Trigeminal neuralgia

Amr Hassan MD,FEBN
Professor of Neurology Cairo University- EGYPT
Trigeminal Neuralgia

Agenda
• Anatomy
• Epidemiology
• Clinical presentation, classification, diagnostic tips
• Differential diagnosis
• Non-Pharmacological treatment
• Pharmacological treatment
• Prognosis

Ophthalmic Branch (Sensory)
Cornea
Ciliary body
Conjunctiva
Nasal cavity
Sinuses
Skin of eyebrows, forehead, and nose
Anatomy of Trigeminal Nerve

Maxillary Branch (Sensory)
Side of nose
Lower eyelid
Upper lip

Mandibular Branch (Sensory)
Temporal
Auricular
Lower face
Lower lip,
Oral Mucosa
Anterior two thirds of Tongue
Mandibular gums and teeth

Mandibular Branch (motor)
Masseter Muscle
Temporalis Muscle
Pterygoid Muscle

Agenda
• Anatomy
• Epidemiology
• Clinical presentation
• Prognosis

• Annual incidence 4-5 patients per 100,000 population.
• Highest incidence between 50 & 70 years of age.
• Estimated 2% of multiple sclerosis patients complain of TN.
• Roughly 15,000 new cases annually in the United States.
• Most cases are sporadic.
van Kleef M et al. Pain Practice. 2009;9:252-259.
Rozen TD. Neurol Clin. 2004;22:185-206.
Katusic S et al. Neuroepidemiology. 1991;10:276-281.
Obermann M et al. Expert Review of Neuropathics. 2009;7:323-329.
Rozen TD et al. Wolff's Headache and Other Head Pain. Oxford University Press, 2001.
Fleetwood IG et al. J Neurosurg. 2001;95:513-517.
Incidence of Trigeminal Neuralgia

0
5
10
15
20
25
30
2nd 3rd 4th 5th 6th 7th 8th 9th
Decade
Age of Onset
• 90% of cases occur after age 40.
• More prevalent in women then men 1.5-2 : 1 ratio.
• More than 70% of patients with TN are over 50 years of age at the time
onset.

Prevalence of Trigeminal Neuralgia

• Pain is brief (Seconds to 1-2 minutes) and paroxysmal, occur in
Several attacks, Stabbing or Shocklike and is typically Severe.
• Pain provokes brief muscle Spasm of the facial muscles, thus
producing the tic.
Clinical picture: pain

• Various triggers may commonly
precipitate a pain attack.
• Light touch or vibration is the most
provocative.
• Activities such as shaving, laughing,
brushing teeth and face washing.
Clinical picture: Trigger points

CONTINUUM: Lifelong Learning in Neurology 23(2):396-420, April 2017.
Clinical picture: Trigger points

• 38 y old lady presented with recurrent
bouts of left sided paroxysmal facial pain
lasting for seconds.
• Provoked by brushing her teeth, eating,
or even talking.
• Her examination was entirely normal
apart from mild left sided facial
hypothesia.
Case 1

• Trigger point; may limit examination for fear of stimulating these points.
• The diagnosis of idiopathic TN is tenable only if no physical findings of fifth
nerve dysfunction are present.
Physical Examination

• Age younger than 40 years
• Bilateral symptoms
• Swallowing difficulties
• Dizziness or vertigo
• Hearing loss or abnormality
• Numbness
• Pain outside of trigeminal nerve distribution
• Visual changes
Trigeminal Neuralgia: Red flags (History)

• Abnormal neurologic examination
• Abnormal oral, dental, or ear examination
• Loss of the corneal reflex
• Any jaw or facial weakness
• Facial hypesthesia or dysesthesia
• Permanent area of numbness
Trigeminal Neuralgia: Red flags (Examination)

TN diagnostic criteria - 13.1.1
A. At least 3 attacks of unilateral facial pain fulfilling criteria B and C
B. Occurring in trigeminal nerve distribution, no radiation
C. Three of the following four characteristics:
1. Lasting max. 2 minutes
2. Severe intensity
3. Electric shock-like, shooting or sharp
4. Precipitated by innocuous stimuli to the affected side of the face
D. No clinically evident neurological deficit
E. Not better accounted for by another ICHD 3 diagnosis
Trigeminal Neuralgia: diagnostic criteria ICHD 3-beta

