Trigeminal neuralgia is a condition characterized by severe, sporadic facial pain. It has been described and studied historically since the 2nd century AD. Modern management involves drug therapies like carbamazepine as first line treatment. When medications fail, various surgical procedures can be used including percutaneous glycerol rhizotomy, balloon compression, and radiofrequency rhizotomy. These aim to destroy nerve fibers supplying the trigeminal ganglion to relieve facial pain from trigeminal neuralgia.
2. “ …. as it were a flash of fire all of a sudden shot
into all those parts …. which made her shreeke
out…”
Letter to a friend by John Locke
December 4, 1677
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3. 2nd Century – Aretaeus of Cappodocia in “Cephalaea”.
1037 AD – Avicenna.
1677 – John Locke first description.
1756 – Nicolas Andre – “tic douloureux” “Cynical spasm”.
1773 – John Fothergill – “Fothergill’s Disease”
1829 – Bell – Anatomy of 5th nerve
HISTORICAL PROSPECTIVE
4. 1853 – Trousseau – “ Neuralgia epileptiform”
1942 – Bergouignan – Phenytoin
1962 – Blom – Carbamazepine
1934 – Dandy – Vascular compression of nerve
1967 – Peter Jannetta devised MVD
1998 - Khan—Gabapentin, for frequency rhizolysis,
trigeminal neuralgia in temperature monitoring, multiple
sclerosis
2007 - Obermann—Pregabalin
HISTORICAL PROSPECTIVE
7. Trigeminal ganglia
Covering –contained in a dural pouch
known as cavum trigeminale(menkel,s
cave)
Dural extend forward as a ballooning
of meningeal layer of dura mater from
posterior cranial to middle cranial
fossabelow attached margin of
tentorium cerebelli
Cavum-
Roof-formed by two meningeal layer
Floor-one meningeal and one endosteal
layer
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8. Definition
Trigeminal Neuralgia is a clinical syndrome
distinguished by brief paroxysms of Unilateral,
lancinating facial pain in the 5th nerve
distribution triggered by cutaneous stimuli, such
as a breeze on the face, chewing, talking or
brushing the teeth.
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9. Epidemiology
Incidence, approximate at 5 in every 100 000
Gender ratio of female : male = 2:1
Right sided 56% of the time
Maxillary (V2) > Mandibular (V3) > Ophthalmic (V1)
Mean age at diagnosis is 60 years-old
It occurs mostly after 5th decade.↑ frequency with age,
highest at >80 years old
10-15% of patients seeking care at dental specialty 4-May-16
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11. Etiology and
Pathophysiology….
Older ideas
Epileptogenesis (seizures from the brain)
Newer theories
Ectopic nerve firing (seizures in the nerve)
Ephapsis (cross-wirednerves)
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12. Etiology and
Pathophysiology….
• Demyelinative lesions of trigeminal fibers
appear to set up ectopic generation of
spontaneous nerve impulses and their ephaptic
conduction to adjacent fibers. (Gardner)
• This can lead to pain attacks at the slightest
stimulation of any area served by the nerve .
• It also hinder the nerve's ability to shut off the
pain signals after the stimulation ends 4-May-16
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23. Trigeminal Neuralgia &
Multiple Sclerosis
10-20% of patients with MS present with TGN
1-8% of MS have TGN (Selby, 1984)
Younger age group – 30 yrs mean
Bilateral pain commoner than in idiopathic
TGN
MR imaging may pick up sclerotic plaque at
root entry zone
2% of all TGN 4-May-16
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24. Investigations
X-Ray skull – AP through orbit, lateral,
Towne’s view
CT head
MRI Brain
Electrophysiological Tests
– Evoked Potential (Lunsford et al, 1985)
- Nd: Yag Laser Pulses (Romaniello et al,
2003) 4-May-16
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25. Higher petrous apex
– Gardner et al, 1956 – 3 times higher incidence
Tip of dens > 5mm above McGregor’s line (43% vs 23%
in controls)
Basilar Impressions
Dolichocephaly
Paget’s disease
Postmenopausal Osteoporosis
X-Rays in TGN
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26. MRI in TGN
Indications – Young patients
- Bilateral pain
- Atypical Neuralgia
- Neurologic deficits
MRI with MRA – Patel et al, 2003 – 92 patients
- 90.5% sensitivity, 100% specificity
MR tomographic angiography – Fukuda et al, 2003 – 67% correlation
MRI with constructive interference in steady state
- Yoshino et al, 2003 – 80% correlation
Functional MRI, PET Scan with Opioidergic Imaging 4-May-16
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30. Diagnosis
TN is often misunderstood as pathology of dental origin. Many patients may
go untreated for long periods of time before a correct diagnosis is made.
TN remains a clinical diagnosis.
MRI and MRA can be performed if there is suspicion of underlying
pathology.
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40. Percutaneous Retrogasserian
Glycerol Rhizotomy
• 1981 – Hakanson – Glycerol+Tantalum dust
• Principle effects of Glycerol
- Neurolytic
- Hyperosmolarity
- Affects large diameter Axons
- Esp. previously demyelinated axons
- Affects the trigger mechanism
- May down regulate central hyperexcibility
- Bengt Linderoth, Sten Hakanson
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41. Indications of PRGR
The main indication for glycerol rhizolysis
remains classic idiopathic TN.
