Cranial nerve 5 neuralgia

1. TRIGEMINAL NEURALGIA
DIAGNOSIS AND TREATMENT
Samuel W Samuel, MD
Associate Fellowship Director
Pain Management Fellowship
The Cleveland Clinic Foundation

2. Objectives
• ICHD classification related to TN
• Diagnosis
• Differential diagnosis
• Medical treatment
• Interventional treatment
• Surgical treatment
• Complications of treatments

3. “ Trigeminal Neuralgia is the Worst pain in the
world” Peter Janetta, MD in “ striking back” 1
TN was first documented in the 1st century AD
and was described in the writings of Aretaeus.
Treatment at the time included Blood letting
and the administration of Arsenic, Cobra
hemlock and mercury soaked bandages.
Weigel G, Kenneth F, Casey M. “Striking back” Gainsville: Trigeminal Neurlagia
Association 2000

4. “Tic Doloureux” is a painful condition of the face
Described in Ibn Sina (980-1073) in an Arabic Text
Johannes Bausch and John Locke Documented Clinical descriptions of
TN in 1672 and 1677
Nicolas Andre in 1765 outlined 5 cases of “unbearable painful twitch”,
other names for TN is Prosopalgia and Neuralgia of the fifth

5. Trigeminal neuralgia is 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 the
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.
http://ihs-classification.org/en/02_klassifikation/04_teil3/13.01.01_facialpain.html

6. A. At least 3 attacks of Unilateral Facial pain fulfilling
criteria B and C
A. Occurring in one or more divisions of the trigeminal
nerve, with no radiation beyond the trigeminal
distribution.
A. Pain has at least 3 of the following 4 characteristics :
I. Recurring in Paroxysmal attacks lasting from
fraction of a second to 2 min.
II. Severe in intensity
III. Electric shock like, shooting, stabbing or sharp
in quality
IV. Precipitated by innocuous stimuli to the
affected side of the face
B. No clinically evident neurological deficit
A. Not better accounted for by another ICHD-3
diagnosis
ICHD 3 Beta diagnostic Criteria

7. Slight female predominance
Female 5.9 per 100,000
Male 3.4 per 100,000
-Right side affected slightly more often
-Occasional familial occurrences
-Slightly elevated risk with
HTN
multiple sclerosis
Demographic

8. Differential diagnosis
1. Musculoskeletal
2. Dentoalveolar
3. Ear, nose and throat
4. Giant cell arteritis
5. Glaucoma
6. Cluster headaches
7. Atypical migraine
8. Chronic paroxysmal hemicrania
9. TMJ syndrome
10. Cracked tooth syndrome
11. Idiopathic stabbing headache
12. Glossopharyngeal neuralgia
13. nervus intermedius neuralgia
14. SUNCT
15. Trigeminal neuropathy
16. Atypical trigeminal neuralgia
17. Typical TN (should consider MS especially in bilateral
cases)
Nurmikko T. Trigeminal Neuralgia and other facial neuralgias. In: Cervero F,
Jensen TS, eds. Handbook of clinical neurology, Vol.81 (3rd series. Vol 3), Pain.
New York: Elsevier; 2006: 573-596

9. Painful cranial neuropathies and other facial pains
1.Trigeminal neuralgia
a. Classical trigeminal neuralgia
i. Classical trigeminal neuralgia, purely
paroxysmal
ii. Classical trigeminal neuralgia with
concomitant persistent facial pain
b. Painful trigeminal neuropathy
i. Painful trigeminal neuropathy attributed to
acute Herpes zoster
ii. Postherpetic trigeminal neuropathy
iii. Painful posttraumatic trigeminal neuropathy
iv. Painful trigeminal neuropathy attributed to
multiple sclerosis plaque
v. Painful trigeminal neuropathy attributed to
space-occupying lesion
vi. Painful trigeminal neuropathy attributed to
other disorder
2.Glossopharyngeal neuralgia
3.Nervus intermedius (facial nerve) neuralgia
4.Occipital neuralgia
5.Optic neuritis
6.Headache attributed to ischemic ocular motor nerve palsy
7.Tolosa-Hunt syndrome
8.Paratrigeminal oculosympathetic (Raeder) syndrome
9.Recurrent painful ophthalmoplegic neuropathy
10.Burning mouth syndrome
11.Persistent idiopathic facial pain
12.Central neuropathic pain
a. Central neuropathic pain attributed to multiple sclerosis
b. Central poststroke pain
From Headache Classification Committee of the International Headache Society (IHS). The
International Classification of Headache Disorders. 3rd edition (beta version). Cephalalgia
2013;33:629–808.

