Trigeminal Neuralgia

Presented by : Dr Abdullah Mumtaz

BDS Roll# 03
Introduction
Also known as tic douloureux, is a chronic pain condition
characterized by recurrent brief episodes of electric shock-
like pains, affecting the fifth cranial (trigeminal) nerve,
which supplies the forehead, cheek and lower jaw.
One of the most painful neurological condition and is often
described as 'lightning bolt' stuck on the face or a stabbing
sensation on the face.
Short-lasting paroxysms of pain occur multiple times
throughout the day debilitating the patient.
Almost always unilateral and can involve one or more
divisions of the trigeminal nerve.
Objectives
Identify the etiology and epidemiology of
trigeminal neuralgia.
Outline the appropriate history, physical, and
evaluation of trigeminal neuralgia.
Review the treatment and management
options available for trigeminal neuralgia.
The Trigeminal Nerve
The trigeminal nerve is the fifth cranial nerve. It is
responsible for the sensory supply of the face and the
motor and sensory supply to the muscles of mastication.
The trigeminal nerve starts at the pons and divides into
three branches:
Ophthalmic (V1): Supplies the eye, upper eyelid, and the
forehead
Maxillary (V2): Supplies lower eyelid, cheek, nostril,
upper lip, and upper gum
Mandibular (V3): Supplies the lower lip, lower gum, jaw
and the muscles of mastication
Etiology
Most cases of trigeminal neuralgia are due to the compression of the trigeminal nerve root,
within a few millimeters of its entry into the pons.

Between 80% and 90% of the cases of TN are caused by compression by an adjacent artery or a
vein.

The blood vessel, which has been mostly implicated in about 75% to 80% of the cases, is the
superior cerebellar artery.

Other blood vessels that are known to cause TN include the anterior inferior cerebellar artery,
the vertebral artery, and the petrosal vein.
Other causes of nerve compression include meningioma, acoustic neuroma, epidermoid cyst,
and rarely by an arteriovenous malformation or a saccular aneurysm.

Multiple sclerosis is a risk factor for TN, and it is reported in about 2% to 4% of patients with
TN. This is secondary to the demyelination of the trigeminal nerve nucleus by multiple
sclerosis.
Epidemiology

Trigeminal neuralgia affects 4 to 13 per 100000 people annually.

 Women are affected more compared to men. The male-to-female
prevalence ratio ranges from 1 to 1.5 to 1 to 1.7.
Most cases occur after age 50; some cases are seen in second and third
decades and very rarely seen in children.
The lifetime prevalence in population-based studies was estimated to
be about 0.16% to 0.3%.
The development of trigeminal neuralgia in a young person should raise
suspicion for multiple sclerosis.
The prevalence of TN in patients with multiple sclerosis is between 1
and 6.3%.
It is also reported that patients with hypertension have a slightly higher
incidence of TN compared to the general population.
Pathophysiology
Trigeminal nerve compression.
Nerve demyelination occurring around the site of compression.It
is thought to be due to the ectopic impulse generation set up by
the demyelinated lesion, thereby causing ephaptic transmission.
The ephaptic link between fibers involved in pain generation and
fibers mediating light touch could account for the precipitation of
shock-like pains in the facial trigger zone by light tactile
stimulation.
A triggered episode followed by refractory periods and a single
stimulus leading to trains of painful sensations indicates the
possible role of the central pain mechanism in TN. Altered grey
matter in the sensory and motor cortex has also been described.
Continued..
Some theories describe demyelination secondary to vascular
compression of the nerve root by tortuous or aberrant vessels. The
vessel mostly implicated is the superior cerebellar artery.This
hypothesis is further strengthened by the relief of symptoms
following surgeries to separate the offending vessels from the nerve.
According to the bio-resonance hypothesis, when the vibration
frequency of the trigeminal nerve and the surrounding structures
come close to each other, the trigeminal nerve fibers are damaged,
leading to abnormal transmission of impulse, thereby resulting in
facial pain.
Multiple other conditions like amyloid infiltration, bony
compression, arteriovenous malformation and small infarcts
in the medulla and pons, have been described to cause TN.
Classification
TN is divided into classic TN, secondary TN and
idiopathic TN in the International Classification of
Headache Disorders
Classic TN: This includes TN related to vascular
compression.
Secondary TN: This includes TN due to a tumor
along the trigeminal nerve or TN due to an underlying
disease like multiple sclerosis.
Idiopathic TN: This is when the cause is unknown. 
Symptomatic Classification
Considering the symptomatic aspect, trigeminal
neuralgia can classify as:

Type 1 - Presence of paroxysmal pain alone

Type 2 - Paroxysmal pain along with constant pain in

the background
History
The pain in trigeminal neuralgia occurs in paroxysms and is usually
maximal at or near onset. Sometimes, with severe pain, facial muscle
spasms can be seen. Hence, TN is also known as 'tic douloureux.' 

