TRIGEMINAL NEURALGIA

DEFINATION:

ACCORDING TO IASP (INTERNATIONAL ASSOCIATION FOR THE STUDY

OF PAIN):

A sudden , usually unilateral , severe , brief , stabbing , recurrent

pain in the distribution of one or more branches of the fifth cranial
nerve.
IHS (INTERNATIONAL HEADACHE SOCIETY) DEFINATION :

Painful unilateral affliction of the face characterized by brief

electric shock like (lancinating) pains limited to the distribution of
one or more divisions of the trigeminal nerve. Pain is commonly
evoked by trivial stimuli including washing , shaving , smoking ,
talking and brushing the teeth but may also occur spontaneously.
The pain is abrupt in onset and termination and may remit for
varying periods.

• Both IASP and the IHS classification recognize two forms of

trigeminal neuralgia:
1. Trigeminal neuralgia or Tic Douloureux is trigeminal
neuralgia of unknown etiology.
2. Secondary or symptomatic trigeminal neuralgia is related
either to central nervous system lesions (tumours or
aneurysms ) or to local facial trauma and is much rarer.
 EPIDEMIOLOGY
• Trigeminal neuralgia is a rare condition. Mac Donald et al.
(2000) assessed the incidence & lifetime prevalence of
neurological disorders in a UK community based study and
found a lifetime prevalence of 0.7/1000 for trigeminal
neuralgia.
• The peak incidence is in 50-60yrs of age group and it
increases with age.
• Women are more likely to get trigeminal neuralgia but it may
be age related .
• At increased risk of developing trigeminal neuralgia are
patients with multiple sclerosis and hypertension .
Hooge & redekop (1995) found in
their 1882 population of multiple sclerosis patients 35(1.9%)
that has trigeminal neuralgia. In 5 of them the trigeminal
neuralgia was the first symptom appearing between 1 and
11 yrs after the diagnosis of multiple sclerosis whereas in 30
it was 3-28 yrs after the multiple sclerosis diagnosis. Two
indicators they identified were younger age and a higher
incidence of bilateral cases(14%).
AETIOLOGY AND PATHOPHYSIOLOGY

• Determining the aetiology of trigeminal neuralgia has been

difficult due to the lack of an animal model but over the
years considerable progress has been made.
A hypothesis for the mechanism of pain generation in
trigeminal neuralgia has been put forward but more clinical
evidence is needed to prove it .
• Following summary of the findings are taken from the
articles by Devor et al (2002) and Love @ Coakham (2001) &
Nurmikko & Eldridge(2001) :-
Most researchers will agree that the most likely site for the
generation of trigeminal pain is in the nerve itself at the
point called the Root Entry Zone (REZ). This is a point at
which the peripheral and central myelins of Schwann cells
and astrocytes meet. This is a vital area as any changes here
result in altered function of the whole neurone. There is
clinical evidence to show that compression of the nerve at
the REZ by blood vessels or tumours does occur. It has also
been shown that plaques of demyelination such as seen in
multiple sclerosis are found in this area. The subsequent
nerve injury results in abnormal firing of the nerve which has
led to the ignition hypothesis (Devor et al 2002). The injured
afferents becomes hyperexcitable and if the after-discharge
becomes sufficiently large it results in a nociceptive signal
being perceived as pain.
It is further postulated that this
constant barrage of impulses can eventually lead to central
sensitization and hence a change in the character of the pain
to the more atypical type which has a continuous
background element similar to other neuropathic pains.
• This hypothesis allows us to explain why the various
treatments all results in pain relief but it also suggests
 that surgical treatment should be carried out earlier
rather than later. Evidence for this needs to be
collected from clinical material where patients pain and
sensory changes have been carefully monitored over a
longer period of time.
Drug therapy attempts to reduce this hyperexcitability.
• Decompression of the nerve allows for return to normal
function , prevents further damage and enables
remyelination to occur. All the other surgical procedure
result in destruction of the relevant fibres and so
prevent pain being transmitted. These later treatments
therefore all results in a element of sensory loss may
allow the process to continue.
However , patients still get recurrences of pain even
after decompression and negative findings on re-
operation and this probably reflects the neuroplasticity
of the nervous system.
CLINICAL FEATURES
1. CHARACTER:
• There is overall agreement that the pain of trigeminal
neuralgia has a sharp quick quality to it.
• Szapiro et al(1985) reported that in 26/70 of their pre
surgical patients there was a background pain that was
not always just dull and aching but at times throbbing
and burning and of considerable intensity.
• Nurmikko and Eldridge(2001) describe the pain in their
atypical trigeminal neuralgia patients as having
additional features of burning and smarting and they
report it as being less severe.
Those with trigeminal neuropathy
also have shooting pain but in addition describe their
pain as dull, smarting and steady duration.
1. PERIODICITY, TIMING, DURATION , ONSET
 • Trigeminal neuralgia is paroxysmal and there are
periods of complete freedom of pain.

