CONTENTS:

1. Introduction

2. Definition

3. Types of TN

4. Etiology

5. Pathophysiology

6. Clinical features

7. Criteria for diagnosis

8. Diagnosis

9. Differential diagnosis

10.Treatment

11.Conclusion
TRIGEMINAL NEURALGIA AND CHRONIC FACIAL PAIN

Introduction

There are many causes of chronic pain affecting the face. These may range from

anything as common as simple tooth decay to some of the rarer conditions

covered in this booklet.

The aim of this booklet is to provide information for those suffering from some

of the more severe and persistent forms of chronic facial pain. The conditions

covered are Trigeminal Neuralgia, Glossopharyngeal Neuralgia, Post-Herpetic

Neuralgia, Atypical Facial Pain, Temporo Mandibular Joint Disease, Cluster

Headache, Atypical Odontalgia and Oral Dysaesthesia.

TRIGEMINAL NEURALGIA

What Is Trigeminal Neuralgia?

Neuralgia is a word meaning nerve ("neur ”) pain ("...algia"). There are two

"trigeminal nerves", one each supplying sensation to the right and left side of

the face. They both have three branches (hence "tri...") which supply the

forehead, cheek and jaw.

Doctors may sometimes refer to these branches as “divisions”.

What Are The Symptoms?

The pain typically only affects one side of the face. It can be severe and is of an

unpredictable, paroxysmal nature. It is often described as; "burning, stabbing,

lancinating, electric, shooting". Whilst it is often mild, for many patients it can

be experienced as a violent and excruciating pain. These severe pains typically

only last for a few seconds. It more commonly affects the jaw and cheek area,

though may cover the whole of one side of the face. There are frequently

“trigger points” on the skin which if touched will provoke a violent spasm of

pain. The pain can often be felt in the teeth. It may be provoked by cleaning the

teeth, hot or cold fluids, wind, shaving, eating and speaking

Attacks may at first be intermittent with long periods of relief occurring

between them. For many these attacks become more frequent and of longer

duration. For some the pain is constant i.e., it repeatedly shoots all day.

It may begin in a rather atypical pattern and many patients find that they have

had extensive treatment from their dentist or others before the diagnosis

eventually becomes apparent.

Who Does It Affect?

It may affect people of all ages though very rarely occurs in children. Whilst it

is relatively more common in the older age groups, it not infrequently affects

young adults. It is twice as common in females as males and similarly it afflicts

the right side of the face twice as frequently as it does the left. Very rarely does

it affect both sides. There have been very few recorded cases of it affecting

more than one member of a family, i.e. it is not hereditary. As many as 1 in 200

people may be suffering from, or have suffered, trigeminal neuralgia in the U.K.

What Causes It?

A mass of theories abound, though in less than 5% of cases is a clear cause to be

found. This small group of patients are sometimes found to have tumours (these

are nearly always benign), infections or other abnormalities pressing upon the

nerve. Trigeminal neuralgia occasionally affects patients with established

multiple sclerosis though it is virtually never their first symptom, i.e. if you

have trigeminal neuralgia there is absolutely no reason to suspect that you may

be developing multiple sclerosis.

For the vast majority of patients with so-called "primary trigeminal neuralgia"

no clear cause has been established. It has variously been proposed that the

underlying abnormality lies within the brain, the nerves inside the head, or, the

nerve as it passes through the face. Currently one of the most popular theories is

that a blood vessel, normal in all respects other than its position, presses upon
the nerve as it emerges from the base of the brain inside the skull. Rather like a

"slipped disc" pressing upon the nerves in the back will cause sciatica, this

blood vessel (about the size of a biro refill) is thought to squeeze the trigeminal

nerve (about twice the size of a biro refill) to cause facial pain.

However, it would be wiser for you to select treatments on the basis of their

safety rather than on these theoretical considerations of origin.

How Is It Diagnosed?

There are no signs for the doctor to detect on examination as the nerves function

normally. Similarly there is no test which can conclusively prove that the

condition is trigeminal neuralgia. The diagnosis is entirely based on the

recognition of the clinical symptoms – your description.

Care must be taken to rule out other causes of facial and oral pain. Problems

with the teeth, in the mouth, in the ear, bones of the skull and in the glands

around the mouth and face need to be excluded. Therefore careful examination

of these areas is required. A brain scan may be performed in order to exclude

those rare lesions inside the head which can cause pressure on the nerves. The

blood vessels possibly involved in causing trigeminal neuralgia cannot

accurately be seen on these scans and in true primary trigeminal neuralgia the

tests will usually be negative.

What Medicines Are Available?

The most powerful medication is Carbamazepine (Tegretol). This is an old and

well established drug which was developed primarily for use in epilepsy. It

dampens the activity in excitable nerves and in this instance its effect is

manifest on the trigeminal system. Carbamazepine may cause a rash and a

number of other rarer side-effects which you may wish to discuss with your

doctor. You will need to have a regular blood test. You may need to take large

quantities of the drug. Its principal side-effect is drowsiness and in some people

this results in insufficient being taken and pain breaking through. However, for

the majority of patients Carbamazepine is safe and all that is required.

