATHOLOGY

Evaluation of CN-V

Sensory- Somatic sensation from face Reflex- Corneal reflex and Jaw jerk. Motor-Masseter, temporalis and

pterigoid

Pattern of Sensory loss

Reflex

Corneal Reflex Jaw Jerk

Jaw Jerk

• Brisk:o Pseudobulbar palsy:

MNDBilateral strokeMS

• Absent:o Bulbar palsy:

MNDPost viral

Motor examination

• Inspection:

• Palpation:

• Motor movement:

Course of Trigeminal nerve

Lesions of Trigeminal Nerve

Level Feature CauseSupra nuclear o Unilateral UMN lesion: Jaw deviates

to opposite sideo Bilateral UMN lesion: Spastic

masticatory paresis

o Vascular

Nuclear or fascicular

o Midpontine syndromes: • Ipsilateral: Weakness & atrophy of muscles of

mastication Hemianesthesia in face Horner syndrome INO• Contralateral: Hemi sensory loss

o Ischemia/ vascular lesiono Demyelinating lesiono Inflammatory lesiono Neoplastic lesiono Cavernous malformationo Syringobulbia

o Lateral medulla: Spinal tract and nucleus of CN V• Ipsilateral: Hemianesthesia in face• Contralateral: Hemi sensory losso Lower medulla: Onion skin pattern of sensory loss

Level Feature Cause

Subarachnoid space

o Preganglionic trigeminal nerve: • Ipsilateral: Weakness & atrophy of muscles of

mastication Hemianesthesia in face Loss of corneal reflex VII & VIIIth CN: CP angel tumor

o Cerebellopontine angel tumor

o Inf/Inflammatory lesiono Neurovascular

compressiono Trauma

Petrous apex &

Meckels cave

o Severe hemifacial pain o Infectiono Neoplastic lesiono Inflammatory lesiono Trauma

o Reader Paratrigeminal Syndrome: • Horner syndrome • Trigeminal neuralgia

o Parasellar: Tumor, aneurysm, trauma,

infection

o Gradenigo Syndrome:• Retro orbital pain• Dipplopia• Otorrhea• Ipsilateral facial pain/numbness• Ipsilateral lateral rectus palsy

o CSOMo Mastoiditiso Skull base osteomyelitis

o Herpes Zoster • Eruption in skin along distribution of CN V

o Varicella Zoster

Level Feature CauseCavernous sinus/ superior orbital fissure

o Cavernous sinus syndrome:Dysfunction of CN III, IV, VI, V1, V2: • Ipsilateral: Ophthalmoplegia Pain and sensory loss in V1, V2 Horner syndrome

o Inf/Inflammatory lesiono Neoplasmo Vascularo Radiation injury

o Superior orbital fissure syndrome: • All above plus proptosis but without V2

Distal trigeminal lesion

o Numb cheek syndrome: • Infraorbital nerveo Numb chin syndrome:o Inferior alveolar nerve

o Traumao Neoplasmo Inflammation

A 45 years old professor presented with lancinating pain in left side of face

Trigeminal Neuralgia

Trigeminal Neuralgia:

• Can occur at any age.

• More common in female.

Aetiology:• Mostly idiopathic:

may have vascular compression• Secondary causes includes-

1. Cholesteatoma2. Brain stem eg, tumor, demyelination3. Perineural spread of tumor4. Injury during surgery

Facts about TN

Risk Factors:o Age: >50yro Female gendero Family historyo MS

Trigger factors:o Talkingo Chewingo Eatingo Brushingo Washing faceo Shaving

Factors decreasing pain threshold:o Stresso Inadequate sleepo Exposure to coldo Hyperglycemiao Drugso Menstruation

Trigger zone

Idiopathic TN

Secondary TN

Difference

Trait Idiopathic TN Symptomatic TN

Age 52-58 year 30-35 year

Pain Paroxysmal May be persistent

Sensory involvement None Present

Focal neurological deficit

None Present

Cause Idiopathic, may have vascular compression

Tumor, MS

Response to treatment Good Poor

Prognosis Better Worse

Treatment of TN

Medical-o Carbamazepine: • 100 mg/day to 1200 mg/day• Effictive in 50-70%

o Oxcarbazepine (300-1200 mg/day)o Lamotrigine (400mg)o Phenytoin. Surgical-o Microvascular decompression:• Success rate is about 90%• About 12% recur in 2 yr• Mortality 1%

o Gamma knife radio-surgery:• Less effective• Less chance of complication

Microvascular decompression

Herpes zoster ophthalmicus

Reactivation of VZ from trigeminal ganglion

Unilateral vesicular eruption

Hutchinson sign-skin lesions at side of nose (precedes opthalmic involvement).

