Trigeminal Neuralgia

1. TRIGEMINAL NEURALGIA
Dr shabeel pn

2. Dr.Haris PS/OMRDr.Haris PS/OMR
IntroductionIntroduction
Disorder characterized by lancinating Disorder characterized by lancinating
attacks of severe facial painattacks of severe facial pain
Diagnosis based primarily on a history of Diagnosis based primarily on a history of
characteristic pain attacks that are characteristic pain attacks that are
consistent with specific research & clinical consistent with specific research & clinical
criteriacriteria

3. Dr.Haris PS/OMRDr.Haris PS/OMR
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

4. Dr.Haris PS/OMRDr.Haris PS/OMR
Nicolas Andre, 1756
“Tic douloureux”
commented that it was exclusive & commented that it was exclusive &
distinctive from all other diseasesdistinctive from all other diseases
John Fothergill, 1773
outlined major clinical features, clearly outlined major clinical features, clearly
establishing the disorder as a discrete establishing the disorder as a discrete
syndromesyndrome

5. Dr.Haris PS/OMRDr.Haris PS/OMR
Epidemiology and DemographicsEpidemiology and Demographics
- Incidence of approx 4 in 100,000
Familial cases also reported
-Majority of cases occur spontaneously
-Slight female predominance
-Over age 50

6. Dr.Haris PS/OMRDr.Haris PS/OMR
-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

7. Dr.Haris PS/OMRDr.Haris PS/OMR
Etiology and Pathogenesis 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)

8. Dr.Haris PS/OMRDr.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.

9. Dr.Haris PS/OMRDr.Haris PS/OMR
Clinical Presentation andClinical Presentation and
Physical FindingsPhysical Findings
Diagnosis of TN based on distinctive signs & Diagnosis of TN based on distinctive signs &
symptoms.symptoms.
White & Sweet articulated diagnostic criteria articulated diagnostic criteria
for TN.for TN.
Consists of 5 major clinical features that Consists of 5 major clinical features that
define the diagnosis of TNdefine the diagnosis of TN

10. Dr.Haris PS/OMRDr.Haris PS/OMR
Sweet diagnostic criteria
3.3.Pain is paroxysmalPain is paroxysmal
4.4.The pain may be provoked by light touch The pain may be provoked by light touch
to the face (trigger zones)to the face (trigger zones)
5.5.The pain is confined to the trigeminal The pain is confined to the trigeminal
distributiondistribution
6.6.The pain is unilateralThe pain is unilateral
7.7.The clinical sensory examination is The clinical sensory examination is
normalnormal

11. Dr.Haris PS/OMRDr.Haris PS/OMR
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)

12. Dr.Haris PS/OMRDr.Haris PS/OMR
Classic TN (13.1.1)
Most common form- idiopathic, and also Most common form- idiopathic, and also
associated with vascular compression.associated with vascular compression.
““a unilateral disorder characterized by brief electric a unilateral disorder characterized by brief electric
shock-like pains, abrupt in onset and termination, limited shock-like pains, abrupt in onset and termination, limited
to the distribution of one or more divisions of trigeminal to the distribution of one or more divisions of trigeminal
nerve. Pain is commonly evoked by trivial stimuli nerve. Pain is commonly evoked by trivial stimuli
including washing, shaving, smoking, talking and/or including washing, shaving, smoking, talking and/or
brushing the teeth (trigger factors) and frequently occurs brushing the teeth (trigger factors) and frequently occurs
spontaneously. Small areas in the nasolabial fold and/or spontaneously. Small areas in the nasolabial fold and/or
chin may be particularly susceptible to the precipitation chin may be particularly susceptible to the precipitation
of pain (trigger areas). The pains usually remit for of pain (trigger areas). The pains usually remit for
variable periods.”variable periods.”

13. Dr.Haris PS/OMRDr.Haris PS/OMR
ICHD Criteria for Classical TN (13.1.1)
B.B.Paroxysmal attacks of pain lasting from a fraction of a Paroxysmal attacks of pain lasting from a fraction of a
second to 2 minutes, affecting one or more divisions of second to 2 minutes, affecting one or more divisions of
the trigeminal nerve and fulfilling criteria B and Cthe trigeminal nerve and fulfilling criteria B and C
C.C.Pain has at least one of the following characteristics:Pain has at least one of the following characteristics:
1. intense, sharp, superficial or stabbing1. intense, sharp, superficial or stabbing
2. precipitated from trigger areas or by trigger factors2. precipitated from trigger areas or by trigger factors
F.F.Attacks are stereotyped in individual patient.Attacks are stereotyped in individual patient.
G.G.There is no clinically evident neurological deficit.There is no clinically evident neurological deficit.
H.H.Not attribute to another disorder. Not attribute to another disorder.

14. Dr.Haris PS/OMRDr.Haris PS/OMR
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.”

15. Dr.Haris PS/OMRDr.Haris PS/OMR
ICHD Criteria for Symptomatic TN (13.1.2)
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.
Pain has at least one of the following characteristics:
1. Intense, sharp, superficial or stabbing
2. Precipitated from trigger areas or by trigger factors.
Attacks are stereotyped in individual patient.
A causative lesion, other than vascular compression, has
been demonstrated by special investigations and/or
posterior fossa exploration.