Other anticonvulsants have been tried and these include Lamotrigine. This drug

has to be started at a low dose and gradually increased if the principal side

effect of a rash is to be avoided. Gabapentin is another though this may be built

up quite quickly. Sodium Valproate (Epilim) is another and may be used instead

of or in combination with Carbamazepine. They may well be less effective

though may be better tolerated, i.e. cause less drowsiness.

Baclofen is a muscle relaxant. Seldom will it work on its own though it may be

effective in combination with Carbamazepine or its alternatives

None of these drugs are available over the counter and you will need a

prescription from your doctor.

None of the standard pain killers, however strong, seem to work on trigeminal

neuralgia and you should stop them as they will add to the cocktail effect of the

medicines without contributing to the pain relief.

What Form Does Surgery Take?

There are many types.

Interrupting the nerve:

A number of different methods are used to interrupt the nerve impulses. Whilst

this may relieve the pain it will nearly always leave an area of lost feeling or

numbness on the face. The interruption can be made at one of three sites.

A. In the Periphery of the Face

The small branches of the nerve which pass through the cheek and forehead

may be cut or frozen. This can usually be carried out with local anaesthetic. A

cut is made on the inside of the mouth or a needle passed to the nerve from the

inside of the mouth.

The procedure will always result in an area of numbness which upon recovery is

usually accompanied by the return of pain. It is a safe treatment in that very few

patients come to harm during the procedure. There are three principal problems.

Firstly the effect may be short-lived. Secondly the numbness may lead to a

different and sometimes worse form of pain. Called "anaesthesia dolorosa" this
numb pain may be even worse than the original problem. Treatment is very

difficult. It is a rare though very troublesome complication. Finally, it can only

be used if the pain is confined to a small area of the face.

B. At the "Ganglion"

This lies just inside the bottom of the skull. It is a swelling on the nerve, the size

and shape of a split broad bean. It contains the cells that supply the nerve fibres

of the trigeminal nerve.

In this technique a needle is passed via the cheek up through a small hole in the

base of the skull to reach the ganglion. An X-ray is used to guide it. The

ganglion may then be injected with alcohol or glycerol, interrupted by heat

(“thermocoagulation”)or cold (“cryotherapy”), or compressed with a small

inflatable balloon positioned at the tip of the needle. These procedures are

carried out with the patient partially awake. The heavy sedation used means that

the procedure is usually well tolerated though the surgeon is able to confirm by

stimulation that the needle has reached the correct place. It can be performed as

a Day Case or with a simple over night stay in hospital.

A broader area of the face may be treated with this technique than can

commonly be achieved by interrupting the nerve in the periphery of the face.

Again there is usually a significant numbness affecting the face though this is

often not as complete as when the nerve is cut. Anaesthesia dolorosa may also

complicate this procedure.

There are a number of additional risks. As the needle penetrates the skull

complications affecting the brain may arise. This occurs in much fewer than 1%

though meningitis, brain abscess and brain haemorrhage have all been recorded.

The result of these unusual events may be death or permanent disability,

(mental or physical handicap). Further, some patients have found the procedure

extremely unpleasant though with good sedation it is fairly well tolerated.

Indeed, in those patients in whom the pain returns many are content to come

back for a second procedure. Finally, in those patients where primarily the

forehead is affected it can be difficult to position the needle into that part of the

nerve and thus a large area of numbness is created before the pain is relieved.

C. The Nerve Trunk

This is the portion of the nerve which emerges from the brain and extends to the

ganglion. It is approximately 1 cm in length and about twice the width of a biro

refill.

In order to undertake this procedure a formal operation is required under general

anaesthesia. An opening at the base of the skull is made just behind the ear.

Using a microscope the nerve is identified as it passes between the brain and the

inside of the skull. A cut is then made in the nerve in order to divide the fibres

that supply feeling to the part of the face which is affected by pain. This

inevitably results in some numbness though often less than might be anticipated.

The operation involves a hospital stay of between five to ten days and you will

usually be off work for a period of some six weeks.

The result is usually lasting relief as the numbness tends not to recover, i.e. it

frequently provides for a permanent cure.

The principal disadvantage of this procedure is the need for an operation in such

a strategic area of the brain. Even in the most expert hands and with the greatest
possible care the risk to life and limb exceeds that of procedures performed

more peripherally.
The exact risk will depend upon the age and general health of the patient but the

incidence of death or serious disability (mental or physical handicap) has been

1% or less in most reports.

In addition to the risk to life and limb the adjacent nerves coming from the brain

stem may be damaged. These include those supplying hearing and balance, i.e.

the patient may suffer unsteadiness or deafness in one ear. More rarely

weakness affecting the face on the side of the operation or difficulty in

swallowing may be encountered. This damage to adjacent nerves has arisen in

something less than 5% of cases. A not infrequent complaint has been pain

around the wound behind the ear. This usually settles though can be a trouble

for some months. Again anaesthesia dolorosa may follow this procedure.

A technique of “Stereotactic Radiosurgery” or “Gamma Knife” treatment has

been developed. This uses a highly focused beam of radiation to interrupt the

nerve just behind the ganglion. It has shown promising early results though

remains very much under evaluation. It has the disadvantages that the benefits

are delayed and that numbness may result bringing with it concerns about future

anaesthesia dolorosa. Additionally there are the long term concerns about

irradiation.
Decompression of the Nerve

The operation of "neurovascular (nerve vessel) decompression" was developed

in a bid to provide pain relief without nerve damage and consequent numbness

or risk of anaesthesia dolorosa.