In medically infirm patients
Anticonvulsants or baclofen causing severe
intolerant side effects particularly to patients
with paroxysmal facial pain associated with
multiple sclerosis (MS).
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42. Technique of PRGR
• No intra-op patient co-operation required – GA
• Position – initially supine – completed in semisitting
• Hartel’s technique
• Cisternography to assess volume
• Average 0.25ml (0.2-0.5ml)
• 99.9% anhydrous glycerol + tantalum injection
• Placed in semisitting position for 2hrs after the procedure
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43. 4-May-16
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Hartel’s anatomic landmarks for access to the foramen of ovale.
A.Surgeon marks three points on the patient’s face:
•A point 3 cm anterior to the external auditory meatus along a line from the external
auditory meatus to the tip of the nose ,
•A point beneath the medial aspect of the pupil on the lower eyelid, and
•A point 2.5 cm lateral to the oral commissure .
B.The target is the foramen ovale, at the intersection of a vertical line (in the sagittal
plane) extending through the point beneath the pupil (1) and a horizontal line (in the axial
plane) through the point anterior to the external auditory meatus (2).
44. Technique of PRGR
• 22-gauge lumbar cannula is
inserted from a point approximately
3 to 4 cm lateral to the corner of the
mouth.
•The trajectory is aimed at a point
that lies, in the lateral view,
approximately 0.5 cm anterior to the
anterior margin of the mandibular
joint, and in the anteroposterior
view, toward the medial margin of
the pupil with the eyeball in the
neutral position. 4-May-16
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45. Cannula trajectory toward the superior-medial aspect of the foramen ovale.
A. The surgeon’s index finger rests alongside the molars, against the lateral pterygoid, to guide
the cannula toward the foramen ovale and prevent penetration of the oral mucosa. The cannula is
aimed at the foramen ovale via Hartel’s landmarks.
B. If direct penetration of the foramen is not achieved, the surgeon can sequentially walk down
the infratemporal fossa (arrow) to the superomedial aspect of the foramenovale.
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46. Technique of
PRGR
Consecutive axial CT scan
images of a patient with
intracisternal tantalum
dust.
The anteroposterior
distance from the anterior
portion of the oval
foramen to the anterior
portion of the mandible is
measured.
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48. Percutaneous Balloon
Compression (PBC)
Principle:
Destruction of large myelinated pre-ganglionic fibers by
compression against petrous bone and dural edge.
•GA, pacemaker, slight neck extension, flouroscopy
•Hartel’s technique
•#4 Fogarty catheter 17 to 22 mm beyond foramen at the porus
trigeminus
•0.75 to 1cc of 180mg% iohexol under pressure of 1200-1500mm Hg
•Tissue compression pressure – 650 – 950 mm Hg
•Upto 1 minute or 1.5 min in recurrence
•Proper inflation – Pear shape : depressor response
Technique of PBC
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50. Principle : Selective destruction of A-delta & C fibers at lower
temperatures
Technique of PSR
•Day care procedure under image guidance
• Supine, Neutral position of head, short GA
• Hartel’s technique
• Proper positioning indicated by CSF flow
• Electrode localisation
• Lesioning at 60-70 degree Centigrade for 70 seconds
• Sequential lesions of 90 seconds with 5 degree Centigrade after
each lesion
• Dense hypalgesia in desired area subjectively assessed.
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52. Vascular Compression of the Nerve
Demyelination of Axons
Ephaptic Transmission
Impaired segmental inhibition
Central Nuclear hyper activity
TGN
Principle of MVD
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54. SCA COMPRESSION
A.The right trigeminal nerve is
compressed by a tortuous basilar
artery and the left trigeminal nerve is
compressed by the main trunk of the
SCA.
B. SCA bifurcates into rostral and caudal
trunks before reaching the trigeminal
nerve. The nerve is compressed by the
caudal trunk.
C. SCA bifurcates distally to the nerve.
The nerve is compressed by the main
trunk.
D. SCA bifurcates before reaching the
nerve. The nerve is compressed by
both the rostral and caudal trunks.
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55. E. the nerve is compressed by a large pontine artery.
F. the nerve is compressed by an AICA that has a high origin and loops
upward into the medial surface of the nerve. The SCA passes around the
brainstem above the nerve.
.
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56. Venous Compression
Superior petrosal Vein that empty into the superior petrosal
sinuses most commonly compress the Trigeminal nerve
Tributaries:
Transverse pontine Vein (most frequent)
Ponto trigeminal Vein
Vein of cerbellopontine fissure and Middle cerebellar
peduncle
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57. A. Anterior view. The veins that commonly compress the trigeminal nerve are tributaries of the superior
petrosal vein
B. a transverse pontine vein compresses the lateral side of the nerve and joins the veins of the middle
cerebellar peduncle and cerebellopontine fissure to empty into a superior petrosal vein.