Penman (1968) made a distinction between (a) Paroxysms or

short periods when pain is continuous (b) Runs when there
are only brief periods of relief between paroxysms (c) Bouts
when there are longer periods of pain relief but some pain
can still occur (d) Remissions when there is no pain at all.

The paroxysms of pain last for only a few minutes or

even seconds but outlast the provoking stimulus.
• Each attack of pain reaches maximum intensity fairly
rapidly, and then becomes stable before finally
subsiding (Kugelberg and Lindblom 1959) .
The pain spreads rapidly at the beginning of the attack
& then recedes more slowly. The attack is followed by a
refractory period whose length is related to the
intensity and duration of the pain and not to the
stimulus.
• Attacks that occur within the refractory period are of
decreased duration and intensity(Kugelberg and
Lindblom 1959).
• It has remained difficult to determine the frequency and
length of remissions and replaces.
Katusic et al(1990) in their epidemiological survey of 75
patients used Kaplan-Meier life table methodology to
estimate frequency of pain episodes and they predicted
that 65% of patients would have the seconds episodes
of pain within 5yrs and 77% within 10yrs. Age did not
correlate with the timing of the next episode.
• Without using any statistical analyses Rusthon & Mac
Donald(1957) suggest that time of onset of disease, first
presentation and duration of the longest pain free interval
could be used to predict the future course of the condition.
 • They make a very important observation that no treatment
can be deemed successful until it has given pain relief for
over one year.
• Trigeminal neuropathy pain is constant with pain-free
intervals and no refractory periods and progresses slowly
(Nurmikko and Eldridge 2001).
SITE AND RADIATION
• Most series describe the site of pain according to the
anatomy of the trigeminal nerve.
• Henderson (1967) preferred to describe the site of pain not
in terms of divisions, but of “ZONES”.
• BOWSHER (2000) points out that careful questioning of the
patients does suggests that although pain is experienced
intraorally it is felt in the gingival area rather than in tooth
itself.
• The predominant side of involvement is the right although a
review of the literature did show some slight variation
(zakrzewska and Hamlyn,1999) and there does not appear to
be a link with handedness, age or gender (Rothman and
Wepsic 1974).
• There is no evidence from cohort studies that show how
number of divisions involved changes with time.
Rothman & Beckman(1974) using case controls showed in
500 patients that those with lower facial involvement with
younger and tended to be male.