10. Nervus Intermedius Neurlagia
• Also know as Geniculate Neuralgia
• Very uncommon of cranial neuralgia
• IHS defined as intermittent episodes of pain located deep in the
ear that lasts for sec. or minutes triggered by sensory of
mechanical stimuli at the posterior wall of the Auditory canal.
• may be due to vascular compression of the VIIth, and VIIIth cranial
nerves
• Other causes of Otalgia must be eliminated these include: Otitis
externa, Media; malignancy, TMJ, vascular lesions, intracranial
lesions in the CP angle and rare syndromes as Eagle’s syndrome
Tang IP, Freeman SR, Kontorinis G, Tang M et al the journal of Laryngology and Otolgy 128:5 (May2014) :
394-9

11. Nervus Intermedius Neuralgia (cont.)
• Treatment include:
A. medical management
1. Carbamazepine 200-800 mg daily
2. Gabapentin 900-3600 mg daily
3. Lamotrigine 200-400 mg daily
4. Amitriptyline 25-125 mg daily
B. Surgical management
excision of the nervus intermedius and the geniculate
ganglion ether via a middle cranial fossa approach or
retromastoid craniectomies with microvascular
decompression
Lovely TJ, Jannetta PJ. Surgical management of geniculate neuralgia. Am J Otol 1997;18:512-17

12. Distribution of Pain by Division
0
5
10
15
20
25
30
35
V2,3 V2 V1,2,3 V3 V1,2 V1 V1,3
32
17 17
15
14
4
0.4
Percent
Trigeminal Division

13. Anatomy of the Gasserian Ganglion
• Lies in the Meckel’s
cavity in the middle
Cranial fossa
• Medially cavernous
sinus
• Superiorly by the
inferior surface of the
temporal lobe
• Posteriorly the brain
stem

14. Gasserian Ganglion Anatomy
• 3 divisions with
characteristic
somatotopic
arrangement
• Ophthalmic (V1) is the
most craniomedial,
mandibular (V2) is the
most caudolateral and
Mandibular lies
inbetween

15. • The ophthalmic nerve
exists through the
superior orbital fissure
• The maxillary through
the foramen rotundum
and the mandibular
nerve through the
foramen ovale

16. • Medical management
1
• Interventional treatment
2
• Surgical treatment
3
Treatment modalities

17. Medical Management
• Carbamazepine the drug of choice 2
• Metabolized in the P450 system
• Slows recovery rate of the voltage gated Na+ channels,
modulates activated calcium channels activity and
activates the ascending inhibitory modulation system.
• starting dose of 100-200 twice daily with gradual titration
until pain relief or side effects
• Average maintenance dose is 600-1200 mg daily in
divided doses
• Rashes, Leucopenia, abnormal liver functions, aplastic
anemia and hyponatremia
Jorns T, Zakrzewska J. Evidence-based approach to the medical management of trigeminal
neuralgia. Br J Neurosurg 2007;21: 253-61

18. Medical management continued
• Oxcarbazepine is being supported to be a first line of
treatment
• Less SE than Carbamazepine and requires less Lab
testing 3
• 10-keto analogue of carbamazepine
• Tolerability is reported to be “good” or “excellent” in
62% compared to 48% of patients receiving
Carbamazepine.
• Dose ranges from 900-1800 mg/day with onset of 24-72
hours
Cruccu G, Gronseth G, Alksne J, et al. AAN_EFNS guidelines on trigeminal neuralgia management Eur J
Neurol 2008;15:1013-28