A - Paroxysmal attacks of pain lasting from a fraction of a second to 2
minutes, with or without persistence of aching between paroxysms,
affecting 1 or more divisions of the trigeminal nerve and fulfilling criteria B
and C
B - Pain has at least 1 of the following characteristics: (1) intense, sharp,
superficial or stabbing; or (2) precipitated from trigger areas or by trigger
factors C - Attacks stereotyped in the individual patient
D - A causative lesion, other than vascular compression, demonstrated by
special investigations and/or posterior fossa exploration
Symptomatic Trigeminal Neuralgia: diagnostic criteria

Cavernous Sinus Syndromes
Cerebral Aneurysms
Cluster Headache
Hemifacial Spasm
Hydrocephalus
Intracranial Hemorrhage
Migraine Headache
Multiple Sclerosis
Postherpetic Neuralgia
Subarachnoid Hemorrhage
Common causes of symptomatic trigeminal neuralgia

• 53 y old lady presented
with continuous facial
pain involving the whole
right side of the face.
• Her neurological
examination was
entirely unrevealing.
Case 2

Case 2
Right Trigeminal Nerve
Compressing vessel
• 53 y old lady presented
with continuous facial
pain involving the whole
right side of the face.
• Her neurological
examination was
entirely unrevealing.

Pain is unilateral (rarely bilateral).
Clinical picture: Distribution
32
17 17
15 14
4
0.4
0
5
10
15
20
25
30
35
Percent
V2,3 V2 V1,2,3 V3 V1,2 V1 V1,3
Trigeminal Division

ICHD-3 beta new subgroups in trigeminal neuralgia:
13.1.1.1 TN with purely paroxysmal pain
13.1.1.2 TN with concomitant persistent pain
Trigeminal Neuralgia: diagnostic criteria ICHD 3-beta

Right Trigeminal Nerve
Compressing vessel
Trigeminal Neuralgia: imaging

Morphologic changes of the trigeminal root

• Demyelinated sensory neurons: hyperexitability
and ectopic pacemaker sites.
• Neuron-to-neuron cross-excitation due to eroded
insulation – amplification and synchronization.
• Ephaptic transmission and crossed after
discharge between non-nociceptive afferents and
nociceptive afferents may explain how innocuous
sensory stimuli can trigger painful paroxysms
Devor et al. 2002
Pathophysiology – “the ignition hypothesis”

• 54 y old lady presented with recurrent bouts of left sided paroxysmal
facial pain lasting for seconds.
• Her neurological examination was entirely normal.
• What is your diagnosis?
• Should we ask for MRI Brain?
Case 3

• Magnetic resonance imaging
(MRI),using a combination of three
high-resolution sequences, should
be performed as part of the work-up
in TN patients, because no clinical
characteristics can exclude
secondary TN.
EAN 2019 Guidelines

• Neurovascular contact plays an
important role in primary TN, but
demonstration of a neurovascular
contact should not be used to
confirm the diagnosis of TN.
• Rather, it may help to decide if and
when a patient should be referred
for microvascular decompression
EAN 2019 Guidelines

• If MRI is not possible, trigeminal
reflexes can be used.
EAN 2019 Guidelines

Trigeminal reflex test to disclose secondary trigeminal
neuralgia

Agenda
• Anatomy
• Epidemiology
• Clinical presentation , classification, diagnostic tips
• Prognosis

CONTINUUM: Lifelong Learning in Neurology 21(4):1072-1085, August 2015.
Trigeminal Neuralgia: differential diagnosis

Condition
Male:Female
Ratio
Age of
onset, y
Localization
Accompanying
Symptoms
Attack
Duration
Cycles Provocation
Trigeminal
neuralgia
1:2 >50 Unilateral None Seconds
Month
intervals
Trigger
zones
Cluster
headache
1:6-9 30-40
Always
unilateral
Horner
syndrome,
conjunctival
injection,
epiphora
15-180
minutes
Clusters
with weeks
to months
intervals
Nocturnal
attacks
Migraine 1:1 10-20 Variable
Photophobia,
phonophobia,
gastrointestina
l symptoms
4-72 hours
Days to
weeks
intervals
Variable

Feature Trigeminal Neuralgia Atypical Facial Pain
Prevalence Rare Common
Main location Trigeminal area Face, neck, ear
Pain duration Seconds to 2 minutes Hours to days
Character Electric jerks, stabbing Throbbing, dull
Pain intensity Severe Mild to moderate
Provoking factors
Light touch, washing, shaving,
eating, talking
Stress, cold
Associated symptoms None Sensory abnormalities