In this procedure the blood vessel found compressing the nerve as it emerges

from the brain stem is mobilised free from the nerve and a soft pad placed

between them (see earlier section entitled "What Causes It?"). In 90-95% of

patients immediate pain relief is provided. Most published accounts have shown

that about 75% of patients will still be pain free some three to five years later.

Whilst recurrence may still occur after 5 years of relief rarely does the

procedure produce loss of sensation and no incidence of anaesthesia dolorosa

has been reported.

Of course the disadvantage of this procedure is that it again requires a formal

operation and the risks attendant on this are the same as those described earlier

in Section C. The same opening is made and again you are in hospital for five to

ten days.

In perhaps 10% to 15% no blood vessel is found or one that is very small and

thought not to be significant. In these circumstances I would routinely partially

section the nerve so that some relief is gained by the operation. (see Section C)

In some special circumstances patients elect not to have this done in the event of

a negative exploration and can then expect to still have pain thereafter. Some

surgeons only offer a decompression if the MRI shows a blood vessel. However
sometimes these show a vessel which in reality is not there and on others no

vessel when one in fact is present i.e., MRI scans of the small, deep vessels may

yield false positive and false negative results. Whilst the accuracy of MRI is

increasing I prefer that you give me consent to section the nerve if I feel it

necessary during the operation.

What Should I Have Done?

If your troubles are controlled by medication then this is certainly the best mode

of treatment. Broadly speaking, two groups of patients go forward for other

treatments; those in whom the tablets never gain control of the pain and those in

which the medications cause intolerable side effects. In these eventualities you

will need to give careful consideration to which treatment you would wish to

have. This is something you will need to discuss with a specialist.

A sensible guiding principle is to put aside the theories relating to the cause of

trigeminal neuralgia and concentrate on weighing the potential benefits and

risks of the various treatments. How likely is it to work, for how long will the

benefit last and to what risk does it expose me as an individual?

A general recommendation has been that for young, healthy patients the

neurovascular decompression operation is the most appropriate. It provides pain

relief without numbness and usually does so in a lasting manner. For those who

are unwell, are elderly or frail, cannot afford the time or are unwilling to take

the risk the usual suggestion is to treat the condition with simpler techniques

(see sections A&B). The benefits will usually be of lesser duration and will

usually then come with an associated numbness. However the procedure can be

repeated and subsequently one can go on for more definitive surgery.

You will need to consider these factors carefully and should feel under no

pressure to accept one or the other as you will receive good support whatever

your decision. All the treatments are available.

There are two other guiding principles when considering intervention beyond

medication that I feel are relevant.

Medications can cause serious side effects especially when taken in high doses

and for long periods. People may even suffer disastrous complications to blood

and liver function and some die each year from the anticonvulsant drugs listed

above. In many studies patients who have had interventions reflecting on their

time on medication wish they had had the surgery sooner. “Coming off the

drugs gave me my life back”

By contrast there are those who rue the day they had surgery. Complications

may occur and the operation may fail. Both misadventures may arise in the

same sole and their lot is a very unhappy one. In addition, however big the

operation and however good its statistics are, no procedure can be said to cure

the condition. There is always a chance it may fail even after years of relief. The

treatments, medical or surgical, should be considered as containment not cure.

Finally, I feel I have given a balanced view. However, you may well benefit

from talking to others and obtaining a second opinion. The latter never bothers

me. So, if you are uncertain do not be afraid to ask me to arrange extra help. As

usual the Brain and Spine Foundation offer helpful advice though I should point

out that I was heavily involved in the writing of their booklet on the condition.

(The Brain and Spine Foundation contact details are given at the end of this

guide)
GLOSSOPHARYNGEAL NEURALGIA

This is a much rarer condition and may often be confused with trigeminal

neuralgia. The pain is of an identical nature though affects a different area.

Glossopharyngeal neuralgia is usually felt at the base of the tongue, the back of

the throat and may radiate to the ear. It affects only one side of the head. The

same areas are affected by hypersensitivity and the pain may therefore be

triggered by touching the ear or swallowing.

The investigations of this pain will be along the same lines as that for trigeminal

neuralgia. The medicines used in its treatment are also the same as those used in

trigeminal neuralgia. Because of its rarity surgical treatment for those patients in

whom the pain breaks through the medications is less established. You will

certainly need to discuss this carefully.

POST-HERPETIC NEURALGIA

What Is It?

This is a form of chronic facial pain which follows an attack of shingles on the

face.

Shingles is caused by the chicken pox virus which most people get as a child.

Also known as the herpes zoster virus, shingles usually occurs in older people

when the organism has been lurking in the body for many years and suddenly

attacks a single nerve. It will most often affect a nerve supplying the skin of the

trunk though it affect a nerve supplying the head and face. It will never cross

from one side of the face to the other.

The illness follows a common pattern. It begins with a dull pain in the skin over

the face to be followed within a few days by the eruption of a weeping rash.

During this acute phase the pain may be very severe though it usually then

settles. It will usually be over within a few weeks. The attack may be shortened

by the use of certain medications like Acyclovir. In some patients the rash may

leave scarring though for the vast majority the pain settles and the rash goes

away.