Rx- • Oral acyclovir

800mg 5 times for 7 days.

• Prednisolon• Eye care

A 15 yr old boy presented with right facial swelling, proptosis and complete opthalmoplegia and loss of sensation from upper part of face (rt) following tooth extraction.

Cavernous sinus thrombosis

Evaluation of Facial Nerve

• Inspection:

• Motor function:

• Taste sensation:

• Hearing:

Special situation

Cause of facial paresis

Unilateral Bilateral

UMN type Stroke

Demyelinating lesion

Tumor

LMN type Bell’s palsy GBS

CSOM Sarcoidosis

Post traumatic DM

Parotid tumor, surgery Lyme disease

Ramsay Hunt Syndrome HIV

CP angel tumor Billateral Bell’s palsy

Brain stem stroke (Millard- Gubler) CTD

Amyloidosis

Mobius syndrome

NMJ disease- MG

Myopathy- FSH MD

Lesions of Facial Nerve

Level Feature Cause

Supra nuclear o Unilateral UMN lesion: involve lower part of face on opposite side

o Vascular

Nuclear or fascicular

o Diagnostic clue: Accompanying brain stem sign eg other CN

palsy + crossed hemiplegiao Foville syndrome: Dorsal pons• Ipsilateral: Lateral gaze palsy Facial palsy Eight and half syndrome/ INO Horner syndrome• Contralateral: Ataxiao Millard-Gubler syndrome: Ventral pons• Ipsilateral: Facial palsy ± Lateral gaze palsy• Contralateral: Hemiplegia

o Ischemia/ vascular lesion

o Demyelinating lesiono Inflammatory lesiono Neoplastic lesiono Syringobulbia

Lesions of Facial Nerve

Level Feature CauseC-P angel o Diagnostic clue:

• Ipsilateral: Facial palsy Loss of taste sensation (Ant 2/3rd of tongue) Hyperacusis Tinnitus Hearing loss ± vertigo± CN V, VIo Hemifacial spasm: Neurovascular compression of motor rooto Geniculate neuralgia/ Hunt neuralgia:Neuralgia affecting nervous intermedious Paroxysmal otalgia AICA compression Rx- CBZ, decompression

o Acoustic neuromao Meningioma

Peripheral lesion

o Bell’s palsyo Ramsay Hunt syndrome: Herpes zoster

oticuso Mobius syndrome: Congenital bilateral CN VI+ VII palsyo Post traumatic facial palsy:

o Idiopathico Malignancyo Infectiono Inflammationo Iatrogenic

Brain stem lesion

A 40 yr old man presented with sudden deviation of angel of mouth to right and inability to close left eye lid

Bell’s Palsy

Bell’s Palsy

• One of the commonest mononeuropathy

• Self limited, monophasic illness

• Onset: Acute/ subacute

• C/F: LMN type facial palsy

• Associated features: Mastoid pain Impaired taste and salivation Impaired lacrimation Hyperacusis

Treatment

oSteroid (prednisolone 60-80 mg first 5 days, tapered over next 5 daysoAntivirals- Aciclovir 400 mg 5 times for 10 daysoEye careoPhysiotherapy

Prognosis

• Recovery: 85% within 3 weeks.

• Good prognostic signs:o Incomplete paralysis in first weeko Taste returns in 1st week.

• Bad prognostic sign:o Complete paralysis in first weeko Pain preceding weaknesso Pregnancyo Elderly patientso Co-morbidities(e.g.DM)o EMG evidence showing spontaneous fibrillation in facial muscles 10-14

days after onset (recovery after 3 months to 2 years & incomplete).

Sequelae

o Persistent severe facial weakness-4%o Synkinetic contraction & twitching of

upper & lower facial muscles-17%o Crocodile tearo Corneal ulcerationo Hemi facial spasm

Melkerson –Rosenthal syndrome

A 45 yr old HIV+ man presented with acute right facial palsy, otalgia, hearing loss and vertigo. Examination revealed vesicular eruption in palate and external auditory canal.

Ramsay Hunt Syndrome

Ramsay Hunt Syndrome

• Loss of taste sensation on anterior 2/3rd of tongue• Occasionally hearing loss and vertigo• Cause: Reactivation of HZV in geniculate ganglion with• C/F:o Ear pain radiating to tonsillar regiono Vesicles in external auditory canal, pinna and anterior pillar

of fauceso Facial paralysis

Hemifacial spasm

Disease mimicking facial nerve lesion

Facial Hemi atrophy

Facial myokymia

• A rare for form of involuntary movement affecting muscles of face.

