TRIGEMINAL NEURALGIA

Introduction

 Disorder characterized by lancinating

attacks of severe facial pain

 Diagnosis based primarily on a history of

characteristic pain attacks that are
consistent with specific research & clinical
criteria
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 In majority of patients, clinical exam-
ination, imaging and lab tests are
unremarkable – Classic TN

 In a smaller group, signs & symptoms

secondary to another disease affecting the
trigeminal system – Symptomatic TN

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Nicolas Andre, 1756
“Tic douloureux”
commented that it was exclusive &
distinctive from all other diseases

John Fothergill, 1773

outlined major clinical features, clearly
establishing the disorder as a discrete
syndrome
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 Epidemiology and Demographics

- Incidence of approx 4 in 100,000

Familial cases also reported
- Majority of cases occur spontaneously
- Slight female predominance
- Over age 50

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- Pain typically consists of lancinating
paroxysms
- Mostly in Second & Third trigeminal
divisions
- Right side most often involved
- Pain attacks stereotyped
- Symptom free between attacks
- Chronic disorder, most patients will
experience pain attacks for years unless
appropriately treated

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 Etiology and Pathogenesis
Cause – not known

Injury to the nerve root – an initiating factor?

(Benign tumors and vascular anomalies
that compress the trigeminal nerve root
can produce symptoms clinically
indistinguishable from classic TN)

Dr.Haris PS/OMR
 Based on the morphologic and physio-
logic changes following partial nerve injury,
Devor et al proposed “ignition hypothesis”.
A trigeminal injury induces physiologic
changes that result in a population of hyper-
excitable and functionally linked primary
sensory neurons. The discharge of any
individual neuron of this group can quickly
spread to activate the entire population.
Such a discharge could underlie the
sudden jolt of pain in TN attack.
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 Clinical Presentation and
Physical Findings

Diagnosis of TN based on distinctive signs &

symptoms.
White & Sweet articulated diagnostic criteria
for TN.
Consists of 5 major clinical features that
define the diagnosis of TN

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 Sweet diagnostic criteria

1. Pain is paroxysmal
2. The pain may be provoked by light touch
to the face (trigger zones)
3. The pain is confined to the trigeminal
distribution
4. The pain is unilateral
5. The clinical sensory examination is
normal

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 Patients who did not meet all the criteria
rarely benefited.

The Sweet criteria were incorporated into

the criteria published by IASP & IHS.

ICHD II (IHS) subdivides Trigeminal

Neuralgia (code 13.1) into,
- Classic TN (code 13.1.1)
- Symptomatic TN (code 13.1.2)
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 Classic TN (13.1.1)
Most common form- idiopathic, and also
associated with vascular compression.
“a unilateral disorder characterized by brief electric
shock-like pains, abrupt in onset and termination, limited
to the distribution of one or more divisions of trigeminal
nerve. Pain is commonly evoked by trivial stimuli
including washing, shaving, smoking, talking and/or
brushing the teeth (trigger factors) and frequently occurs
spontaneously. Small areas in the nasolabial fold and/or
chin may be particularly susceptible to the precipitation
of pain (trigger areas). The pains usually remit for
variable periods.”
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 ICHD Criteria for Classical TN (13.1.1)
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 from trigger areas or by trigger factors
C. Attacks are stereotyped in individual patient.
D. There is no clinically evident neurological deficit.
E. Not attribute to another disorder.

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 Symptomatic TN (13.1.2)

- Results from another disease process

(MS or a cerebellopontine angle tumor)

“Pain indistinguishable from 13.1.1

classic TN but caused by a demonstrable
structural lesion other than vascular
compression.”

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 ICHD Criteria for Symptomatic TN (13.1.2)
A. Paroxysmal attacks of pain lasting from a fraction of a
second to 2 minutes, with or without persistence of
aching between paroxysms, affecting one or more
divisions of 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 from trigger areas or by trigger factors.
C. Attacks are stereotyped in individual patient.
D. A causative lesion, other than vascular compression, has
been demonstrated by special investigations and/or
posterior fossa exploration.
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The pain of TN……

- Paroxysmal attacks
- Electric shock like quality
- Sudden onset & severe in intensity  facial
grimace
- Duration btw 1 sec and 2 min
- Instantaneous electric shock sensation that’s
over in much less than a sec – ‘lightning bolt’
- Symptom free btw attacks.