19. Lamotrigine
• Decreases the repetitive firing of Na+ channels by
slowing the recovery rate of voltage gated channels
• In a small RCT patients who were refractory to
Carbamazepine or phenytoin, Lamotrigine (400mg)
increased the number of patients who improved
after 4 weeks of treatment.4
• SE include Dizziness, constipation, nausea and
drowsiness, Steven-Johnson Syndrome occurred
1:10,000 patients on Lamotrigine
Zekrzewska JM, Chaudry Z, Nurmikko TJ et al Lamotriginein refractory trigeminal neuralgia: results
from a double blind placebo controlled cross-over trial Pain 1997;73:223-30

20. • Is one of the oldest Anticonvulsants
• Blocks Na+ channels in rapidly discharging
neurons and inhibiting presynaptic Glutamate
release
• No controlled studies supporting its efficacy
despite being one of the longest used Rx for TN
• Hyperglycemia, hepatotoxicity, gingival
hyperplasia and megaloblastic anemia
Phenytoin

21. • Analogue of the neurotransmitter γ-
aminobutyric acid.
• It is used in TN because it depresses the
excitatory synaptic transmission in the spinal
trigeminal nucleus.
• Starting dose is 5 mg TID with gradual titration
to 50-60 mg per day in divided doses
• Sedation, dizziness, and dyspepsia are common
SE
Baclofen
Fromm GH, Terrence CF, Chatta AS et al. Baclofen in Trigeminal neuralgia its
effect on the spinal trigeminal nucleus: a pilot study Arch Neurol
1980;37:768

22. Interventional treatment
• When medical treatment is unsuccessful or has too many
side effects.
• 5 clinically appropriate possibilities + one experimental
1. Surgical microvascular decompression (MVD)
2. Stereotactic radiation therapy, Gamma knife.
3. Percutaneous balloon micro-decompression
4. Percutaneous Glycerol Rhizolysis
5. Percutaneous Radiofrequency treatment of the
Gasserian Ganglion
6. Gasserian Ganglion stimulation/neuromodulation
(experimental)

23. Surgical (MVD)
• During MVD, the vessels that are in contact
with the root entry zone are coagulated and
the arteries are separated from the nerve
with an inert sponge or Felt.
• Generally it is accepted that MVD is the first
choice of treatment in younger patients
Jannetta PJ, McLaughlin MR, Casey KF. Technique of microvascular decompression.
Technical note. Neurosurgery. 2005;18:E5

24. The most recent studies support MVD as the best therapeutic
option with regard to improvement in quality of life and when
considering the long-term pain relief after surgery.
Increased morbidity and mortality associated with the elderly
patients with MVD

25. Surgical (MVD)
Barker and colleagues reported results of 1185 patients
underwent MVD over 20 year period.
Ten years after surgery, 70% of the patients (as determined
by Kaplan-Meier analysis) had excellent final results-that is,
they were free of pain without medication An additional 4%
had occasional pain that did not require long-term
medication
It was shown that the rates of complications were reduced
and no deaths occurred after 1980 when intra-operative
monitoring of the brainstem-evoked response was used.
Barker FG, Jannetta PJ, Bissonetter DJ, et al the long term outcome of microvascular
Decompression for trigeminal neuralagia. N Engl J Med 1996:334:1077-84

26. MVD
• MVD was previously contraindicated in patients with MS;
however there is a small subset of 35 patients who
received 22% fair and 39% excellent long term relief from
MVD.
• In general patients with MS responds less to medical and
interventional treatments these patients may require
more aggressive treatments compared to patients with
CTN
Broggi G, Ferroli P, Franzini A et al operative findings and outcomes of microvascular
Decompression for trigeminal neuralgia in 35 patients affected by multiple sclerosis
Neurosurgery 2004:55:830-9