• Cause: o MS o Brainstem gliomao Recovery from GBS

• Feature: o Continuous twitching of small bands or

strips of muscles. o Gives an undulating or rippling

appearance to overlying skin, descriptively called as `bag of worms' appearance.

Evaluation of Vestibulocochlear nerve

• Cochlear parto Test hearing in each ear separatelyo Rinne’s testo Weber’s testo External auditory meatus (auroscope)

• Vestibular parto Dix-Hallpike’s testo Oculocephalic reflex/VOR

Rinne’s Test

Weber test

Test Result Interpretation

Rinne Positive (AC>BC) Normal

Negative (BC>AC) Conductive deafness

Negative- False + Weber to opposite Severe sensory neural deafness

Weber Central Normal

Lateralized- Opposite Sensory neural deafness

Lateralized- Same side Conductive deafness

Dix-Hallpike’s test

Oculocephalic reflex

Deafness

Conductive deafness: (External /middle ear )

o Wax in the ex. meatuso CSOMo ASOMo Cholesteatomao Trauma to

eardrum/ossicleso Otitis externao Otosclerosis

Sensorineural deafness: (Inner ear/ central auditory pathway)

• Damage to hair cells:o Presbycusiso Viral infectionso Drugs-

Aminoglycosides Frusemide Cisplatin Quinine

o Meningitiso Ménière’s diseaseo Intense noise(>85dB)o Fracture-temporal bone

Sensoryneural deafness

• Central auditory pathway:o Neoplasm:

o C-P angle tumor

o Vascular: o Stroke(AICA syndrome-dorsal

cochlear nucleus at inferior pons)

o Demyelinationo Degenerationo Infections

• Genetic causes: >400 syndromic forms of hearing losso Usher syndrome

(Retinitis pigmentosa + hearing loss)o Pendred syndrome

(Thyroid organification defect+ hearing loss)

o Alport syndrome:

(Renal disease + hearing loss)o Neurofibromatosis II

(Bilateral acoustic neuroma)o Mitochondrial disorders:

MELAS MERRF PEO

Meniere’s Disease

• Also called endolymphatic hydropes

• Classic Triad: Vertigo Deafness Tinnitus

• Rx: Salt restriction Diuretics Vestibular sedatives Inj. gentamicin in middle ear Vestibular nerve section Labyrinthectomy

A 34 yr old man presented with progressive hearing loss, tinnitus and vertigo. His physician noticed some abnormal eye movements during examination.

CP angle tumor

CP Angle tumor

• Most common neoplasm of posterior fossa.

• About 5-10% of all intracranial tumor.

• Cause:o Vestibular schwannoma (85%)o Meningiomas (3-13%)o Epidermoids (2-6%)o Facial and lower cranial nerve schwannomas (1-2%)o Arachnoid cysts (1%)o Lipoma, dermoid tumor, cysto Medulloblastomao Arteriovenous malformation

CP Angle tumor

• C/F:o Hearing loss - 95%o Tinnitus - 80%o Vertigo/unsteadiness - 50-75%o Headache - 25%o Facial hypesthesia - 35-50%o Diplopia - 10%

• O/E: o Papilloedemao Ipsilateral V, VII, VIII palsyo Contralateral long tract sign

• Rx: Surgical

CP Angle mass

Vertigo

Definition:

Sense of feeling of rotation in the space.Types:o True vertigo:

CentralPeripheral

o Pseudo vertigo:

-Sensation of non rotatory movement /falling / unconsciousness

Trait Central Peripheral

Onset Acute More acute

Severity Less More

Nausea/vomiting Less More marked

Imbalance More Less

Aural symptoms Less More marked

Focal neurological deficit Present Absent

Nystagmus

o Type All types Usually horizontal

o Latency None Long

o Fatigability None 10 sec and upto 30 degree

o Duration Long Short

o Direction Change with gaze Not changed

o Fixation of gaze Does not disappear Disappear

Cause • Vascular• Demyelinating• Neoplasm

• BPPV• Meniere’s Disease• Acute vestibular neuronitis• Labyrinthitis• Trauma• Toxin

BPPV

• Incidence: 7-8% in community

• Paroxysmal vertigo, related to change in head position

• Mechanism: Otolothic debris in posterior semicircular canal

• Presentation: Middle age: 20-40 yr Usually in late night Short lasting: <1 min Associated with nausea and vomiting, no aural symptoms Spinning movement related only to change in head position High rate of recurrence

• Dix-Hallpike test: Diagnostic

• Rx: Vestibular sedatives, Epley maneuver