Rarely, the pain may continue and it is this chronic pain which is called post-

herpetic neuralgia. The pain is usually of a burning, aching or throbbing nature

and may often be associated with extreme tenderness. Rubbing of clothes or

wind blowing against the face may be unbearable. Oddly, firm pressure may be

well tolerated. Despite the extreme tenderness in the affected area ordinary

sensation may in fact be blunted.

As with many chronic pains the symptoms may be worsened by physical or

mental stress. In addition sleep is often interrupted as bedclothes brush against

the face.

Who Gets Post-Herpetic Neuralgia?

Anyone who has had shingles may get post-herpetic neuralgia though in reality

it is very rare for young patients to develop it. Probably 100,000 people per year

develop post-herpetic neuralgia on some part of the body, though only a small

percentage of these patients will have it affecting the face.

What Is The Cause Of Post-Herpetic Neuralgia?

The virus damages the nerve and as a result the area supplied by that nerve goes

partly numb. However the damaged nerve pathways then start to generate pain.

Precisely how this comes about is unknown.

What Treatments Are Available?

The effects of treatment are much better when given early. If after an attack of

shingles on the face you still have pain a month after the rash has settled you

should go to your doctor immediately.

Ordinary painkillers usually have little effect and so may in fact only waste

valuable time.

The principal drug used for this condition is Amitriptyline which is a drug more

commonly used for depression, though has very powerful effects on certain

forms of nerve pain.

You may need to take the Amitriptyline for two or three weeks before it

provides relief.

Other drugs such as creams and lotions, or the anticonvulsant medication

Carbamazepine (Tegretol) may also be used.

Surgical procedures to cut the nerve, such as those used in trigeminal neuralgia,

are always ineffective.

OTHER CAUSES OF CHRONIC FACIAL PAIN

These conditions are considered together because they have many common

features. They are not necessarily rarer than the conditions considered in the

previous sections.

Temporo-mandibular Joint Dysfunction

This condition is sometimes also called “facial arthromyalgia” or “Costen’s

Syndrome”.

The pain is in the form of a dull ache which affects the jaw and muscles over

the side of the face. It may also cause clicking of the jaw joint and difficulty in

opening the mouth because of spasm in the jaw muscles. The pain may extend

over the side of the head and down into the neck. Often pain may be felt in the

ear where there may also be a sense of fullness or buzzing. Sometimes dizziness

may occur.

The cause of this pain is unknown though for some the problem relates to

disease in the jaw joint. It may also occur when the teeth do not align properly.

This can happen when teeth have been lost or if dentures do not fit well. The

treatment must therefore begin with a careful assessment from a dental

specialist.

Atypical Odontalgia

Odontalgia is a medical word for toothache. Atypical odontalgia is a severe

discomfort in the teeth or a tooth socket in the absence of any of the usual dental

causes. Oddly the pain may be made worse if dental treatment is pursued though
it is very common for teeth to have been removed by the time the diagnosis

becomes clear.
Oral Dysaesthesia

This is a group of problems which include a burning or altered sensation in the

tongue and gums. It may be associated with a nasty taste. Some patients have a

sense that they produce too much or too little saliva. Dentures, crowns and

bridges may become uncomfortable such that it is impossible to wear them

despite all attempts to modify the shape.

Some sufferers may develop a “phantom bite” whereby they feel the teeth do

not meet properly.

How Are These Conditions Diagnosed?

As with trigeminal neuralgia the diagnosis is made on clinical grounds. That is

to say there is no specific test that will prove the existence of any of these

conditions.

Very careful examination of the mouth and the associated structures will be

required by a relevant dental or medical specialist. Often investigations such as

X-rays or scans may be performed, though these are to exclude other conditions

and may well be normal. On other occasions abnormalities may be shown

which are not relevant to your condition. The pain may be very severe and there

is a temptation for patient and doctor alike to clutch at any anomaly seen on an

X-ray or scan. Blind alleys of treatment may be pursued with failures then

increasing the distress of all concerned. Usually this is not now a problem and

once the diagnosis has been established and other problems excluded treatment

may begin.
What Causes The Pain?

It is true to say that a full understanding of these conditions is not yet available.

However, it is clear that these pains are not imagined and are very real. Whilst

the trigger in many cases is unknown the pain may be mediated by cramped

muscles and dilated blood vessels. As with many chronic pain states they tend

to be exacerbated by tiredness, intercurrent illness and stress.

What Treatments Are Available?

Simply to have an accurate diagnosis and some understanding of the condition

often leaves many sufferers much better able to cope with their pains. Treatment

is based on medications often supported by “pain management techniques” (see

below). There is no operative solution to these problems though occasionally

allied pathologies may require treatment. You may need to see several specialist

before the correct advise is found.

What Medications Are Used?

The most frequently used medications are anti-depressant drugs. These are NOT

prescribed because it is felt that you are necessarily depressed, but because

these drugs are now known to have a specific effect on certain forms of chronic

pain. If the pain has made you depressed then they will of course help that

problem too. The most commonly used medicine is Amitriptyline or

Nortriptyline. They will often need to be taken for several months before they

become fully effective, and they may need to be taken for a year if the course is

to be of lasting benefit.
These are “safe” drugs which very seldom have serious side effects though

frequently can cause mild drowsiness, a dry mouth and occasionally

constipation. They are best avoided if you suffer from certain conditions such as

glaucoma, or prostate problems.