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Trigger zones……

A TN “trigger zone” is an area of facial skin or oral

mucosa where a low intensity mechanical
stimulation can elicit a typical pain attack.
- Only a few mm in size
- In perioral region
- First division trigger zones are very rare.
- Presence of trigger zone pathognomonic.
- May result from ephatic coupling btw partially
damaged trigeminal axons.
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- Pain confined to trigeminal zone
- Most frequently in 3rd division
- Less frequently in 2nd or in both divisions
- Pain attacks are stereotyped
- Unilateral
- Bilateral in MS
- Clinical sensory examination is normal

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 Clinical evaluation
Diagnosis based on clinical history,
supplemented by physical examination
findings and cranial imaging studies.
Detailed intraoral examination to rule out
odontogenic and non odontogenic source
for the pain
Examination of CN V, VII & VIII
Symptomatic TN from a CPA mass often
shows facial weakness and hearing loss on
that side
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 Diagnostic testing
Diagnostic brain imaging to visualize
anatomic landmarks around trigeminal
ganglion and CPA

CT, MRI – to rule out CPA lesions and to

visualize subtle vascular anomalies
causing compression

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 Medical Management and
Treatment

TN unique – majority of patients respond to

treatment and may have total elimination
of pain attacks

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 Pharmacologic therapy
Primary drug therapy

Bergouignan, 1942 found that the

anticonvulsant phenytoin effectively
controlled attacks of TN

Similarity in mechanisms between epilepsy &

TN pain attacks.

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Routine therapy begins with single agent, in
gradually increasing doses until pain
attacks are suppressed or satisfactorily
reduced.

 Carbamazepine (CBZ)
 Baclofen (BCF)
 Lamotrigine (LTG)

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CBZ superior to Phenytoin
CBZ monotherapy provides symptom control
in up to 80% patients
BCF equally effective, better tolerated

Others
- Clonazepam, Gabapentin, Topiramate,
Oxcarbazepine, Tiagabine, Levetiracetam
and Zonisamide.

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 Multiple drug therapy

- When a patient respond only partially to

single drug therapy at dosages that evoke
side effects……

- When patients do not satisfactorily respond

to 2 AED’s, they should be considered for
surgical interventions.

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Surgical options

Highly effective and well tolerated

Cumulative risk of multiple pharmacological

agents may exceed the risk of surgical
complications, especially in the elderly

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 3 SURGICAL APPROACHES
1. Percutaneous stereotactic radiofrequency
thermal lesioning of the trigeminal ganglion
and/or root (RFL)
2. Posterior fossa exploration and microvascular
decompression (MVD) of the trigeminal root
3. Gamma knife radiation to the trigeminal root
entry zone (GKR)
Produce satisfactory relief of TN symptoms in 80
– 90% of patients. Incidence of complications
is low and specific for the technique employed.
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1. RFL

- Produce mild injury to the sensory fibers

in the trigeminal root.
- Minimally invasive
- Controls symptoms in > 85% of patients
- Principal side effect – sensory loss and
occasional dysesthesia

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2. Posterior fossa exploration and MVD

- More complex and invasive

- Directly treats the hypothetical cause

while minimizing any sensory damage

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3. GKR

- Relatively recent
- Employs computerized stereotactic
methods to concentrate gamma radiation
on the trigeminal root entry zone
- Could be highly effective
- Long term benefits to be established

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 RFL for patients who are elderly or medically
frail.

 Posterior fossa exploration and MVD for

younger healthier patients who can tolerate the
longer more invasive surgical procedure.

 GKR as an alternative to RFL in frail or elderly

patients. MVD or RFL remains the standard for
surgical treatment of younger patients who have
considerable life expectancy
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 Conclusion
- Many fundamental questions about patho-
physiology of the disorder remain unanswered

- Development of drugs specific for TN

- Lack of objective testing remains as a problem

- fMRI – potential future diagnostic tool

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