SEVERITY

• The pain of trigeminal neuralgia can be suicidal (Harris,

1926).
• Upto 60% patients report that the pain increases with
severity after the first attack(Bowsher,2000).
PROVOKING AND RELIEVING FACTORS
• One of the diagnostic criteria for trigeminal neuralgia is that
it is light touch provoked.
• Henderson (1967) suggested that stress may be a provoking
factor.
• The stimulus can be mechanical(76%) or thermal(60%).
• Many suggests that the response to Carbamazepine can be
taken as evidence that the correct diagnosis has been made
but from RCT’s case reports between 20 & 30% of patients
may not have a positive response.
ASSOCIATED FACTORS
• Vasodilation and swelling has been reported in patients with
severe pain (Nurmikko et al 2000).
• Patients with trigeminal neuralgia may have hemifacial
spasm.
• Most reports indicate that patients are rarely affected by
pain at night and Bowsher(2000) has reported that flexing of
the head forward brings on an attack of pain.
• Neither IHS nor IASP descriptions provide any indications of
the effect trigeminal neuralgia has on patients quality of life
and psychosocial functioning which may be diagnostic of the
condition.
Bowsher (2000) reported that 72% of their patients reported
severe disturbance in daily living.
• Gordon and Hitchcock (1983) assessed personality and
illness behaviour in patients with facial pain.
• Marbach and Lund (1981) reported that in 89 trigeminal
neuralgia patients (non pain controls used) depression
correlated with pain severity.
FINDINGS ON EXAMINATION
• Several studies have shown that sophisticated sensory
testing picks up some sensory changes but not in all
modalities.
The sensory loss can be in a small area round the trigger
point and too subtle for the patients to notice.
• Bergenheim et al (1997) tested 37 patients prior to surgery
and found on clinical examination that 7 had rduced
 sensation to cotton wool (1 totally impaired ) , 7 had
impaired pin prick sensation (2 totally impaired) and the
corneal reflex was diminished in 1 and totally absent in 1
patient.
• It is important to repeat neurological examinations at
intervals as changes may occur indicating that there is a
secondary cause of trigeminal neuralgia. These sensory
changes may be indicative of more extensive nerve changes
but there are few carefully controlled studies using
independent observers to correlate clinical findings and
outcomes.
CLINICAL FEATURES IN SECONDARY TRIGEMINAL NEURALGIA
• Secondary causes of trigeminal neuralgia include benign or
malignant tumours of the posterior fossa or multiple
sclerosis and these may present later in disease process.
• Meaney et al (1995b) demonstrated that 7 patients with
multiple sclerosis and trigeminal neuralgia had either
tumours or vascular compression on magnetic image
resonance scanning in addition to plaques of multiple
sclerosis.
Broggi et al (1999) have also reported compression in
patients with trigeminal neuralgia and multiple sclerosis.
• Compression of the trigeminal nerve can also occur
intraorally and the most common area is the mental region
where trauma or loss of alveolar bone after tooth extraction
leads to the mental nerve being at risk of compression from
dentures.
INVESTIGATIONS
1) PSYCHOLOGICAL ASSESSMENT :
• The long Mc Gill pain questionnaire which is
multidimensional , has been used to distinguish between
trigeminal neuralgia and atypical facial pain.
• Other quality of life , psychosocial or daily activities
measures are used to gain improved insight on the disability
 caused by the condition and to evaluate treatment outcomes
.
Daily diaries are a way of gaining further diagnostic and
treatment outcome data.
1) HAEMATOLOGICAL AND BIOCHEMICAL INVESTIGATIONS:
• All patients for medical management of trigeminal neuralgia
should have some baseline haematological and biochemical
investigations performed so as to evaluate whether side
effects to therapy are related to biochemical or
haematological effects.
• Investigations should include haemoglobin with a full blood
count , red cell, folate and B12 estimations, urea and
electrolytes as well as liver function tests including gamma
GT.
1) SENSORY TESTING , AUDITORY TESTING , SPEECH
DISCRIMINATION:
• Sensory testing using special equipment is not done
routinely at present but these have showed that patients do
have increased temperature and tactile thresholds.
• Sophisticated sensory testing should be considered in
patients with non classical features or changing symptoms.
• Audiometry and speech discrimination is not done routinely
but this may be necessary if you are trying to assess how
frequent hearing loss is after posterior fossa surgery.
1) RADIOLOGICAL INVESTIGATIONS:
• In patients suspected of also having a dental cause of pain
,intraoral radiographs and rotational tomograms of the upper
and lower jaws are required.
These should be evaluated either by a dentist or a dental
radiologists.
• Computed tomography(CT) scans are useful for picking up
tumours but the most important investigations used in
patients with trigeminal neuralgia are magnetic resonance
imaging (MRI) and fast-inflow steady state precision(FISP).
• The MRI can determine if there are any benign or malignant
lesions present or plaques of multiple sclerosis.
Neurosurgeons will also gain information on the presence or
absence of vessels in contact with trigeminal nerve and this
will help them to determine how to explore the area. It has
now become fairly standard practice to perform an MRI and
FISP prior to performing a microvascular decompression.
• The axial MRI T2 weighted fast spin echo sequences are
used routinely for assessing head & neck pathology and will
pick up structural lesions such as tumours and multiple
sclerosis .
• Enhanced T1-W sequences will give improved sensitivity for
small tumours and meningeal disease , gadolinium being the
contrast agent.
• In order to assess the relationship of vessels with trigeminal
nerve , sequences which are more sensitive to flow have
been utilized e.g. MRA ,3D flash – inflow with steady state
precision (FISP) ,MP-RAGE or constructive interference in
steady state three dimensional Fourier transformation MRI
(CISS).
Arterial contacts are easier to detect than venous ones for
which contrast medium may be required.
• It is important to ensure that not just the root entry
zone(REZ) but also the pontine