Cranial nerve disorder

Dr .Sugandha Shrestha
Trigeminal Neuralgia
 Trigeminal neuralgia is characterized by paroxysmal
severe, lancinating, electric shock-like bouts of pain
restricted to the distribution of the trigeminal nerve
(CN V).

It is also known as Tic Douloureux

Unilateral (esp. right side)

The mandibular (V3) and/or maxillary (V2) branch

or, rarely, the ophthalmic (V1) branch.

TN Spontaneously attack or triggered by trigger zone &
movement of the face.

Pain seldom lasts more than few seconds or mins.

Onset is typically sudden and bouts tends to persist

for weeks or months before remitting spontaneously.
Epidemiology
Middle-aged and elderly persons are affected
primarily. Mean age is 50 yrs.
Patients who present with the disease when aged 20-
40 years are more likely to suffer from a
demyelinating lesion in the pons secondary to
multiple sclerosis.
Female predominance i.e F:M = 3:2
Prevalence is approximately 1.5 cases per 10,000
population, with an incidence of approximately
15,000 cases per year.
Anatomy
The trigeminal nerve is the largest of all the cranial
nerves.
It exits laterally at the mid-pons level and has 2
divisionsa smaller motor root (portion minor) and a
larger sensory root (portion major).
The gasserion ganglion is located in the trigeminal
fossa (Meckel cave) of the petrous bone in the middle
cranial fossa. It contains the first-order general
somatic sensory fibers that carry pain, temperature,
and touch.
 The peripheral processes of neurons in the ganglion
form the 3 divisions of the
trigeminal nerve
(ie, ophthalmic, maxillary,
and mandibular).
Opthalmic division exits Cranium
via sup.orbital Fissure ;
mandibular and Maxillary
division exits via
Foramen rotundum and
Foramen ovale respectively
.

The trigeminal (fifth cranial) nerve supplies sensation to the skin

of the face, mucous membrane and anterior half of the head.
Its motor part innervates the masseter and pterygoid
masticatory muscles.
.
.
Etiology
Idiopathic : most of cases

Trigeminal neuralgia is divided into 2 categories,

classic and symptomatic

Classic :compressing of vessels :most commonly the

superior cerebellar artery(64%); Venous compression
(36%)

Symptomatic: it can have multiple origins.

Aneurysms, tumors, chronic meningeal inflammation,
or other lesions may irritate trigeminal nerve roots
along the pons causing symptomatic trigeminal
neuralgia.
Pathophysiology
Symptoms results from ectopic generation of action
potentials in pain sensitive afferent fibers of 5th CN
before entering the lateral surface of pons.
Compression or other pathology in nerve leads to
demyelination of large myelinated fibers.
Becomes hyperexcitable and electrically coupled with
smaller unmyelinated or poorly myelinated pain fibers
in close proximity.
Compression of trigeminal nerve root by blood
vessels i.e superior cerebellar most commonly or
tortuous vein occasionally is source of trigeminal
neuralgia in subsequently proportion of patients.
.
Clinical manifestation
Trigeminal Neuralgia presents as attacks of stabbing
unilateral facial pain ( most often on the right side of
the face).

Very rarely in eye.

The number of attacks may vary from less than 1-12

per day or more per hour and up to hundreds per day.

Pain seldom lasts for more than few seconds or

minutes and occur both day or night for several
weeks at a time.
 Triggering of pain attacks include the following:
May occur spontaneously or with movements of
affected areas.
Chewing, talking, or smiling

Drinking cold or hot fluids

Touching, shaving, brushing teeth, blowing the nose

Encountering cold air from an open automobile

window.
 Pain localization is as follows:
Patients can localize their pain precisely.

The pain commonly runs along the line dividing either

the mandibular and maxillary nerves or the
mandibular and ophthalmic portions of the nerve.

The pain shoots from the corner of the mouth to the

angle of the jaw (60% cases).
 In 30%, pain jolts from the upper lip or canine teeth to
the eye and eyebrow, sparing the orbit itself.

In less than 5% of cases, pain involves the

ophthalmic branch of the facial nerve.