The effects of drowsiness and constipation may be worse in the elderly. Your

doctor will advise you.

Other related drugs may be used if side effects are encountered or anticipated.

In addition if the pain is severe at night occasional night sedation will be

required.

What Are Pain Management Techniques?

Pain can feel worse if you feel it has become out of control and if stress

develops. These pain management techniques aim to teach you methods of

coping better with the pain when it breaks through the treatments. They aim also

to help you better control stress and thus prevent this from exacerbating the

pains. These methods are often taught by Psychologists. A referral to a Clinical

Psychologist should NOT be taken as meaning that the doctor believes your

pains are due to some psychiatric disorder. People with chronic pain of any

source can gain benefit from such treatment.

The treatment sessions will usually consist of teaching you relaxation

techniques and coping strategies for when the pain is severe. You may need to

attend on several occasions so the idea is for you to learn the methods for

yourself.

It is true to say that the use of these techniques varies very much from one

specialist unit to another. However they are becoming increasingly popular and

you will be able to discuss them with your doctor.

What Other Help Is Available?

There is a self-help group for Trigeminal Neuralgia sufferers in the UK run by

Mrs. A. Conn, 27 St. Kenya Avenue, Hove, East Sussex, BN3 4PN.

There is an American Trigeminal Neuralgia Self-help Group who may be

contacted at the Trigeminal Neuralgia Association, P.O. Box 340, Barnegat

Light, NJ 08006, USA

Phone: USA - (609) 361 1014 Fax: USA - (609) 361 0982

There is a free information pack provided by the Brain and Spine Foundation

which covers this problem at www.brainandspine.org.uk

The Brain and Spine Foundation is a charitable organisation and they would

receive any contribution you can make with gratitude. They fund valuable

research and education programs into the neurological disorders.

I am unaware of Self-help Groups relating to the other conditions covered in

this booklet and would be pleased to hear from you should you find one.

Finally, always consider talking to your General Practitioner. They help people

every day in making decisions about complex medical issues. They will also be

able to place your problems within the context of any other health issues you

have.

* All copyright reserved for Mr P J Hamlyn. The information sheets provided

by Mr Hamlyn are intended for use by his patients and need to be read in

conjunction with the conversations they have with him and members of his

team. It is not advised that they are used outside of this context.
What is trigeminal neuralgia (TN) and what are the symptoms?

Neuralgia means pain coming from a nerve. In TN you have sudden pains that

come from one or more branches of the trigeminal nerve. The pains are usually

severe. The second and third branches are the most commonly affected.

Therefore, the pain is usually around your cheek or jaw or both. The first branch

is less commonly affected so pain over your forehead and around your eye is

less common. TN usually affects one side of your face. Rarely, both sides are

affected.

The pain is stabbing ("like electric shocks"), piercing, sharp, or knife like. It

usually lasts a few seconds but can last up to two minutes. The pain can be so

sudden and severe that you may jerk or grimace with pain. The time between

each pain may be minutes, hours, or days. Sometimes several pains repeat in

quick succession. After an attack of pain you may have a dull ache and

tenderness over the affected area which soon eases. However, constant pain in

the face is not usually a feature of TN.

You may have 'trigger points' on your face where touch or even a draught of air

can trigger a pain. These are often around the nose and mouth. Because of these,

some people do not wash or shave for fear of triggering a pain. Eating, talking,

smoking, brushing teeth, or swallowing may also trigger a pain. Between

attacks of pain, there are usually no other symptoms, the nerve works normally,

and a doctor's examination would find no abnormality.

Etiology of trigeminal neuralgia

Extracranial causes:

Vascular Factors

Dental Etiology –Westrum & Black -1976

Infections

Post Traumatic Neuralgia

Viral Etiology

Ratner’s jaw bone cavities -1979

 PATHOPHYSIOLOGY

 TYPES OF TRIGEMINAL NEURALGIA:

Idiopathic (ITN)

Pathologic /Symptomatic (STN)

Etiology

-Multiple sclerosis

-Basiliar artery aneurysm

-Posterior fossa tumour

-Cerebellopontine angle tumours

-Brain stem diseases from stroke or expanding lesions.

 What Questions You Should Ask?

H/o Trauma to face/tooth

Recent Tooth infection Or RCT

Tooth extraction on same side as your facial pain sometimes can cause pain in

area of missing tooth.

Any area of painful facial blisters.(Herpes, Shingles)

History of pain in TN

Site: Trigeminal nerve.

Duration: Paroxysmal

Character: Sharp, shooting, electric shock like.

Severity:Very severe, suicide.

Relieving factors: Sleep, local anesthesia.

Associated factors: Wt.loss, anxiety, depression.

Provoking factors: Non noxious stimuli

Examination: No gross neurological deficit.

 Clinical Features:

Incidence – About 4 in 100000.

Age –Late Middle Age or Later in life( Fifth to sixth

decade).

Sex – Female (58%).

Side –Right (60%).

