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Diagnosis and Treatment of Trigeminal Neuralgia

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Title: Diagnosis and Treatment of Trigeminal Neuralgia


1
Diagnosis and Treatment of Trigeminal Neuralgia
2
Trigeminal Nerve Anatomy
3
Functional Anatomy
  • GSA general sensation from head and facial
    structures
  • Main sensory nucleus
  • Descending tract of V to spinal trigeminal
    nucleus
  • Functional equivalent of substantia gelatinosa of
    spinal cord
  • GSE muscles of mastication
  • SVE branchial arch muscles
  • Tensor veli palatini
  • Tensor tympani

4
Demographics
  • 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 associated with HTN and
    multiple sclerosis

5
Classic Trigeminal NeuralgiaBurchiel Type I
  • Brief (seconds to minutes) episodes of severe,
    sharp, stabbing, lancinating, pain
  • Almost always unilateral
  • Bilateral V1 pain sugestive of MS
  • Pain occurs along one or more trigeminal
    divisions
  • Spontaneous or evoked pain
  • Cutaneous trigger zones
  • Multiple attacks may occur over short periods
  • Asymptomatic between attacks
  • Normal facial sensation

6
BurchielClassification of Facial Pain
  • Spontaneous Onset
  • TN Type 1 (Classic TN)
  • gt 50 episodic pain
  • TN Type 2 (Atypical TN)
  • gt 50 constant pain
  • Trigeminal Injury
  • Symptomatic TN (Multiple sclerosis)
  • Trigeminal neuropathic pain (post-traumatic)
  • Trigeminal deafferentation pain (RF lesion, GKR,
    etc.)
  • Post-herpetic facial pain
  • Secondary TN
  • Tumors, aneurysm, AVM, etc.
  • Atypical facial pain (somatiform pain disorder)

7
Age of Onset
More than 70 of patients with TN are over 50
years of age at the time onset
8
Distribution of Pain by Division
9
Diagnosis of Trigeminal Neuralgia
  • ALL FACIAL PAIN IS NOT TRIGEMINAL NEURALGIA!
  • Successful treatment of any patient with facial
    pain in general and TN in particular depends on
    making the correct diagnosis at the outset

10
Pharmacological Treatment for Trigeminal Neuralgia
  • AEDs are the cornerstone of treatment
  • Start low, titrate to relief or side effects
  • Monitor side effects and drug interactions
  • Monitor levels and blood tests if indicated
  • Rotate other AEDs or add as needed
  • Tegretol remains the gold standard
  • Response thought to be diagnostic

Tegretol is the ONLY drug that has been shown to
be effective for treatment of TN in a randomized
controlled trial
11
Pharmacological Treatment
  • AEDs
  • Tegretol (carbamazepine)
  • Tripeptal (oxcarbazepine)
  • Dilantin (phenytoin)
  • Neurontin (gabapentin)
  • Lyrica (pregabalin)
  • Lamictal (lamotrigene)
  • Topamax (topirimate)
  • Gabatril (tiagabine)
  • Keppra (levateracitam)
  • TCAs
  • Elavil (amitriptyline)
  • Pamelor (nortriptyline)
  • Desipramine (norpramin)
  • Baclofen (lioresal)
  • Opioids

12
Adverse Effects of AEDs
  • Cognitive changes
  • Sedation
  • Nystagmus, ataxia, diplopia, dizziness
  • Nausea, vomiting, headache
  • Allergic reaction
  • Up to 7 with CBZ
  • Some cross-reactivity between CBZ and PHT

13
Imaging in Trigeminal Neuralgia
  • In patients with types 1 and 2 trigeminal
    neuralgia (TN1 and TN2) one can identify
  • Presence of neurovascular compression (NVC)
  • Degree of NVC
  • Nature of the compressing vessel
  • Location of NVC along the nerve
  • Findings can be confirmed during MVD

14
CB - MRA (TOF)
Right Trigeminal Nerve
Compressing vessel
15
CB - T1 (FSE) Gad
Right Trigeminal Nerve
Compressing vessel
16
3D TOF
3D FSE Gad
3D T2
17
MRI does accurately predict the symptomatic side
  • Ho (null hypothesis) there is no difference
    between MRI prediction and surgical side
  • Result Fail to reject Ho (P 0.40)
  • MRI does predict the symptomatic (surgical) side
  • Sensitivity of MRI for predicting symptomatic
    side 78

18
The symptomatic nerve shows a higher degree of
compression than the asymptomatic nerve
  • Ho (null hypothesis) there is no difference
    between degree of compression on symptomatic and
    asymptomatic side
  • Reject Ho (P 0.0003)
  • MRI does demonstrate a higher degree of
    compression on the symptomatic side

19
MRI can accurately detect arterial v. venous
compression
  • Ho (null hypothesis) MRI cannot distinguish
    between arteries and veins compressing the nerve
  • Reject Ho (P 0.36)
  • MRI can differentiate arterial and venous
    compression
  • Highly correlated with surgical findings

