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Trigeminal neuralgia


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Trigeminal neuralgia

  1. 1. Trigeminal Neuralgia Yury Khelemsky, MD Assistant Professor Anesthesiology and Pain Medicine The Mount Sinai Medical Center
  2. 2. Introduction <ul><li>Common cause of facial pain </li></ul><ul><li>Sudden, usually unilateral, severe, brief, stabbing/lancinating, recurrent episodes of pain in one or more branches of the 5 th cranial nerve. </li></ul>
  3. 3. Anatomy <ul><li>Sensory supply to face and sensory and motor to muscles of mastication </li></ul><ul><li>3 major divisions: V1 (ophthalmic), V2 (Maxillary), V3 (Mandibular) </li></ul><ul><li>Nerve starts at midlateral surface of pons, sensory ganglion ( gasserian ganglion resides in Meckel ’s cave in the floor of the middle cranial fossa </li></ul>
  4. 4. Epidemiology <ul><li>Annual incidence 4-13/100k </li></ul><ul><li>15k new cases per year in US </li></ul><ul><li>Incidence increases with age (as with PHN). </li></ul><ul><li>Male:Female 1:1.5 </li></ul><ul><li>Rare familial cases. Most sporadic. </li></ul>
  5. 5. Etiology <ul><li>Classic : Most cases (80-90%) due to compression of trigeminal nerve root by aberrant loop of artery or vein. Usually within a few mm of entry into pons. Primary TN also includes idiopathic cases </li></ul><ul><li>Secondary: acoustic neuroma, meningioma, AVN, saccular aneurysm, multiple sclerosis. </li></ul>
  6. 6. Pathogenesis <ul><li>Mechanism: demyelination in area around vascular compression. Ephaptic (occurring without neurotransmitters) crosstalk between light touch and pain fibers. </li></ul><ul><li>Central pain/sensitization develops </li></ul>
  7. 7. Classification <ul><li>International Headache Society (IHS) </li></ul><ul><li>Classic: idiopathic (since most do not have surgery most of these are likely vascular) and vascular </li></ul><ul><li>Secondary: all structural lesions other than vascular compression </li></ul>
  8. 8. Clinical Features 1 <ul><li>Sudden, unilateral, severe, brief, stabbing, electric </li></ul><ul><li>Maximal at or near onset </li></ul><ul><li>May have facial muscle spasm (tix douloureux) </li></ul><ul><li>Refractory period is common </li></ul><ul><li>Typically does not awaken patient </li></ul><ul><li>Unilateral, may be bilateral, but not simultaneously </li></ul>
  9. 9. Clinical Features 2 <ul><li>Mostly V2/3. V1<5%. </li></ul><ul><li>Trigger zones: in distribution of affected nerve, closer to midline, patient protect these areas, may demonstrate on physical exam </li></ul><ul><li>Other triggers: chewing, talking, brushing teeth, cold air, smiling, grimacing </li></ul><ul><li>Pretrigeminal neuralgia – dull aching continuous pain evolving into TN </li></ul><ul><li>May be precipitated by dental procedures </li></ul>
  10. 10. Course <ul><li>Variable </li></ul><ul><li>Episodes may last weeks – months, followed by pain free intervals. </li></ul><ul><li>Recurrence common, some patients have continuous pain </li></ul><ul><li>Most often, TN waxes and wanes in severity and frequency of exacerbations </li></ul>
  11. 11. Diagnosis <ul><li>International Headache Society (IHS) </li></ul><ul><li>Classic: </li></ul><ul><li>Paroxysmal pain in one or more division of CNV </li></ul><ul><li>Pain has at least one: intense, sharp, superficial, or stabbing; precipitated from trigger areas or by trigger factors </li></ul><ul><li>Attacks are stereotyped in the individual patient </li></ul><ul><li>No clinically significant neurologic deficit </li></ul><ul><li>Not attributable to another disorder </li></ul><ul><li>Secondary: demonstrable structure other than vascular compression </li></ul>
  12. 12. Neuroimaging <ul><li>Some obtain MRI in all TN pts </li></ul><ul><li>Some only in: young patients, bilateral sx, trigeminal sensory loss </li></ul><ul><li>American Academy of Neurology (AAN) and European Federation of Neurologic Societies (EFNS) review </li></ul><ul><li>Routine imaging identified secondary cause in 15% of patients </li></ul><ul><li>Insufficient evidence to support or refute utility of MRI to identify neurovascular compression or indicate most reliable MRI technique. </li></ul>
