BJA Education Advance Access published April 26, 2016
BJA Education, 2016, 1–4
Matrix reference
ID02, 2E03, 3E00
Trigeminal neuralgia
C K Vasappa MBBS DA FRCA FFPMRCA1, *, S Kapur MBBS MD FRCA FFPMRCA2,
and H Krovvidi MBBS MD FRCA3
1
Consultant Anaesthetics and Pain Management, Russells Hall Hospital, Pensnett Road, Dudley DY1 2HQ, UK,
2
Consultant Anaesthetics and Pain Management, University Hospital Birmingham, Birmingham, UK, and
3
Consultant Anaesthetics, University Hospital Birmingham, Birmingham, UK
*To whom correspondence should be addressed. Tel: 01384244809; E-mail: kumar.vasappa@dgh.nhs.uk
Key points
• Trigeminal neuralgia (TN) is a relatively rare but de-
bilitating facial pain condition.
• Classical TN occurs without any apparent cause
other than possible microvascular compression.
• TN is a clinical diagnosis, but MRI is helpful to ex-
clude secondary causes.
• Many patients respond to pharmacological therapy
and carbamazepine remains the first-line drug.
• Microvascular decompression has the best outcome
in terms of quality and duration of pain relief.
Trigeminal neuralgia (TN) is a characteristic neuropathic pain
involving the trigeminal nerve distribution. The International
Association for the Study of Pain (IASP) defines TN as ‘a unilateral
painful disorder that is characterised by brief, electric shock like
pains, is abrupt in onset and termination, and is limited to the dis-
tribution of one or more divisions of the trigeminal nerve’. The an-
nual incidence of TN in the UK is around 26/100 000.1 Worldwide
prevalence varies from 10 to 300/100 000. The peak age of onset
is between 50 and 60 yr with a male-to-female ratio of 1:2. In
this article, we present a review of the pathophysiology, diagnosis,
and treatment of TN based on available evidence.
Aetiology and pathophysiology
The exact aetiology and pathophysiology of TN remains to be
clearly elucidated. According to the ‘ignition theory’ (the most
common hypothesis), TN is the result of abnormalities in the
afferent neurones of the trigeminal root or ganglion.1 Any injury
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doi: 10.1093/bjaed/mkw015
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to the axons can make them hyperexcitable, leading to this pain-
ful neuropathic condition. It has been suggested that central
sensitization also plays a role in TN. Some of the risk factors in
developing TN are multiple sclerosis (MS), increased age, stroke,
hypertension (in women), Charcot–Marie–Tooth disease, and
tumours in the region of the trigeminal nerve root.
In the majority of TN cases, the cause is thought to be demye-
lination of the trigeminal nerve root near its entry into the pons.2
This area (called the root entry zone) is where the peripheral
myelin of Schwann cells meet the central myelin of the astro-
cytes. The area of nerve root that has been affected can spontan-
eously discharge impulses that trigger TN. A-β fibres (carrying
touch sensation) lie in close proximity with A-δ and C fibres (car-
rying pain sensation) in the root entry zone, leading to ephaptic
cross-talk between them. This might explain precipitation of
attacks of TN by trivial tactile stimulation.1,3 The trigeminal gan-
glion itself can show pathological changes like hypermyelination.4
In many patients, the demyelination is caused by compression by
a vascular structure. MS, tumours, and arteriovenous malforma-
tions can lead to primary demyelination. TN when associated
with MS may show plaques of demyelination at the root entry
zone. These patients present at a younger age and although do
not tolerate anticonvulsants very well, their response to interven-
tions is similar to patients with classical TN.
The theory of vascular compression leading to TN has been
supported by the results of surgical decompression and evidence
from MRI and nerve conduction studies. However, vascular
contact is not a consistent finding in all TN patients and cadaver-
ic studies have shown vascular compression as an incidental
finding in people who did not suffer from TN.4
Diagnosis
TN is essentially a clinical diagnosis based on its characteristic
presentation.
Page 1 of 4
Trigeminal neuralgia
A: Paroxysmal attacks of pain lasting from a fraction of a second to 2
min, affecting one or more divisions of the trigeminal nerve and ful-
filling criteria B and C
B: Pain has at least one of the following characteristics:
Intense, sharp, superficial, or stabbing
Precipitated from trigger 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
Ref: NICE Clinical Knowledge Summaries: Trigeminal Neural-
gia, December 2014.
Differential diagnosis
TN has to be differentiated from other causes of orofacial pain
and headaches.