20
Surgical Treatment of TN
  • 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)

21
Advantages of MVD
  • 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

22
Disadvantages 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

23
Surgical Technique
  • Positioning
  • Skin Incision
  • Iniomeatal line transverse sinus
  • Digastric groove
  • ¾ - ¼ rule
  • Retromastoid craniectomy
  • Expose sigmoid-transverse sinus junction
  • Mastoid emissary vein
  • Bevel bone laterally
  • Sufficient anterior exposure reduces amount of
    cerebellar retraction
  • T-shaped dural opening
  • Exposure of most superior and lateral corner

24
Surgical Technique
  • Exposure of CPA
  • Turning the corner is the most dangerous stage
    of the operation and must be executed with
    patience and the utmost care (Peter Jannetta)
  • CSF drainage
  • Gentle retraction of ala of cerebellum
  • Identify and divide petrosal vein
  • Visualization of trigeminal nerve
  • Visualize the ENTIRE nerve from its exit from
    the pons to its exit laterally from the CPA
  • Decompression
  • Mobilize and pad arteries
  • Coagulate and divide veins

25
Operative Findings
  • Arterial compression
  • Superior cerebellar artery (SCA) most common
  • AICA
  • PICA
  • Vertebrobasilar artery
  • Venous compression
  • More common with atypical TN
  • Combined arterial and venous compression

26
Intraoperative Observations
  • 579 consecutive patients undergoing MVD
  • 97 (560/579) had one or more vessels
  • Multiple vessels found in 38
  • SCA 88
  • AICA 25
  • Vein 28
  • Basilar artery 4
  • Location of NVC (medial-lateral)
  • Trigeminal REZ 52
  • Middle 1/3 54
  • Lateral 10

Sindou M, et. al. Acta Neurochir (Wien)
1441-12, 2002
27
Intraoperative Observations
  • Location of NVC
  • Supero-medial 53.9
  • Supero-lateral 31.6
  • Inferior 14.5
  • Severity of NVC
  • Simple contact 17.6
  • Distorsion of nerve 49.2
  • Marked indentation 33.2
  • Other Findings
  • Global atrophy 42
  • Arachnoid thickening 18
  • Angulation near petrous bone 13

Sindou M, et. al. Acta Neurochir (Wien)
1441-12, 2002
28
Operative Findings
29
Complications of MVD
  • Cerebellar injury lt1
  • 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 lt1 (0-19)
  • Stroke
  • Mortality lt 1

30
Complications of MVD
31
Outcome Following Initial MVD(N1204 patients)
Barker F, Jannetta P, Bissonette D, et.al. NEJM,
1996
32
MVD - 10-Year Outcome Barker F, Jannetta P,
Bissonette D, et.al. NEJM, 1996
33
Long-Term Results of MVDTypical TN
  • Review of 19 series with 2,747 patients
    (17-1,204)
  • Average follow-up, 4.4 years (4 months to 10
    years)
  • 78 with excellent-good results (62-92)
  • gt90 initial success with positive findings
  • Failure rate 22 (8-30)
  • Complications 4-34
  • Facial numbness, 3-29
  • Hearing loss, 0-19
  • Mortality, 0.5

Lovely T, Janetta P Neurosurgery Clinics of
North America. 1997
34
Long-Term Results of MVD
Taha and Tew
35
Factors Influencing Outcome of MVDDuration of TN
  • Kolluri et. al., 1984
  • TN gt 4 years Recurrence 25
  • TN lt 4 years Recurrence 15
  • Bederson et. al., 1989
  • TN gt 4 years Excellent/good 91
  • TN lt 4 years Excellent/good 75
  • Broggi, et. al., 2000
  • TN gt 7 years ONLY poor prognostic factor for
    favorable outcome

36
Factors Influencing Outcome of MVDPrevious
Ablative Procedures
  • Barba et. al., 1984
  • Success rate of MVD reduced from 91 to 43
  • Bederson et. al., 1989
  • Excellent outcomes reduced from 78 to 63
  • Walchenbach et. al., 1994
  • Past ablative procedure - 50 good result
  • MVD primary procedure 86 good result
  • Best result appear to be achieved when MVD is
    performed as the primary procedure for treatment
    of TN

37
Special Considerations
  • MVD in elderly patients
  • Typical (Type I) vs. atypical TN (Type II)
  • Repeat MVD
  • Role of MVD in patients with MS
  • MVD following percutaneous procedures

38
Repeat MVD for Recurrent TN
  • All procedures used to initially treat TN CAN be
    effective for recurrent TN
  • Less than 1/3 of patients undergo repeat MVD
  • Lower success rates
  • Findings New compressive vessel, compression by
    felt
  • Higher incidence of perioperative morbidity
  • Increased risk of cranial nerve palsy
  • Increased incidence of facial numbness (8)
    and/or facial dysesthesias