16. Dr.Haris PS/OMRDr.Haris PS/OMR
The pain of TN……
-Paroxysmal attacksParoxysmal attacks
-Electric shock like qualityElectric shock like quality
-Sudden onset & severe in intensity Sudden onset & severe in intensity  facial facial
grimacegrimace
-Duration btw 1 sec and 2 minDuration btw 1 sec and 2 min
-Instantaneous electric shock sensation that’s Instantaneous electric shock sensation that’s
over in much less than a sec – ‘lightning bolt’over in much less than a sec – ‘lightning bolt’
-Symptom free btw attacks.Symptom free btw attacks.

17. Dr.Haris PS/OMRDr.Haris PS/OMR
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.

18. Dr.Haris PS/OMRDr.Haris PS/OMR
-Pain confined to trigeminal zonePain confined to trigeminal zone
-Most frequently in 3Most frequently in 3
rdrd
division division
-Less frequently in 2Less frequently in 2
ndnd
or in both divisions or in both divisions
-Pain attacks are stereotypedPain attacks are stereotyped
-UnilateralUnilateral
-Bilateral in MSBilateral in MS
-Clinical sensory examination is normalClinical sensory examination is normal

19. Dr.Haris PS/OMRDr.Haris PS/OMR
Clinical evaluationClinical 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

20. Dr.Haris PS/OMRDr.Haris PS/OMR
Diagnostic testingDiagnostic testing
Diagnostic brain imaging to visualize Diagnostic brain imaging to visualize
anatomic landmarks around trigeminal anatomic landmarks around trigeminal
ganglion and CPAganglion and CPA
CT, MRI – to rule out CPA lesions and to CT, MRI – to rule out CPA lesions and to
visualize subtle vascular anomalies visualize subtle vascular anomalies
causing compressioncausing compression

21. Dr.Haris PS/OMRDr.Haris PS/OMR
Medical Management and Medical Management and
TreatmentTreatment
TN unique – majority of patients respond to TN unique – majority of patients respond to
treatment and may have total elimination treatment and may have total elimination
of pain attacksof pain attacks

22. Dr.Haris PS/OMRDr.Haris PS/OMR
Pharmacologic therapyPharmacologic therapy
Primary drug therapy
Bergouignan, 1942 found that the , 1942 found that the
anticonvulsant anticonvulsant phenytoin effectively effectively
controlled attacks of TNcontrolled attacks of TN
Similarity in mechanisms between epilepsy & Similarity in mechanisms between epilepsy &
TN pain attacks.TN pain attacks.

23. Dr.Haris PS/OMRDr.Haris PS/OMR
Routine therapy begins with single agent, in Routine therapy begins with single agent, in
gradually increasing doses until pain gradually increasing doses until pain
attacks are suppressed or satisfactorily attacks are suppressed or satisfactorily
reduced.reduced.
 Carbamazepine (CBZ)
 Baclofen (BCF)
 Lamotrigine (LTG)

24. Dr.Haris PS/OMRDr.Haris PS/OMR
CBZ superior to PhenytoinCBZ superior to Phenytoin
CBZ monotherapy provides symptom CBZ monotherapy provides symptom
control in up to 80% patientscontrol in up to 80% patients
BCF equally effective, better toleratedBCF equally effective, better tolerated
OthersOthers
- - Clonazepam, Gabapentin, Topiramate,
Oxcarbazepine, Tiagabine, Levetiracetam
and Zonisamide..

25. Dr.Haris PS/OMRDr.Haris PS/OMR
Multiple drug therapy
-When a patient respond only partially to When a patient respond only partially to
single drug therapy at dosages that evoke single drug therapy at dosages that evoke
side effects……side effects……
-When patients do not satisfactorily respond When patients do not satisfactorily respond
to 2 AED’s, they should be considered for to 2 AED’s, they should be considered for
surgical interventions.surgical interventions.

26. Dr.Haris PS/OMRDr.Haris PS/OMR
Surgical options
Highly effective and well toleratedHighly effective and well tolerated
Cumulative risk of multiple pharmacological Cumulative risk of multiple pharmacological
agents may exceed the risk of surgical agents may exceed the risk of surgical
complications, especially in the elderlycomplications, especially in the elderly

27. Dr.Haris PS/OMRDr.Haris PS/OMR
3 SURGICAL APPROACHES
Percutaneous stereotactic radiofrequency
thermal lesioning of the trigeminal ganglion
and/or root (RFL)
Posterior fossa exploration and microvascular
decompression (MVD) of the trigeminal root
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.

28. Dr.Haris PS/OMRDr.Haris PS/OMR
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

29. Dr.Haris PS/OMRDr.Haris PS/OMR
1.Posterior fossa exploration and MVD
-More complex and invasive
-Directly treats the hypothetical cause
while minimizing any sensory damage

30. Dr.Haris PS/OMRDr.Haris PS/OMR
1.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

31. Dr.Haris PS/OMRDr.Haris PS/OMR
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

32. Dr.Haris PS/OMRDr.Haris PS/OMR
Conclusion
-Many fundamental questions about patho-Many fundamental questions about patho-
physiology of the disorder remain unansweredphysiology of the disorder remain unanswered
-Development of drugs specific for TNDevelopment of drugs specific for TN
-Lack of objective testing remains as a problemLack of objective testing remains as a problem
-ffMRIMRI – potential future diagnostic tool– potential future diagnostic tool