The majority of the patients describe the pain as electric shock-like pain,
lasting from one to several seconds. Pain in TN is typically unilateral.
Occasionally, it is bilateral, but very rarely occurs simultaneously on both
sides.The pain episodes rarely occur during sleep.

V2 and V3 divisions of the trigeminal nerve are usually involved in the pain
distribution.When the V1 subdivision is involved, mild autonomic
symptoms like lacrimation, rhinorrhea, and conjunctival injection can be
seen. However, isolated V1 division involvement is very rare and is seen in
less than 5% of patients with TN. 
Continued..
Trigger zones may be present in the distribution of the affected nerve.
These are usually located near the midline.Mostly reported in the nasal
and perioral regions.Pain is triggered by lightly touching these zones.

Patients with TN are usually aware of these zones and avoid any
stimulation of them. All patients with TN may not have trigger zones, but
trigger zones are nearly pathognomonic for TN. 

Other triggers reported causing trigeminal neuralgia paroxysms include

brushing teeth, shaving, washing the face, smoking, chewing, talking,
grimacing, or exposure to cold air.

In younger patients, who present with symptoms of TN, other neurological
conditions like multiple sclerosis should merit consideration in the
differentials. Such patients should be asked about other neurological
symptoms like focal weakness, vision changes, dizziness, and ataxia. 
Examination
In patients with TN, the physical examination is generally normal. Hence, the
physicians should perform a detailed physical examination of the head, neck,
eyes, ears, teeth, mouth, and the temporomandibular joint to rule out other
causes of facial pain.

The finding of typical trigger zones is suggestive of TN. 

In patients with classic TN, the neurologic examination is normal. Hence, physical
examination showing a sensory loss in trigeminal nerve distribution, loss of
corneal reflex, or weakness in facial muscles should prompt the physician to
consider secondary TN and other differentials.
 
Several patients with TN complain of toothache and pain with brushing teeth. A
detailed oral examination can help in differentiating the dental causes of pain from
trigeminal neuralgia.
Diagnostic criteria established by the ICHD-
3
A) Recurrent paroxysms of facial pain unilaterally in the
distribution of trigeminal nerve and fulfilling criteria B and C.
B) Pain has the following characteristics:
Pain lasting a fraction of a second to about 2 minutes
Pain with severe intensity
Electrick-shock like or shooting pain with sharp quality
C) Innocuous stimuli precipitate the pain in the affected
distribution
D) No alternative ICHD-3 diagnosis better explaining the
symptoms
The subtypes of TN are defined by ICHD-3 as follows 
Differentiating the types
Classic TN: This is secondary to neuromuscular compression and
fulfilling the criteria above. This requires demonstration of the
compression on an MRI or during the surgery for neuromuscular
compression, with associated morphological changes in the
trigeminal nerve root. 

Secondary TN: This is defined as TN secondary to an underlying

disease. Some of the reported causes are multiple sclerosis,
arteriovenous malformation, and cerebellopontine angle tumor. 

Idiopathic TN: This is defined as TN with no abnormalities seen

on MRI or electrophysiological tests. 
Further Investigations
Neuroimaging studies like MRI Brain or CT Head can help in identifying
causes like cerebellopontine angle tumor or multiple sclerosis, which can cause
secondary TN. 
Magnetic resonance imaging or high-resolution MRI can This can give a
detailed picture of the blood vessels and the brain.
FIESTA sequences in some MR machines. In these machines, sections as thin
as 1 mm can be taken in a coronal plane, without any skips in between the
images. This way, the imaging of the entire course of the trigeminal nerve can
be obtained, and the offending vessel causing compression can be identified. 
Hence, though TN is a clinical diagnosis, MRI of the brain with and without
contrast is recommended to rule out a structural brain lesion in all patients
with clinically suspected TN. It is also important to note that patients less than
40 years of age, patients with bilateral symptoms, and with the sensory loss on
physical examination are at a higher risk of secondary trigeminal neuralgia. 
Differential Diagnosis
Postherpetic neuralgia: This is secondary to acute Herpes zoster. This usually presents with a severe preceding
rash. Often involves the first division of the trigeminal nerve, and the pain is usually continuous. TN pain is
intermittent and lasts a few seconds.

Dental pain: This is usually continuous and intraoral pain, which can be dull or throbbing. TN pain is usually
sharp, intermittent, and electric-shock like. Also, abnormalities are found on oral examination if the pain is from a
dental source.