An essential feature of trigeminal neuralgia is

that objective signs of sensory loss cant be
demonstrated on examination.
 The pain has the following qualities:

Characteristically severe, paroxysmal, and

lancinating.

Commences with a sensation of electrical

shocks in the affected area.

Crescendos in less than 20 seconds to an

excruciating discomfort felt deep in the face,
often contorting the patient's expression.
 Begins to fade within seconds, only to give way to a
burning ache lasting seconds to minutes.

Pain fully abates between attacks, even when they are

severe and frequent .

Attacks may provoke patients to grimace, wince, or

make an aversive head movement, as if trying to
escape the pain, thus producing an obvious
movement, or tic; hence the term "tic douloureux"
 Other diagnostic clues are as follows:
Patients carefully avoid rubbing the face or shaving a
trigger area, in contrast to other facial pain
syndromes, in which they massage the face or apply
heat or ice

Many patients try to hold their face still while talking,

to avoid precipitating an attack.

In contrast to migrainous pain, attacks of TN rarely

occur during sleep.
Criteria for Trigeminal Neuralgia
Strict criteria for TN as defined by the International
Headache Society (IHS) are as follows :
A Paroxysmal attacks of pain lasting from a fraction
of a second to 2 minutes, affecting 1 or more
divisions of the trigeminal nerve and fulfilling criteria B
and C
B Pain has at least 1 of the following
characteristics: (1) intense, sharp, superficial or stabbing; or
(2) precipitated from trigger areas or by trigger factors
C Attacks stereotyped in the individual patient
D No clinically evident neurologic deficit
E Not attributed to another disorder
Diagnosis
TN mostly diagnosed clinically.

Other disorders that cause facial pain should be ruled

out before TN is diagnosed.

Neuroimaging study not effective. But may helpful to

rule out underlying causes in some extent.

ESR is indicated if temporal arteritis is suspected.

Differential diagnosis
Multiple sclerosis (most common)

Migraine

Cluster headache

Cavernous sinus syndrome

Management
Treatment of TN comprises the following:
Pharmacologic therapy

Percutaneous procedures (eg, percutaneous

retrogasserian glycerol rhizotomy)

Surgery (eg, microvascular decompression)

Radiation therapy (ie, gamma knife surgery)

Pharmacology therapy
Pharmacologic trials alone is adequate treatment for
75% of patients .
Single-drug therapy may provide immediate
and satisfying relief .
Carbamazepine is the best studied drug for
TN .
Lamotrigine and baclofen are second-line
therapies .

Gabapentin has demonstrated effectiveness in

TN, especially in patients with multiple
sclerosis .
Surgical therapy
Features of surgical treatment include the following:
Gasserian ganglion level procedure:
Micro vascular decompression (MVD)

Ablative treatments

Radiofrequency thermocoagulation (RFT)

Glycerol rhizolysis (GR)
Balloon compression (BC)
Stereotactic radiosurgery (SRS)
Peripheral procedures:
Peripheral neurectomy

Cryotherapy (cryonanlgesia)

Alcohol block
 Percutaneous surgeries :older patients with medically
unresponsive trigeminal neuralgia

Younger patients and those who are expected to do

well under general anesthesia should first consider
micro vascular decompression.
 Microvascular decompression
.
.
.
BELLS PALSY
Bells palsy
One of the most common neurologic disorders of the
cranial nerves.

Described by Sir Charles Bell in the 19th century.

Common form of facial paralysis with rapid onset is

Bells palsy.

Mostly causes unilateral facial paralysis.

Spontaneous recovery (80%)

 Less common before the age of 15yrs.

The incidence in men and women is similar.

Approximately 6-9% develop recurrent Bells Palsy

Facial paresis alone occurred in 31% and Complete

paralysis in 69%.
Etiology
Most cases unknown.
Vascular congestion with secondary ischemia to the
nerve: Vasospasm would lead to ischemia, nerve
edema, and secondary compression within the
fallopian canal.