C. the medial side of the nerve is compressed by a tortuous transverse pontine vein.
D, the lateral side of the nerve is compressed by the junction of the transverse pontine vein with
the veins of the middle cerebellar peduncle and the cerebellopontine fissure 4-May-16
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59. Procedure of MVD
For decompression of the fifth cranial nerve, the incision (dotted line) is positioned as shown
so that two thirds of the length is above the level of the mastoid notch.
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60. A. Schematic view of a left trigeminal
nerve decompression.
•The superior cerebellar artery is
compressing the superior edge of the
trigeminal nerve.
B. Elevation of the superior cerebellar
artery reveals indentation and grooving
by the artery.
C. Shredded Teflon felt is gently worked
in between the nerve and the
compressing artery.
D. The shredded Teflon felt is placed
between the artery and the nerve so that
the thrust of the arterial force is now
directed away from the underlying
nerve. 4-May-16
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62. Results of MVD
• Average success rate 78%, >90% when vessels found
• Avg recurrence rate 20-25%
• Max recurrence within 2yrs
• Major complications around 4% - cranial nerve deficits
• Mortality – 0.5%
• Numbness – 3-29%
• Hearing Loss < 4%
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63. Prognostic Factors in MVD
Female gender
Duration of symptoms > 8yrs
Venous compression only
Lack of immediate post-op pain relief
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64. Gamma Knife Radiosurgery for TGN
• 1951 – Lars Leksell introduced
• Principle – Radiation induced damage to REZ after a latent period
- Minimising damage to surrounding structures.
•Indications: Failed medical therapy
Poor medical condition
Recurrent TGN
Patient’s choice
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65. Gamma Knife
Single high dose radiotherapy delivered with exquisite precision to a
radiographically defined target, at the junction of trigeminal nerve and brain stem.
Success rate is 70%
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66. Technique of GKRS:
• Accurate imaging of the REZ with 1mm MRI slices
• 4mm isocenter targeted to REZ 2-4mm anterior to brain stem
• Brain stem receives < 20% isodose
• Length of nerve irradiated at 50% isodose is 4mm
• Total radiation dose of 70-90 Gy over 30mins
• Latent period for pain relief – upto 10 weeks
Robert W. Rand, 1997
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67. Cyberknife Radiosurgery
• Developed in 1994 – Accuracy, Inc., Sunnyvale, CA
- Adler 1999, Chang 2001
• Non invassive head immobilisation & advanced image - guidance
• Dynamic tracking of skull – ensures target accuracy of 1.1mm
• Frameless procedure
• Delivers non isocentric, conformal, homogenous radiation to non
spherical structures.
• Romanelli et al, 2003 – 10 patients with Trigeminal Neuralgia –
70% response.
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68. Technique
• Single session – Medium maximum dose 78 Gy,
median, marginal dose 65.5 Gy
• Median Target volume = 0.085 cm3
• Length of nerve encompassed by 79% isodose line =
7.2mm
• Target volume placed 2-3 mm anterior to REZ
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69. Results:
• 92.7% successful pain relief at 7 days
• Pain relief – Excellent – 87.8 %
Moderate – 4.9%
No change – 7.3%
• Long Term response rate 78%
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72. Choice of surgical treatment
Relatively young patients with no co-morbidities: MVD
Patients unable to tolerate GA:
Percutaneous procedures
Stereotactic radiosurgery
Multiple sclerosis: SRS/ Percutaneous techniques/MVD
Final choice based on patient’s preference and ability to
tolerate GA
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73. Trigeminal Neuralgia: Opportunities
for Research
Treatment of trigeminal neuralgia is like playing the game of
dart, so far .
• Treatment is empirical, but not satisfactory 4-May-16
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74. Individuals of note with TN include
Entrepreneur and author Melissa Seymour (Australia) was
diagnosed with TN in 2009 and underwent microvascular
decompression.
Salman Khan , was diagnosed with TN in 2011 He underwent
surgery in the US.
All-Ireland winning Gaelic footballer Christy Toye was
diagnosed with the condition in 2013.
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Three-dimensional (3D) constructive interference in steady state (CISS) is a gradient-echo MRI sequence that is used to investigate a wide range of pathologies when routine MRI sequences do not provide the desired anatomic information. The increased sensitivity of the 3D CISS sequence is an outcome of the accentuation of the T2 values between cerebrospinal fluid (CSF) and pathological structures. Apart from its well-recognized applications in the evaluation of the cranial nerves, CSF rhinorrhea and aqueduct stenosis, Trigeminal neuralgia is caused most commonly by compression of the root entry zone of the trigeminal nerve by a vascular loop. [9] This compression and displacement of the nerve by the vascular loop is well evaluated by the CISS sequence [Figure 2], which demonstrates the thinning of the root entry zone and allows exact identification of the vascular loop. It has been proposed as the initial screening procedure for all patients with refractory trigeminal neuralgia, especially if surgical intervention is being considered. [10] Contrast-enhanced CISS is useful for evaluating the trigeminal ganglion [11] and the cisternal segment of the nerve.