Headache disorders: cluster headache, trigeminal autonomic ce-
phalalgia
Dental pain: cracked tooth, dental abscess
Temporo-mandibular joint disorders
Other neuralgias: occipital neuralgia, glossopharyngeal neuralgia,
post-herpetic neuralgia, atypical facial pain
Tumours: acoustic neuromas, meningiomas
Sinusitis
Classification
TN is classified5 as:
(i) Classical TN—occurs without any apparent cause other
than microvascular compression. It is further subdivided as:
(a) purely paroxysmal: where the patient is pain free
between attacks and
(b) with concomitant persistent facial pain(also called as
atypical TN or TN type 2): a low-grade background
facial pain persists between the attacks. Central sensi-
tization may account for the persistent pain. Neurovas-
cular compression may not be demonstrable in this
type and is resistant to several treatment modalities.
(ii) Symptomatic TN—caused by another recognizable disorder
that leads to neural damage (e.g. MS, herpes zoster, trauma,
space-occupying lesion).
Investigations
TN is diagnosed on the basis of typical history and clinical fea-
tures. Although there are no specific investigations to confirm
the diagnosis, it may be necessary to undertake some to rule
out symptomatic TN:
(i) A neurological examination would help to detect any
neurological deficit, area of involvement, and trigger areas.
A sensory deficit, absent trigeminal reflexes, and bilateral
involvement suggests symptomatic TN.6
(ii) MRI scanning of the brain is useful to detect any secondary
causes like MS or tumours. Routine neuroimaging may iden-
tify a cause in up to 15% of patients.6,7 High-resolution MRI
may be able to identify vascular compression.2,6
(iii) Evoked potentials, quantitative sensory testing, and electro-
physiological studies can also help detect symptomatic TN,
but as yet do not have enough evidence to be routinely
recommended.2,6
(iv) Preoperative magnetic resonance tomographic angiography
has been suggested as useful in patient selection and out-
come prediction for microvascular decompression (MVD).7
Page 2 of 4 BJA Education | 2016
Relevant clinical anatomy
The trigeminal nerve is the fifth and largest cranial nerve and
composed of both sensory and motor components. The sensory
nuclei are present throughout the brainstem. It is a paired nerve
and exits the lateral surface of pons bilaterally as separate
sensory and motor roots. The sensory root forms the trigeminal
(Gasserian) ganglion in the middle cranial fossa and is located in
a cavity called Meckel’s cave. This ganglion divides into ophthal-
mic (V1), maxillary (V2), and mandibular (V3) nerves. The motor
root passes along with the sensory root, but is distributed only to
the mandibular division.
The ophthalmic nerve exits the cranium through the superior
orbital fissure and innervates the skin above the eye, forehead,
and globe. The maxillary nerve exits the cranium through the
foramen rotundum and supplies the skin between the eye and
mouth. The mandibular nerve exits through the foramen ovale.
Sensory fibres of V3 innervate the skin of the lateral part of the
head and lower jaw, tongue, mucosa of oral cavity, and teeth.
Motor fibres innervate the muscles of mastication.
The trigeminal nerve, through its branches, carries parasym-
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pathetic supply to ganglia and the lacrimal glands (V1, V2), nasal
glands (V2), submandibular, sublingual, and parotid glands (V3).
Treatment
Pharmacological
Optimal pharmacotherapy should be the first line of treatment in
the majority of patients with TN. Symptomatic TN needs to be
managed by addressing the treatment of the causative condition.
Carbamazepine (200–1200 mg) remains the drug of choice,
with good evidence1 of efficacy in TN and a number needed to
treat of 1.8. It is an anticonvulsant drug that blocks the use-
dependent sodium channels and may also prevent synaptic
transmission in the trigeminal nucleus. The major limiting factor
in its use is the high incidence of side-effects, including drowsi-
ness, dizziness, rash, liver damage, hyponatraemia, and ataxia.
Patients on carbamazepine should therefore have their liver
function, blood count, and serum electrolytes checked at regular
intervals. Oxcarbazepine, a derivative of carbamazepine, has a
better side-effect profile and similar efficacy.
Gabapentin, pregabalin, and amitriptyline are commonly
used in TN because of their efficacy in treating neuropathic
pain conditions; however, evidence of their effectiveness specific
to TN is not strong. Baclofen and lamotrigine have been used as
add-on therapy. Baclofen may be useful in patients with MS suf-
fering from TN.1 Similarly, phenytoin has been used with limited
benefit in some cases of TN.
Surgical
Patients who fail to benefit from pharmacological therapy or
experience significant side-effects should be considered for sur-
gical interventions. These procedures can be carried out at three
levels.