Short-lasting unilateral neuralgiform headache attacks (SUNA) and short-lasting unilateral neuralgiform
headache attacks with conjunctival injection and tearing (SUNCT): These present as sudden, brief attacks of
unilateral pain in orbital, periorbital and temporal regions. Ipsilateral autonomic symptoms also accompany
these. 

Trigeminal neuropathy: This condition presents with persistent pain and can be associated with sensory loss.

Temporomandibular joint syndrome: This condition presents with persistent pain. Localized tenderness and
jaw abnormalities can be demonstrated. 

Glossopharyngeal neuralgia: Patients present with pain in tongue, mouth, and throat. The pain is triggered by
chewing, talking, and swallowing.
Treatment/Management
The management options for patients with trigeminal
neuralgia depends on a variety of factors, including;
 age
general health
disease severity
the underlying cause.
The decision should be taken after a thorough
discussion with the patient and other doctors involved
in the care of the patient.
Pharmacological
First-line treatment for patients with classic TN and idiopathic TN is
pharmacologic therapy. The most commonly used medication is the;
Anticonvulsant drug, carbamazepine. It is usually started at a low dose, and the
dose is gradually increased until it controls the pain.
Oxcarbazepine is a newer drug and is being increasingly used as first-line therapy
for TN in patients who do not respond to or who cannot tolerate carbamazepin
Baclofen is a muscle relaxant that can be used to treat TN. Side effects include
dizziness, sedation, and dyspepsia. 
Other medications include lamotrigine, phenytoin, gabapentin, clonazepam, and
valproic acid.
Newer drugs like eslicarbazepine, an active metabolite of oxcarbazepine, and the
new Nav1.7 blocker, vixotrigine, are being explored for the pain relief in TN.
Patients with secondary TN also can respond well to pharmacotherapy. However, it
is recommended to treat the underlying lesion or disease.
Surgical Treatment

Botulinum Toxin Injections:This can be beneficial for some patients, particularly the middle-aged and the
elderly, who are refractory to medical therapy or who cannot tolerate medical therapy due to their side effects.


Surgical Therapy

Patients who are refractory to medical therapy can be considered for surgery.


Microvascular decompression ;This is one of the most common procedures used to treat trigeminal
neuralgia. This is beneficial for patients with TN, where compression of the nerve root is the cause


Ablative procedures include rhizotomy with thermocoagulation, chemical injection, or mechanical
balloon compression. These procedures involve damaging the trigeminal nerve root, thereby interrupting
the pain transmission signals to the brain.


Radiosurgery: This procedure involves using radiosurgery instrumentation


Peripheral neurectomy and nerve block: The neurectomy can be performed on peripheral branches of
trigeminal nerve like the supraorbital, infraorbital, lingual, and the alveolar nerves.
Prognosis
Not a life-threatening condition.
However, it can lead to life long pain and can be disabling.
The course of TN is variable. Some patients may have
episodes lasting weeks or months, followed by pain-free
intervals. Some patients have persistent background facial
pain concomitantly with TN. In some patients, the pain
attacks worsen over time, with fewer and shorter pain-free
intervals before they recur.
Also, the medications might lose effectiveness over time.
Correct diagnosis and proper management can be
beneficial to the patients and leads to a good prognosis. 
Complications
The pain in trigeminal neuralgia is so severe and debilitating that the patients can
develop depression, if not adequately treated.
Patients with severe pain associated with facial twitches can become socially
withdrawn due to embarrassment and fear of an impending attack.
Patients treated with anticonvulsant drugs over the long term can have adverse
drug effects.
Microvascular decompression and percutaneous neurosurgical procedures can
pose surgical risks. 
Some patients permanently develop facial numbness on the affected side.
Occasionally, patients develop corneal anesthesia and jaw weakness.

Anesthesia dolorosa is seen in a few patients. It is an intractable facial

dysesthesia, which can be more disabling than original TN.
Take Home Notes
Trigeminal neuralgia is a chronic painful condition, that presents with unilateral facial
pain.

The pain in trigeminal neuralgia is usually described as a sharp, electric shock-like,

stabbing, or lancinating pain in the distribution of one or more divisions of
the trigeminal nerve
. 
The majority of the cases of trigeminal neuralgia are due to neurovascular compression.

Although TN is a clinical diagnosis, neuroimaging study is recommended in all patients

with clinically suspected TN, to differentiate classic TN from secondary TN.

Carbamazepine is a first-line medication in the treatment of trigeminal neuralgia.

Microvascular decompression is one of the most effective surgical modalities for the
treatment of trigeminal neuralgia.  
References
https://www.ncbi.nlm.nih.gov/books/NBK554486/
Thankyou 