Pharmacological treatment of Trigeminal Neuralgia: LOE A

Pharmacological treatment of Trigeminal Neuralgia: LOE B

Pharmacological treatment of Trigeminal Neuralgia: LOE C

• Carbamazepine and oxcarbazepine
should be used as first-line
prophylactic treatments of TN.
EAN 2019 Guidelines

Dropouts due to adverse events

• Lamotrigine, gabapentin, botulinum
toxin type A, pregabalin, baclofen,
and phenytoin may be used either
alone or as add-on therapy.
EAN 2019 Guidelines

Botulinum toxin type A in trigeminal Neuralgia

EAN 2019 Guidelines
• Patients should be offered surgery if
pain is not sufficiently controlled
medically or if medical treatment is
poorly tolerated
• In patients with classical TN,
microvascular decompression is
recommended as first-line surgery

• Microvascular decompression (MVD)
• Percutaneous ablative procedures
– Radiofrequency gangliolysis
– Glycerol rhizolysis
– Balloon compression
• Stereotactic radiosurgery
– Gamma knife
– Linac-based
• Peripheral ablative procedures (V1 and V2 pain)
– Peripheral branch neurectomy
– Alcohol neurolysis
• Open destructive procedures
– Partial sensory rhizotomy
– Subtemporal ganglionectomy (Frazier-Spiller procedure)
Surgical Treatment of TN

Microvascular decompression:
– Requires general anesthesia.
– 2.5- to 3-cm craniectomy is performed, the dura is opened, and the
cerebellum is microsurgically retracted.
– Typically, an artery or other vascular cross-compression of the nerve
is identified, the vascular structure is padded away from the nerve
with polytetrafluoroethylene (Teflon) felt.
– This operation has a low mortality rate 0.1 and 0.5% in most series.
– Serious morbidity probably between 1 and 5%.
• Numbness, hearing loss, dizziness, cerebellar syndrome, CSF leaks,
meningitis, diplopia.

• MVD is the ONLY non-destructive procedure for the treatment of TN
• Low risk of facial sensory loss with subsequent dysesthesias or
anesthesia dolorosa
• ONLY operation that addresses what is believed to be the primary
underlying pathology; i.e. vascular compression
• Long-term results are at least equivalent if not superior to any other
procedure
Advantages of MVD

• Requires major surgery – may not be suitable for patients with significant
medical co-morbidity
• MVD is generally associated with more risks than percutaneous
procedures or radiosurgery
• More costly than percutaneous procedures
Disadvantages of MVD

• Cerebellar injury <1%
• Infectious complications
– Bacterial meningitis
– Aseptic meningitis
• CSF leak 0-4%
• Cranial nerve deficits
– Diplopia
– Sensory loss or dysesthesias 0.5-17%
– Facial weakness 0.5-15%
– Hearing loss <1 (0-19%)
• Stroke
• Mortality < 1%
Complications of MVD

Percutaneous procedures:
– Less risk
– Local or brief general anesthesia
– A needle or trocar is inserted on the cheek just lateral to the corner of
the mouth, under fluoroscopic guidance, introduced into the ipsilateral
foramen ovale.
– Gangliolysis is performed.
– Different types of procedure:
• Percutaneous radiofrequency trigeminal gangliolysis (PRTG).
• Percutaneous retrogasserian glycerol rhizotomy (PRGR).
• Percutaneous balloon microcompression (PBM).

Glycerol Injection
Contrast in trigeminal cistern Contrast under temporal lobe

Complications of TN surgeries

Gasserian ganglion percutaneous techniques Success Rates 1:
• Initial: 90%
• 12 months: 68-85%
• 36 months: 54-64%
• 60 months: 50%
MVD Success Rates 2:
• Initial: 90%
• 12 months: 80%
• 36 months: 75%
• 60 months: 73%
1 year after gamma knife therapy, complete pain relief with no medication occurs in up to 69%
of patients. This falls to 52% at 3 years 3.
1 Obermann M et al. Expert Review of Neuropathics. 2009;7:323-330. Zakrzewska JM et al. Pain. 1999;79:51-58
2 Obermann M et al. Expert Review of Neuropathics. 2009;7:323-330. Barker FG 2nd et al. N Eng J Med. 1996;334:1077-1082
Gronseth G, Cruccu G, Alksne J, et al. Practice parameter: the diagnostic evaluation and treatment of trigeminal neuralgia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology
and the European Federation of Neurological Societies.
Prognosis after TN surgeries

THANK YOU
amrhasanneuro@kasralainy.edu.eg

Trigeminal neuralgia

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