39
Typical vs. Atypical TN Tyler-Cabara E, et.al.
J Neurosurgery 96527-531, 2002
40
MVD in Elderly Patients Ashkan K, Marsh H
Neurosurgery, 55840-850, 2004
41
MVD in Multiple Sclerosis
  • MS traditionally considered an absolute
    contraindication to MVD
  • Presumption that demyelination is the exclusive
    causative factor for TN in MS
  • Neuroimaging has raised the possibility of a role
    for vascular compression
  • Add Galligan)

42
MVD in Multiple Sclerosis Broggi et. al.
Neurosurgery, 55830-839,2004
  • 35 MS patients with medically-intractable TN
  • 74 - MRI evidence of demyelinating lesion on the
    symptomatic side (26 of 35)
  • 46 (16 of 35 patients) had obvious vascular
    compression
  • Long-term outcome
  • Excellent 39
  • Good 14
  • Fair 8
  • Poor 39
  • Results of MVD in MS patients are much less
    satisfactory than in the idiopathic group.

43
MVD in Multiple Sclerosis
  • 9 patients with MS underwent PF exploration
  • 7 MVD alone
  • 2 MVD PSR
  • 100 evidence of vascular compression on MRA
  • Initial pain relief excellent in all patients
  • Recurrence
  • 5 of 7 with MVD alone
  • 1 of 2 with MVD PSR
  • 4 of 9 patient had long-term pain relief
  • Because of the high recurrence rate together
    with the morbidity.MVD should not be offered to
    patients with TN and MS.

Eldridge P, et.al. Stereotact Funct Neurosurg
8157-64, 2003
44
Percutaneous Procedures
  • Radiofrequency thermal coagulation
  • Glycerol rhizolysis
  • Balloon compression

45
(No Transcript)
46
Needle Insertion
47
Radiofrequency Lesion
48
Glycerol Injection
Contrast in trigeminal cistern
Contrast under temporal lobe
49
Balloon Compression
50
MVD vs. Percutaneous Procedures
  • INITIAL PAIN RELIEF
  • MVD 98
  • RF rhizotomy 98
  • Balloon 93
  • Glycerol 91
  • RECURRENCE RATES
  • Glycerol 54 (4 years)
  • RF rhizotomy 23 (9 years)
  • Radiosurgery 25 (3 years)
  • Balloon 21 (2 years)
  • MVD 15 (5 years)

Taha J, Tew J Neurosurgery 38865871, 1996
51
Trigeminal Nerve Complications
Taha J, Tew J Neurosurgery 38865871, 1996
52
Radiosurgery for TN
53
Duration and Maintenance of Pain Relief
More than 50 pain relief/Complete relief 1
year 75.8 2.9 63.6 3.3 2 years 71.3
3.3 59.2 3.5 3 years 67.2 3.9 56.6
3.8 3.5 years 65.1 4.3 - 5 years 55.8
9.3 37.7 15.6
54
GKR
55
GKR
56
GKR
57
Decision-Making in TN
  • When should surgery be considered?
  • Success/failure of medical therapy
  • Frequency of recurrences
  • Duration of symptoms
  • Which operation should be done?
  • Age and health of patient
  • Willingness to except facial sensory loss
  • Previous procedures for TN
  • Desires of patient
  • Experience of surgeon

58
Glossopharyngeal Neuralgia
  • Pain most often occurs in the territory of the
    glossopharyngeal nerve
  • GSA input from external/middle ear, posterior
    tongue, and pharnyx
  • Classic GPN pain primarily in tongue and
    pharnyx
  • Otalgic GPN pain primarily occurs in ear
  • Unilateral, paroxysmal, lancinating pain last
    seconds to minutes
  • Pain may occur in clusters
  • Irregular intervals over days, weeks or months
  • Spontaneous occurrence or precipitated by
    swallowing
  • Peak incidence 5th to 7th decade
  • Pain relieved by anesthetizing posteior pharynx
    with 10 cocaine
  • 5 - 8 of cases caused by posterior fossa tumor
  • Pain may be due to elongated styloid process
    (Eagles syndrome)

59
GPN vs. TN
  • TN 70-100x more common than GPN
  • GPN shows no sex predilection
  • TN slightly more common in women (32)
  • GPN occurs more commonly on the left side (32)
  • TN more common on the right (53)
  • Bilateral involvement is uncommon in both
    conditions
  • TN 4 GPN 2
  • Clinical presentation of GPN tends to be more
    variable
  • 10 of patients have BOTH TN and GPN
  • Secondary GPN usually associated with malignant
    skull base neoplasms
  • Secondary TN due to benigng intradural tumor
  • MS almost never encountered in association with
    GPN

60
Treatment of GPN
  • Medications tend to be less effective than in
    patients with TN
  • Microvascular decompression of the 9th and 10th
    cranial nerves
  • Intracranial rhizotomy of 9th nerve and upper
    1/3 of vagus
  • 85 success rate
  • 20 risk of swallowing dysfunction
  • Percutaneous glossopharyngeal rhizotomy

61
Summary and Conclusions
  • All procedures are initially highly effective in
    alleviating the symptoms of TN
  • Each case should be treated individually and
    multiple options should be discussed and offered
    to each patient.
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