Trigeminal neuralgia is an excruciating pain felt in the distribution of the trigeminal or fifth cranial nerve

and is classically described as a brief sharp shooting pain (Kitt et al. 2000). Trigeminal neuralgia has been
defined by the InternationalHeadache Society’s classification subcommittee as (IHS 2004):
A) _ Paroxysmal attacks of pain lasting from a fraction of a second to 2 minutes, affecting one or more
divisions of the trigeminal nerve and fulfilling criteria B and C.
B) Pain has at least one of the following characteristics:
1. intense, sharp, superficial or stabbing
2. precipitated fromtrigger areas or by trigger factors
C) Attacks are stereotyped in the individual patient
D) There is no clinically evident neurological deficit
E) Not attributed to another disorder
Although this definition has never been tested for reliability and proven to be valid, either it, or a
derivation, arewidely accepted and utilized extensively for both research and patient care purposes.
Note: the terms classical trigeminal neuralgia and idiopathic trigeminal neuralgia are used when a
secondary cause for this pain is not readily identifiable. Symptomatic trigeminal neuralgia is used when a
secondary cause for this pain has been identified, such as an aneurysm, tumor or stroke.
Characteristics
Trigeminal neuralgia occurs in approximately 4 per 100,000 people and has an onset later in life, with the
median age of diagnosis being 67 years old. It occurs about twice as often in women as in men (Katusic et
al. 1990), but this may be from sampling error, since good prevalence data do not exist (Zakrzewska and
Hamlyn 1999). This severe pain is described by patients as having an electric or lightening bolt quality
and being brief in duration, lasting less than 30 seconds. Sometimes patients describe a dull, burning or
throbbing pain that occurs between attacks of intense pain. Trigeminal neuralgia pain is most commonly
felt in the maxillary division and to a lesser extent in the mandibulardivision. Pain is infrequently felt only
in the ophthalmic división of the trigeminal nerve. It is almost always unilateral, especially on initial
presentation and does not cross the midline. Theremay be predilection for right-sided pain (Zakrzewska
and Hamlyn 1999), which would suggest an anatomical correlation. Paroxysms of pain may be
spontaneous, occurring without a known reason or triggered by non-painful stimuli such as light touch or
wind on the face, eating and grooming. The triggering area may be the area where the pain is felt or
different, but it is always on the same side as the pain and is often perioral. Local anesthetic applied to the
triggering área will abate the ability to trigger the pain. Once pain is triggered it is self-sustaining and
frequent triggering often results in a decrease in pain intensity in the later bouts of pain. Interestingly,
even though this pain is one of the worst pains imaginable, it is not triggerable during unconsciousness
and rarely wakes people from sleep. Also, trigeminal neuralgia is characterized as having periods of
spontaneous remission, which may last days, months or even years.
Diagnostic Process
The diagnosis of trigeminal neuralgia is based strictly on clinical data, i.e. a history and physical
examination, since there are no laboratory tests or imaging studies that can either confirm or refute its
presence (Merskey and Bogduk 1994; IHS 2004). Brain imaging is frequently ordered to ensure that
trigeminal neuralgia is not caused by an intracranial space-occupying lesión or _ demyelinating process.
Trigeminal neuralgia is 20 times more common in people with multiple sclerosis (Katusic et al. 1990), so
when it occurs in young patients, the appropriate evaluative measures should be performed. Patients
sometimes present to their dentist first, because this pain is frequently felt in the jaws and teeth. Often
their description is of a continuous dull ache, similar to toothache. There is controversy about this pain
because itmayactually be aprodrome totheclassically described trigeminal neuralgia, occurring days to
years before the more recognizable symptoms occur. This prodrome pain has been termed pre-trigeminal
neuralgia and correspondsto the initialpresentation features of trigeminal neuralgia. This concept is
supported by patient reporting and by clinical experience that this prodrome pain responds to
pharmacotherapy like classic trigeminal neuralgia does (Fromm et al. 1990). The opposing opinión is that
the continuous dull ache pain is really of odontogenic origin. Dental interventions, like root canals and
tooth extractions, employed to address pathology result in peripheral nerve injury and this cumulative
injury leads, in part, to the formation of trigeminal neuralgia.
Etiology and Pathophysiology
The etiology of trigeminal neuralgia is not established and little is truly understood about the underlying
pain mechanisms, since a valid animalmodel does not exist. Trigeminal nerve root compression, at the
entry zone into the _ pons, has been observed in patients with trigeminal neuralgia (Dandy 1934). The
anatomical arrangement of blood vessel impingement on the nerve root has also been described in
patients after surgical exploration (Jannetta 1967), but it is not present in all patients with trigeminal
neuralgia and sometimes it is noted in imaging of non-trigeminal neuralgia patients (Majoie et al. 1997).
