Diseases of Cranial nerves 5th, 7th and 8th

PRESENTER
DR. A T M HASIBUL HASAN
MD (Neurology) Thesis part student
Department 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

INDIVIDUAL CRANIAL
NERVE
PATHOLOGY

Evaluation of CN-V
 Sensory- Somatic sensation from face
 Reflex- Corneal reflex and Jaw jerk.
 Motor-Masseter, temporalis and
pterigoid

Reflex
Corneal Reflex Jaw Jerk

Jaw Jerk
• Brisk:
o Pseudobulbar palsy:
MND
Bilateral stroke
MS
• Absent:
o Bulbar palsy:
MND
Post viral

Motor examination
• Inspection:
• Palpation:
• Motor movement:

Lesions of Trigeminal Nerve
Level Feature Cause
Supra nuclear o Unilateral UMN lesion: Jaw deviates
to opposite side
o 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 lesion
o Demyelinating lesion
o Inflammatory lesion
o Neoplastic lesion
o Cavernous malformation
o Syringobulbia
o Lateral medulla:
Spinal tract and nucleus of CN V
• Ipsilateral:
• Contralateral:
 Hemi sensory loss
o Lower medulla:
 Onion skin pattern of sensory loss

Level Feature Cause
Subarachnoid
space
o Preganglionic trigeminal nerve:
• Ipsilateral:
 Weakness & atrophy of muscles of
mastication
 Loss of corneal reflex
 VII & VIIIth CN: CP angel tumor
o Cerebellopontine angel
tumor
o Inf/Inflammatory lesion
o Neurovascular
compression
o Trauma
Petrous apex
&
Meckels cave
o Severe hemifacial pain o Infection
o Neoplastic lesion
o 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 CSOM
o Mastoiditis
o Skull base osteomyelitis
o Herpes Zoster
• Eruption in skin along distribution of CN V
o Varicella Zoster

Level Feature Cause
Cavernous
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 lesion
o Neoplasm
o Vascular
o Radiation injury
o Superior orbital fissure syndrome:
• All above plus proptosis but without V2
Distal
trigeminal
lesion
o Numb cheek syndrome:
• Infraorbital nerve
o Numb chin syndrome:
o Inferior alveolar nerve
o Trauma
o Neoplasm
o 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. Cholesteatoma
2. Brain stem eg, tumor, demyelination
3. Perineural spread of tumor
4. Injury during surgery

Facts about TN
Risk Factors:
o Age: >50yr
o Female gender
o Family history
o MS
Trigger factors:
o Talking
o Chewing
o Eating
o Brushing
o Washing face
o Shaving
Factors decreasing pain threshold:
o Stress
o Inadequate sleep
o Exposure to cold
o Hyperglycemia
o Drugs
o Menstruation

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

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:

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 hemiplegia
o Foville syndrome: Dorsal pons
• Ipsilateral:
 Lateral gaze palsy
 Facial palsy
 Eight and half syndrome/ INO
 Horner syndrome
• Contralateral:
 Ataxia
o Millard-Gubler syndrome: Ventral pons
• Ipsilateral:
 Facial palsy ± Lateral gaze palsy
• Contralateral:
 Hemiplegia
o Ischemia/ vascular
lesion
o Demyelinating lesion
o Neoplastic lesion
o Syringobulbia

Lesions of Facial Nerve
Level Feature Cause
C-P angel o Diagnostic clue:
• Ipsilateral:
 Facial palsy
 Loss of taste sensation (Ant 2/3rd
of tongue)
 Hyperacusis
 Tinnitus
 Hearing loss ± vertigo
± CN V, VI
o Hemifacial spasm:
Neurovascular compression of motor root
o Geniculate neuralgia/ Hunt neuralgia:
Neuralgia affecting nervous intermedious
 Paroxysmal otalgia
 AICA compression
 Rx- CBZ, decompression
o Acoustic neuroma
o Meningioma
Peripheral
lesion
o Bell’s palsy
o Ramsay Hunt syndrome: Herpes zoster
oticus
o Mobius syndrome:
 Congenital bilateral CN VI+ VII palsy
o Post traumatic facial palsy:
o Idiopathic
o Malignancy
o Infection
o Inflammation
o Iatrogenic

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 days
oAntivirals- Aciclovir 400 mg 5 times for 10 days
oEye care
oPhysiotherapy

Prognosis
• Recovery: 85% within 3 weeks.
• Good prognostic signs:
o Incomplete paralysis in first week
o Taste returns in 1st
week.
• Bad prognostic sign:
o Complete paralysis in first week
o Pain preceding weakness
o Pregnancy
o Elderly patients
o 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 tear
o Corneal ulceration
o 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 region
o Vesicles in external auditory canal, pinna and anterior pillar
of fauces
o Facial paralysis

Disease mimicking facial nerve lesion

Facial myokymia
• A rare for form of involuntary
movement affecting muscles of face.
• Cause:
o MS
o Brainstem glioma
o 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 part
o Test hearing in each ear separately
o Rinne’s test
o Weber’s test
o External auditory meatus (auroscope)
• Vestibular part
o Dix-Hallpike’s test
o Oculocephalic reflex/VOR

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

Deafness
Conductive deafness:
(External /middle ear )
o Wax in the ex. meatus
o CSOM
o ASOM
o Cholesteatoma
o Trauma to
eardrum/ossicles
o Otitis externa
o Otosclerosis
 Sensorineural deafness:
(Inner ear/ central auditory pathway)
• Damage to hair cells:
o Presbycusis
o Viral infections
o Drugs-
 Aminoglycosides
 Frusemide
 Cisplatin
 Quinine
o Meningitis
o Ménière’s disease
o 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 Demyelination
o Degeneration
o Infections
• Genetic causes:
>400 syndromic forms of hearing loss
o 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, cyst
o Medulloblastoma
o 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 Papilloedema
o Ipsilateral V, VII, VIII palsy
o Contralateral long tract sign
• Rx: Surgical

Vertigo
Definition:
Sense of feeling of rotation in the space.
Types:
o True vertigo:
Central
Peripheral
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

Diseases of Cranial nerves 5th, 7th and 8th

Diseases of Cranial nerves 5th, 7th and 8th

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