Viral polycranioneuropathy: Herpes simplex virus and

herpes zoster virus
Presence of HSV-1 DNA in endoneurial fluid and posterior
auricular muscle, suggesting that a reactivation of this virus
maybe responsible.
 Clinical manifestation
Unilateral facial paralysis
Inability to close the eye

Absence of the nasolabial

fold
May be loss of taste on

ant. tongue.
Pain behind the ear

Drooling

Sag of the eyebrow

Evaluation of acute facial
paralysis
House-Brackman grade system:
I. Normal: Normal facial functioning of all areas
II. Mild dysfunction: slight weakness noticeable only
on close inspection
At rest: normal symmetry and tone

Motion: some to normal movement of forehead

Ability to move corners of mouth with maximal effort

and slight asymmetry
Ability to close eye with minimal effort
No synkinesis, contractur, or hemifacial spasm
 III. Moderate dysfunction:
obvious but not disfiguring difference between two side
No function impairment
Noticeable but not severe synkinesis, contracture, and hemi
facial spasm
At rest: normal symmetry and tone
Motion:

slight to no movement of forehead

Ability to close eye with maximal effort and obvious asymmetry

Ability to move corners of mouth with maximal effort and obvious
asymmetry
Patients with obvious but not disfiguring synkinesis,
contracture, and hemifcial spasm are grade 3
regardless of degree of motor activity.
 IV. moderate severe dysfunction:
Obvious weakness and disfiguring asymmetry

At rest: normal symmetry and tone

motion:

no movement of forehead

Inability to close eye completely with maximal effort

Asymmetrical movement of corners of mouth with
maximal effort
Patients with synkinesis, mass action, and
hemifacial spasm severe enough to interfere with
function are grade 4 regardless of degree of motor
activity
 V. severe dysfunction:
Only barely perceptible motion

At rest: possible asymmetry with droop of corner of

mouth and decreased or absent nasolabial fold

Motion:

No movement of forehead

Incomplete closure of eye

Slight movement of corner of mouth
Synkinesis, contracture, and hemifacial spasm usually
absent

VI. total paralysis: no movement

 Differentiate between upper and lower motor neurone
lesion

UMNL:
frontalis is spared allowing normal furrowing of brow
and eye blinking

LMNL:
all muscles of facial expression are affected
Diagnosis
The diagnosis of Bells palsy can usually be
made clinically in patients with
No risk factors or preexisting symptoms for

other causes of facial paralysis.

Typical presentation.

Absence of cutaneous lesions of herpes

zoster in the external ear canal.

Normal neurologic examination with the

exception of the facial nerve.
 A careful history of the patients illness

Sudden in onset and frequently evolve over 2-3

weeks after onset

Any palsy progression over 3 weeks should be

evaluated for a neoplasm

Any palsy persist for 6 month without any recovery

should be considered for a neoplasm.
 Audiometry : to rule out any involvement of the
auditory nerve
CT and MRI: for patient without fully recovery, to
identify the site of lesion.
Electrophysiological testing to determine prognosis.
Serologic studies can be considered to evaluation for
lyme disease, autoimmune disorders, or other central
nervous system disease
Differential diagnosis
Ramsay Hunt syndrome
Lyme disease

Melkersson-Rosenthal syndrome

Infarcts,

Demyelinating lesions of multiple sclerosis,

Tumors

Bilateral facial paralysis can be seen GBS.

Ramsay hunt syndrome
painful rash over the ears by H. Zoster

It is manifest by a facial palsy with a vesicular

eruption over a distribution of a cranial nerve

Sensorineural hearing loss and vertigo may also be

present in up to 20% of cases.

Prognosis is poor than Bells palsy

.
 Melkersoon Rosenthal syndrome:
Consists of a triad of

Recurrent facial paralysis,

Recurrent - and eventually permanent facial

(particularly labial) edema,

Plication of the tongue.

Cause is unknown
Treatment
Symptomatic treatment:
Use of paper tape upper eyelid during sleep.

Prevent corneal drying.

Massage of the weakened muscles

Medical measures:
Steroid 1mg/kg/day: Prednisone 1mg/kg up to

80mg max per day tailing off in second week

(reduces oedema)
Anti-virus: Aciclovir given within first 72hrs

(prevents viral replication)

Vitamine B: B1 & B12