It has been hypothesized that the presence of _ ectopic action potentials arising from peripheral nerves
and the failure of central inhibition within the trigeminal system occur simultaneously to produce
paroxysmal pain. This explains why certain medications are beneficial and others are not in the treatment
of trigeminal neuralgia (Fromm et al. 1984). Currently, the phenomenon of ephaptic cross talk is used to
explain how non-painful stimuli are translated into painful ones and crossed _ afterdischarge has been
cited as the mechanism implicated in further pain amplification and prolongation. These two assumptions
have been collectively termed the ‘ignition hypothesis’, which defines trigeminal neuralgia as a peripheral
nerve disorder (Devor et al. 2002). This hypothesis explains why people with demyelinating disorders,
such as multiple sclerosis, frequently experience trigeminal neuralgia. It may also account for the clinical
observation that trigeminal neuralgia seems to start after recent dental interventions.The continuous dull
burning background pain sometimes felt by patients would best be explained by the development of
central sensitization secondary to a lack of inhibition, akin to deafferentation pain and other continuous
neuropathic pains.
Definition
The International Association for the Study of Pain (IASP) defines trigeminal neuralgia as “A sudden,
usually unilateral, severe, brief, stabbing, recurrent pain in the distribution of one or more branches of the
fifth cranial nerve” (Merskey and Bogduk 1994).
Characteristics
Epidemiology
The incidence of _ trigeminal neuralgia is 4.3/100,000 (2.96male, 3.47 female/100,000 based on data
from the US). The point prevalence is 0.1%. The peak incidence is in the age group 60–69, and it is rare
in patients under the age of 40. There is a strong link between multiple sclerosis and trigeminal neuralgia,
and hypertension may also be a risk factor.There are little data on the natural history and prognostic
features (_ trigeminal neuralgia, features), but data from the US suggests that it does not affect survival,
although attacksgetmore severewith time (Zakrzewska and Hamlyn 1999).
Etiology and Pathogenesis
As there are no satisfactory animalmodels of trigeminal neuralgia, it still remains difficult to elucidate
fully the aetiology and pathogenesis of trigeminal neuralgia. The ignition hypothesis byDevor et al.
(2002) (_ trigeminal neuralgia, ignitiontheory) isacceptedbymany,although direct support of it from
trigeminal electrophysiological studies is very limited.According to this theory, chronic irritation of the
trigeminal nerve leads to focal demyelination, which results in the generation of ectopic action potentials
and impaired segmental inhibition. This leads to hyper-excitability of the afferents that give rise to pain
paroxysms as a result of synchronised after-discharge activity. This theory is supported by clinical
observations thatpatientswith trigeminalneuralgia inthemajority of cases are found to have blood vessels
compressing the trigeminal nerve, either at the nerve root entry zone or less commonly the brain stem.
Electron microscopic examination of nerve roots taken from patients with such compressions has revealed
focal demyelination in the region of the compression, with close apposition of demyelinated axons and an
absence of intervening glial processes. A process of re-myelination does occur, and this could be
responsible for the spontaneous remission of the neuralgia. The most effective drugs are anticonvulsants,
and they probablywork by suppressing ectopic hyper-excitability in the nerve or central neurons.
Clinical History
The following list provides the diagnostic criteria as suggested by the InternationalHeadache Society
(Anon 1988) (_ trigeminal neuralgia, diagnostic method):
1. Paroxysmal attacks of facial or frontal pain last a few seconds to less than two minutes.
2. Pain has at least 4 of the following characteristics:
– Distributionalongoneormoredivisionsof trigeminal nerve
– Sudden, intense, sharp, superficial, stabbing or burning in quality
– Pain intensity severe
– Precipitation fromtrigger areas, or by certain daily activities such as eating, talking,washing the face or
cleaning the teeth – Between paroxysms entirely asymptomatic
3. No neurological deficit.
4. Attacks are stereotyped in individual patients.
5. Exclusion of other causes of facial pain.
It is essential to take a very careful history, as this is the only reliable method of making the diagnosis. It
is especially important to elucidate the sharpness and paroxismal quality of this pain, which differentiates
it from most other facial pains. A particular feature of trigeminal neuralgia is that it is usually precipitated
from one or more trigger areas (especially in the second or third trigeminal divisions), upon tactile
stimulation or daily activities such as eating, talking,washing or shaving the face or cleaning the teeth.
Each bout of pain is very quick (seconds), but patients may get many of these in quick
succession,andsothepainmayseemtobelastinglonger. The nerve eventually becomes refractory and there is
a period when the patient is pain free. Classically, there are also periods of complete pain remission which
last for weeks or months. These pain remissions gradually get shorter and shorter. The most common
divisions to be affected are the second and third, and it is rare for the first division alone to be affected. In
about 3%ofpatients thepain becomesbilateral,but it isunusual for both sides to be active at the same time.
Other features that need to be assessed include the quality of life and the level of anxiety and depression.
Most patients with severe trigeminal neuralgia find it impossible to socialise because of fear of
developing an attack of pain while eating. These patients will often lose weight and become depressed.
Ideally, patients should be evaluated with standard assessment measures such as the McGill Pain
Questionnaire, some form of anxiety or depression scale such as the Hospital Anxiety and Depression
Scale, and a quality of life assessment such as the Brief Pain Inventory or SF36. Some patients will
have,what are termed, atypical features andwill report a constant, dull, aching background pain, exhibit no
paroxysmal features, or have no pain free periods. Some of these symptomsmay be related to stress and
anxiety. Although 70% of patients will gain relief of pain from the use of carbamazepine, this is not
always diagnostic.