Clinical Features
Bilateral Cases-3%

Division of involvement – Max - 66%

- Mand - 49%

- Opthal –16%

- Both Max & Mand –19%

- All 3 Divisions- 1%

(Katusic et al -1990)

 Clinical Features:

Sudden, Unilateral, Intermittent, Paroxysmal, Sharp, Shooting, lancinating,

Shock like pain Elicited By touching TRIGGER POINTS.

Pain usually Unilateral But occasionally Spread to An Adjacent Division.

Pain Is Of Short Duration & Lasts for few Sec.

Some Patients – Dull Ache In B/W Attacks.

Paroxysms Occur in cycles, Each cycle lasts for wks or Months.

 Clinical Features:

Extreme cases –Frozen or mask like face.

Pain on touching, chewing, speaking, smiling, brushing, shaving, washing of

face and even on exposure to wind.

V2- Skin of upper lip, ala nasi, cheek or upper gums.

V3 – Lower lip, Teeth or gums of lower jaw.

Tongue is rarely involved.

V1- Supraorbital ridge of affected side.

Attacks do not occur during sleep.

International Headache Society criteria for TN:

Paroxysmal attacks of facial pain that lasts from few sec to < 2 min.

Pain has at least 4 of following characteristics:

a.Distribution along 1 or more divisions of Tri.N.

b.Sudden, intense, sharp, superficial, stabbing or burning quality.

c.Severe intensity.

d.Precipitation from trigger areas or by certain daily activities, such as

eating, talking, washing the face or cleaning the teeth.

e.The pt is entirely asymptomatic b/w paroxysms

Criteria for TN

No neurologic deficit.

Attacks are stereotyped in individual patient.

Exclusion of other causes of facial pain by history, physical examination

findings & special investigation. (when necessary).

-DCNA-2005

 Diagnosis:

History

Examination of cranial nerve

Diagnostic nerve blocking.

Blood count & liver function tests.

CSF studies or serology relevant to certain collagen vascular diseases.

Brain MRI with contrast.

RECENT METHODS OF DIAGNOSIS OF TN.

Brainstem reflexes.

Quantitative sensory thresholds.

Positron Emission tomography (PET)

Functional magnetic resonance(fMRI)

imaging.
Neurography.

Electroencephalography

Magnetoelectroencephalography.

Differential diagnosis

Tri neuralgia PHN

Minor injury

V2 and V3

Localised tactile trigger areas.

Shocklike pain

Demyelination of nerve root

Decompression of vessels

Carbamazepine
Major injury

V1

Non noxious tactile trigger

Shocklike pain

Damage to dorsal horn, nerve root

Dorsal root entry zone lesions

Amitriptyline

Differential diagnosis:

2. Dental pain

3. Trigeminal neuropathy

4. Post traumatic neuralgia

5. Multiple sclerosis
Differential diagnosis

Multiple sclerosis:

- 2% cases

- Earlier age of onset

- Symptoms are bilateral

- Pain severe and lancinating

- Trigger zones are absent.

Differential diagnosis

6. Gloosopharyngeal neuralgia

7. Atypical facial pain

8. Lyme disease

9. Cluster headache

Tri neuralgia Atypical facial pain

Along the distribution of nerve

Age-50-70 yrs

Sec to 1-2 min

Severe

Sharp, stabbing

Terrifying, blinding

Trigger points present

Signs of TIC

Carbamazepine

Do not follow anatomic pathway

Around 40 yrs

Constant

Moderate to severe

Diffuse, burning

Excruciating

Trigger seldom present

Allodynia, paraesthesia

Tricyclic antidepressants

Differential diagnosis

10.Chronic paroxysmal hemicrania

11.Charcot Marie tooth syndrome

12. SUNCT syndrome

Differential diagnosis
13. Raeder syndrome

14. Gradenigo syndrome:

- Suppurative otitis media

- Abducent nerve palsy

- Trigeminal nerve pain

What causes trigeminal neuralgia?

About 9 in 10 cases are caused by a blood vessel pressing on the root of the

nerve where the nerve comes out from the brain through the skull. However, it

is not known why a blood vessel should start to press on the trigeminal nerve in

later life. Rarely, TN is a symptom of another condition. For example, TN may

develop as a result of a tumour, multiple sclerosis, or an abnormality of the base

of the skull. In some cases the cause is not known.

Who gets trigeminal neuralgia?

TN is uncommon. About 10 people in 100,000 develop it each year. It mainly

affects older people, and it usually starts in your 60s or 70s. It is rare in younger

adults. Women are more commonly affected than men.

How does trigeminal neuralgia progress?

A first attack of pain usually occurs 'out of the blue' for no apparent reason.

Further pains then come and go. The frequency of the pains varies from up to a

hundred times a day, to just an occasional pain every now and then. This first

'bout' or 'episode' of pains may last days, weeks, or months, and then typically

the pains stop for a while.

Further bouts of pain usually develop sometime in the future. However, several

months or even years may pass between bouts of pains. It is impossible to

predict when the next bout of pains will occur, or how often the bouts will

recur. Bouts of pains tend to become more frequent as you become older.

Are there any complications?

The pain itself can be severe and distressing. If left untreated, this may make

you depressed or anxious. You may neglect to clean your teeth or not eat for

fear of triggering the pain. This can lead to weight loss and poor mouth hygiene.