27. • High dose irradiation of a small section of the trigeminal
nerve
• Results in non-selective damage to Gasserian Ganglion
• Non-invasive treatment
• Initial efficacy appears to be limited between 60-70%
indicate reduction in pain
• Long term effects are not yet know
Stereotactic radiation therapy
Gamma Knife

28. PERCUTANEOUS BALLOON
MICROCOMPRESSION
Trigeminal nerve is compressed by a small balloon which is percutaneously
introduced into the Meckel’s cavity
Relies on Ischemic damage of the ganglion cell
Insufficient data in regards to efficacy
Comparable to Percutaneous RF of the Gasserian ganglion
Suitable for treatment of the TN of the first Branch and thus corneal reflex
remains intact

29. Percutaneous Glycerol Rhyzolysis
• A needle is introduced into the
trigeminal Cistern
• Under fluoroscopy in the
seated position
• Head is flexed
• Contrast is injected to
determine the size of the
cistern
• Equal volume of Glycerol is
injected
Hakanson S. trigeminal neuralgia treated by the injection of Glycerol into the
trigeminal cistern. Neurosurgery 1981;9:638-646

30. Percutaneous RF treatment of the Gasserian
Ganglion
• Should be considered in the elderly patient
• The outcome is slightly less favorable than MVD
• Less invasive
• Lower morbidity and mortality

31. • About 60% of the patients treated with RF are pain free
for at least 60 months if the treatment is correctly given
• About 50% of the patients there is sensory loss in the
treated branches of the trigeminal nerve
• Should not be used in secondary TN as in Post-herpetic
neuralgia
• The only exception to that is secondary TN due to MS
• Pulsed RF would seem to be a reasonable alternative to
thermocoagulation in the only RCT PRF failed to
demonstrate efficacy
Erdine S, Ozyalsin NS, Cimen A, Celik M, Taha GK, Disci R. Comparison of pulsed radiofrequency with conventional
readiofrequency in the treatment of idiopathic trigeminal neuralgia. Eur J Pain 2007;11:309-313

32. Technique of RF treatment of the Gasserian Ganglion

35. Gasserian Ganglion Radiofrequency ablation

36. • Patient in the Supine position with Sedation (watch for Bradycardia
up to Asystole)
• C-arm is tilted to obtain Submental view then rotated
Obliquely towards the affected side until the foramen
Ovale is visualized medially with respect to the
Mandibular process and lateral to the Maxilla.
• If Maxillary and mandibular branch are to be treated the
entry point is 2 cm lateral to the corner of the mouth and
the needle is aimed to the middle of the Foramen
• If mandibular branch only the entry is more medial to the
mouth (1cm) and the aim is to the lateral part of the
foramen
• If the ophthalmic is targeted the entry is 3 cm lateral to
the mouth and aim to the medial aspect of the foramen

37. Complications
• Kanpolat et al reported the results of 25 years
experience with 1600 patients
1. Decreased corneal reflex (5.7%)
2. Weakness and paralysis of the Masseter (4.1%)
3. Dysesthesia (1%)
4. Anesthesia Dolorosa (0.8%)
5. Keratitis (0.6%)
6. Temporary paralysis of the third and fourth
cranial nerves (0.8%)
Kanpolat Y, Savas A, Bekar A, Berk C. Percutaneous controlled radiofrequency trigeminal
Rhizotomy for the treatment of idiopathic trigmeninal neuralgia: 25-year experience
With 1600 patients. Neurosurgery 2001;48:524-532

38. Practical approach to TN
management
van Kleef M, van Genderen WE, Narouze S, Nurmikko TJ, van Zundert J, Geurts JW, Mekhail N; World Institute
of Medicine.
Pain Pract. 2009 Jul-Aug;9(4):252-9.

39. Practical approach to TN management

40. Thank you