Peripheral
Peripheral techniques involve neurolysis of the trigeminal
nerve branches distal to the gasserian ganglion. This can be ac-
complished by using laser therapy, alcohol injections, or neurec-
tomy. These procedures are less invasive than other surgical
interventions, but the pain relief is short term, lasting 6 months
to 1 yr. Patients may also develop dysesthesias post-procedure.6

However, they can be useful in patients who are not fit for any
other surgical interventions, or as an emergency procedure.
At the gasserian ganglion
The gasserian ganglion can be ablated using thermal (radiofre-
quency), chemical (glycerol, phenol, alcohol), or mechanical
(balloon compression) techniques. A needle is passed percutan-
eously into the foramen ovale to reach the ganglion to facilitate
these procedures.
The procedure is usually performed under local anaesthetic
and sedation, as the patient’s co-operation is necessary. Fluro-
scopy-guided technique is more precise in localizing the foramen
ovale. The patient is placed supine, with neck slightly extended.
A submental view X-ray is obtained. Then, by gradually moving
the C arm obliquely towards the affected side, the foramen
ovale is located between mandibular process and maxilla. A nee-
dle is directed towards the foramen ovale using X-ray guidance
(Fig. 1). Needle tip position is confirmed using lateral views
(Fig. 2). Once the needle is in a satisfactory position, rhizotomy
is achieved by radiofrequency, glycerol injection, or balloon
compression.
These interventions provide longer duration of pain relief,
lasting around 4–5 yr in 50% of patients.2,6 However, there is a
high incidence of sensory loss and dysaesthesias, with around
4% developing anaesthesia dolorosa.6 Balloon compression can
lead to arrhythmias, aseptic meningitis, and temporary diplopia.
Posterior fossa
(i) MVD: The trigeminal nerve in the posterior fossa is accessed
by a suboccipital craniotomy. The nerve root is freed from
vessels compressing it (most commonly superior cerebellar
artery) and a teflon felt is placed between them. MVD has a
very good initial success rate (80–90%) and provides the most
Fig 1 Submental view of needle in foramen ovale.
Trigeminal neuralgia
sustained pain relief of all the procedures available. The
relapse rate at the end of 10 yr is about 30–40%.8,9 Since
MVD is a major neurosurgical procedure, it is associated
with a mortality of 0.5%. Other complications include asep-
tic meningitis (11%), hearing loss (10%), sensory loss (7%),
cerebrospinal fluid leaks, haematomas, and infarcts. The
complication rate is lower in centres that perform MVD
regularly.
(ii) Gamma knife stereotactic radiosurgery: is a destructive pro-
cedure that aims at delivering a focused beam of radiation to
trigeminal nerve root in the posterior fossa where there is a
proven vascular compression. MRI mapping is used to locate
the exact site of microvascular compression. This procedure
provides complete pain relief in up to 69% of patients by the
end of 1 yr,6 but the benefits may not be sustained, although
it can be repeated in recurrent TN. Complications include
facial numbness and paraesthesia. It remains a useful
option in patients not suitable for MVD.
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Psychological interventions
TN, like any other chronic pain condition, can have a significant
impact on the patient’s psychological well-being. Although there
is a lack of good quality studies assessing usefulness of psycho-
logical interventions, it is worth having a multidisciplinary
team including psychologist in managing these patients and
considering psychological interventions such as cognitive behav-
ioural therapy and acceptance and commitment therapy.10
Other modalities
Neuromodulation techniques targeting the trigeminal nerve,
occipital nerves, peripheral nerves, spinal cord, and motor cortex
are being performed at some centres, although at present, the
evidence is not robust enough to warrant routine use.
Non-invasive repetitive transcranial magnetic stimulation of
motor cortex has been shown to be of benefit in orofacial pain,
including that involving the trigeminal nerve. However, its role
in managing TN is yet to be established.
Fig 2 Lateral view of needle in foramen ovale.
BJA Education | 2016 Page 3 of 4
Trigeminal neuralgia
There are some case reports and open-ended studies regard-
ing the use of botulinum toxin type A in TN. Well-conducted
studies with large samples are required before it can be recom-
mended in TN.
Transcutaneous electrical nerve stimulation, acupuncture,
and 5% lidocaine patches have also been tried in TN with varying
results.
Conclusion
Patients with TN suffer one of the most severe pains described.
Appropriate and early diagnosis is important to formulate an
optimal management plan. Pharmacotherapy with carbamaze-
pine is worth trying in the first instance before considering
invasive procedures. As treatment options become more inva-
sive, the results improve, but at the cost of increased side-effects.
Hence, it is important to individualize the management plan
according to the patient’s circumstances.
Declaration of interest
None declared.
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