However, in most cases where the cause is due to pressure from a blood vessel,

there are no complications affecting the trigeminal nerve itself or affecting the
brain.

In the small number of cases where TN occurs as a result of another condition,

then other symptoms and problems of the condition may develop. For example,

as mentioned, a rare cause of TN is multiple sclerosis. In this situation, other

symptoms and problems associated with multiple sclerosis are likely to develop.

Do I need a brain scan or other tests?

Often not. The diagnosis of TN is based on the typical symptoms. A 'typical'

person with TN is an older person, has classic symptoms, has no other

symptoms to suggest an underlying disease such as multiple sclerosis, and

medication works well (see below). In this typical situation, tests are not usually

needed. However, an MRI scan (magnetic resonance imaging) may be

considered in some cases. For example, this may be when:

 The diagnosis is in doubt (if there are non-typical symptoms).

 An underlying cause is suspected (apart form the usual cause of a

pressing blood vessel).

 TN occurs in a younger person (younger than about 40).

 The condition does not improve with drug treatment (see below).

 Surgery is being considered as a treatment.

Your doctor will advise if you need an MRI scan.

Signs and symptoms

Trigeminal neuralgia

This disorder is characterized by episodes of severe facial pain along the

trigeminal nerve divisions. The trigeminal nerve is a paired cranial nerve that

has three major branches: the ophthalmic nerve (V1), the maxillary nerve (V2),

and the mandibular nerve (V3). One, two, or all three branches of the nerve may

be affected. Trigeminal neuralgia most commonly involves the middle branch

(the maxillary nerve or V2) and lower branch (mandibular nerve or V3) of the

trigeminal nerve.[8]

An individual attack usually lasts from a few seconds to several minutes or

hours, but these can repeat for hours with very short intervals between attacks.

In other instances, only 4-10 attacks are experienced daily. The episodes of
intense pain may occur paroxysmally. To describe the pain sensation, people

often describe a trigger area on the face so sensitive that touching or even air

currents can trigger an episode; however, in many people, the pain is generated

spontaneously without any apparent stimulation. It affects lifestyle as it can be

triggered by common activities such as eating, talking, shaving and brushing

teeth. The wind, chewing, and talking can aggravate the condition in many

patients. The attacks are said by those affected to feel like stabbing electric

shocks, burning, sharp, pressing, crushing, exploding or shooting pain that

becomes intractable.

The pain also tends to occur in cycles with remissions lasting months or even

years. 1–6% of cases occur on both sides of the face but extremely rare for both

to be affected at the same time.[9] This normally indicates problems with both

trigeminal nerves, since one serves strictly the left side of the face and the other

serves the right side. Pain attacks are known to worsen in frequency or severity

over time, in some people. Pain may migrate to other branches over time but in

some people remains very stable.[10]

Rapid spreading of the pain, bilateral involvement or simultaneous participation

with other major nerve trunks (such as Painful Tic Convulsif of nerves V & VII

or occurrence of symptoms in the V and IX nerves) may suggest a systemic

cause. Systemic causes could include multiple sclerosis or expanding cranial

tumors.[11]
The severity of the pain makes it difficult to wash the face, shave, and perform

good oral hygiene. The pain has a significant impact on activities of daily living

especially as people live in fear of when they are going to get their next attack

of pain and how severe it will be. It can lead to severe depression and

anxiety.[12]

However, not all people will have the symptoms described above and there are

variants of TN. One of which is atypical trigeminal neuralgia ("trigeminal

neuralgia, type 2" or trigeminal neuralgia with concomitant pain ),[13] based on a

recent classification of facial pain.[14] In these instances there is also a more

prolonged lower severity background pain that can be present for over 50% of

the time and is described more as a burning or prickling, rather than a shock.

Trigeminal neuropathic pain is similar to TN2 but can have the electric pulses

associated with classic TN. The pain is usually constant and can also give off a

tingling, numbness sensation. This pain is due to unintentional damage to one or

more of the trigeminal nerves from trauma, oral surgery, dentistry work, etc. It

is difficult to treat but sufferers are usually given the same anticonvulsant and

tricyclics antidepressant medicines as with the other types of neuralgias.

Surgical options are DREZ (dorsal root entry zone) lesion and MCS or Motor

Cortex Stimulation.

TN needs to be distinguished from other forms of unilateral pain which are

related to damage to the trigeminal nerve by trauma to the face or dental

treatments. This is often termed painful trigeminal neuropathy or post-traumatic

neuropathy as some sensory changes can be noted e.g. decrease in pain

sensation or temperature. This is important as different care pathways are used.

Trigeminal pain can also occur after an attack of herpes zoster, and post-

herpetic neuralgia has the same manifestations as in other parts of the body.

Trigeminal deafferentation pain (TDP), also termed anesthesia dolorosa, is from

intentional damage to a trigeminal nerve following attempts to surgically fix a

nerve problem. This pain is usually constant with a burning sensation and

numbness. TDP is very difficult to treat as further surgeries are usually

ineffective and possibly detrimental to the person.

Society and culture

Some individuals of note with TN include:

 Entrepreneur and author Melissa Seymour was diagnosed with TN in

2009 and underwent microvascular decompression surgery in a well

documented case covered by magazines and newspapers which helped to

raise public awareness of the illness in Australia. Seymour was

subsequently made a Patron of the Trigeminal Neuralgia Association of

Australia.[41]
 Salman Khan, one of India's biggest film stars, was diagnosed with TN in

2011, resulting in tremendous media coverage in the country and abroad.

He underwent surgery in the US.[42]

 All-Ireland winning Gaelic footballer Christy Toye was diagnosed with

the condition in 2013. He spent five months in his bedroom at home,

returned for the 2014 season and lined out in another All-Ireland final

with his team.[43]

 Jim Fitzpatrick - Member of Parliament for Poplar and Limehouse -

disclosed he suffered from trigeminal neuralgia before undergoing

neurosurgery. He has openly discussed his condition at parliamentary

meetings and is a prominent figure in the TNA UK charity.[44]

 Andrea Jenkyns - Member of Parliament for Morley and Outwood -

diagnosis with TN came to light during her television debate on Prime

Minister’s Questions where she struggled to get her words out.[45]

 Jefferson Davis - President of the Confederate States of America [46]

 Charles Sanders Peirce - American philosopher, scientist and father of

pragmatism. (Joseph Brent, Charles Sanders Peirce: A Life

[Bloomington: Indiana University Press, 1993] p. 39-40)

 Gloria Steinem - American feminist, journalist, and social and political

activist [47]
  Anneli van Rooyen, Afrikaans singer-songwriter popular during the

1980s and 1990s, was diagnosed with atypical trigeminal neuralgia in

2004. During surgical therapy directed at alleviating the condition

performed in 2007, Van Rooyen suffered permanent nerve damage,

resulting in her near-complete retirement from performing.[48]

 H.R., singer of hardcore punk band Bad Brains

What are the drug treatments for trigeminal neuralgia?

Carbamazepine is the usual treatment

Carbamazepine is classed as an anticonvulsant drug. It is normally used to treat

epilepsy. TN is not epilepsy. However, the effect of carbamazepine is to quieten

nerve impulses and it often works well for TN. There is a good chance that

carbamazepine will ease symptoms of TN within 1-2 days. A low dose is started

and built up gradually until a dose is reached that stops the pains. You should

then take it regularly to prevent pains from returning. The dose of

carbamazepine needed to control the pains varies from person to person.

It is common to take carbamazepine until about a month after the pains have

stopped. The dose may then be reduced gradually, and stopped if possible. After

this there is often a period when pains do not occur for some time (remission).

However, the pains are likely to return sometime in the future. Treatment can

then be restarted. Some people find that carbamazepine works well at first but

less well over the years.

Side-effects occur in some people who take carbamazepine. Side-effects are

more likely if higher doses are needed. Read the drug packet leaflet for a full list

of possible side-effects. The most common include: drowsiness, feeling sick,

tiredness, and dizziness. Quite often these are only temporary, so it is worth

persisting with the drug if the pains ease and side-effects are not too bad.

Rarely, carbamazepine can cause serious blood or liver problems. Therefore,

tell your doctor if you develop any of the following whilst taking this drug:

fever, sore throat, ulcers in your mouth, unexplained bruising or bleeding,

yellowing of your skin, a rash - particularly if the rash is of small purple spots,

peeling of the skin, abdominal pain, nausea or vomiting. (These symptoms may

be due to blood or liver problems caused by medication.)

Other drugs
Other drugs may be tried if carbamazepine does not work well or causes bad

side-effects. These include drugs that 'quieten' nerve impulses. For example,

gabapentin, oxcarbazepine, baclofen or lamotrigine. A combination of two

drugs is occasionally tried if one alone does not help.

Normal painkillers such as paracetamol or codeine do not work for TN.

Surgical options for treatment

An operation is an option if medication does not work or causes troublesome

side-effects. Basically, surgery for TN falls into two categories:

Decompression surgery

This means an operation to relieve the pressure on the trigeminal nerve. As

mentioned earlier, most cases of TN are due to a blood vessel in the brain

pressing on the trigeminal nerve as it leaves the skull. An operation can ease the

pressure from the blood vessel (decompress the nerve) and therefore ease

symptoms. This operation has the best chance of long term relief of symptoms.

However, it is a major operation involving a general anaesthetic and 'brain

surgery' to get to the root of the nerve within the brain. Although usually

successful, there is a small risk of serious complications such as a stroke or

deafness following this operation. A very small number of people have died as a

result of this operation.

Ablative surgical treatments

Ablative means 'to destroy'. There are various procedures that can be used to

'destroy' the root of the trigeminal nerve, and thus ease symptoms. For example,

one procedure is called stereotactic radiosurgery (gamma knife surgery). This

uses radiation targeted at the trigeminal nerve root to destroy the nerve root. The

advantage of these ablative procedures is that they can be done much more

easily than decompression surgery as they do not involve formal 'brain surgery'.

So, there is much less risk of serious complications or death than there is with

decompression surgery. However, there is more of a risk that you will be left

with a lack of sensation in a part of your face or eye as the treatment may mean

that the trigeminal nerve will not function normally again. Also, there is a

higher chance that the symptoms will return at some stage in the future

compared to decompression surgery.

The chance of a cure from both decompression and ablative treatments is good.

But, there are pros and cons of each. If you are considering surgery, the advice

from a specialist is essential to help you decide which procedure is best for you.
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