  13. 13. Electrophysiologic Testing <ul><li>Trigeminal Reflex Tests: Blink Reflex and Masseter Inhibitor Reflex. 2008 AAN/EFNS: high sensitivity (94%) and specificity (87%) for distinguishing between secondary and classic TN </li></ul><ul><li>Trigeminal Evoked Potentials: not clinically useful </li></ul>
  14. 14. DDx <ul><li>Short-Lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing (SUNCT) </li></ul><ul><li>Cluster-tic syndrome </li></ul><ul><li>Jabs and jolts syndrome </li></ul><ul><li>Other neuralgias </li></ul>
  15. 15. Treatment: Medical <ul><li>Carbamazepine - complete or near complete pain control attained in 58 to 100 percent of patients, compared with 0 to 40 percent of patients on placebo </li></ul><ul><li>NNT <2 </li></ul><ul><li>sometimes poorly tolerated, with numbers needed to harm for minor and severe adverse events of 3 and 24 respectively. </li></ul>
  16. 16. Treatment: Medical Carbamazepine <ul><li>The usual starting dose 100 to 200 mg Q12h. </li></ul><ul><li>Increase by 200 mg daily as tolerated until sufficient pain relief is attained. </li></ul><ul><li>The typical maintenance dose is 600 to 800 mg/day, given in two divided doses for tablets and extended release capsules, or four divided doses when for oral suspension. </li></ul><ul><li>The maximum suggested total dose is 1200 mg daily. </li></ul>
  17. 17. Treatment: Medical Carbamazepine <ul><li>Adverse effects carbamazepine: drowsiness, dizziness, nausea and vomiting; slow titration may minimize these effects. </li></ul><ul><li>Carbamazepine-induced leukopenia is not uncommon, but it is usually benign; aplastic anemia is a rare side effect. </li></ul>
  18. 18. Treatment: Medical Carbamazepine <ul><li>The HLA-B*1502 allele is a genetic susceptibility marker in Asians that is associated with an increased risk of developing Stevens-Johnson syndrome and/or toxic epidermal necrolysis. </li></ul><ul><li>For most patients of Asian ancestry, genetic testing for the presence of this marker is recommended by the manufacturer prior to initiation </li></ul>
  19. 19. Treatment: Medical: Oxcarbazepine <ul><li>equally effective as carb., decreased SE, with a >50 percent reduction of attacks achieved by 88 percent or more of patients in both treatment groups. </li></ul><ul><li>started at a total dose of 600 mg daily, given in two divided doses. </li></ul><ul><li>The dose can be increased as tolerated in 300 mg increments every third day to a total dose of 1200 to 1800 mg daily. </li></ul>
  20. 20. Treatment: Medical Baclofen <ul><li>Limited evidence from a small double-blind crossover trial </li></ul><ul><li>Treatment with baclofen 40 to 80 mg daily resulted in a reduction in paroxysms in 7/10 patients with typical TN, compared with 1/10 placebo. </li></ul><ul><li>The starting dose 15 mg daily given in three divided doses, with gradual titration to a maintenance dose of 50 to 60 mg per day. </li></ul><ul><li>drug should be discontinued slowly since seizures and hallucinations have been reported with upon withdrawal. </li></ul>
  21. 21. Treatment: Medical Opioids <ul><li>no controlled data regarding the efficacy of opioids in TN </li></ul><ul><li>may help make the pain bearable while other, more effective and long-term, treatments take effect. </li></ul><ul><li>may be effective at lower doses when combined with neuropathic agents </li></ul>
  22. 22. Treatment: Medical Other treatments <ul><li>Small open label studies have suggested benefit with a number of medications used for TN: phenytoin, valproic acid, gabapentin, pregabalin, clonazepam, topiramate, misoprostol (with MS) </li></ul><ul><li>Lidocaine IV – 100-300mg over 30 minutes. </li></ul><ul><li>No controlled trial comparing monotherapy with combination therapy </li></ul>
  23. 23. Treatment: Interventional Overview <ul><li>Microvascular decompression </li></ul><ul><li>Ablative procedures, including: </li></ul><ul><li>Rhizotomy with either radiofrequency thermocoagulation, mechanical balloon compression, or chemical (glycerol) injection </li></ul><ul><li>Gamma knife radiosurgery </li></ul><ul><li>Peripheral neurectomy and nerve block </li></ul>
  24. 24. Treatment: Interventional Microvascular Decompression <ul><li>Craniotomy </li></ul><ul><li>Initial pain relief 90%, 1 yr. (80%, 3 yr. (75%), 5 yr. (73%) </li></ul><ul><li>0.2% mortality </li></ul><ul><li>4% major adverse events: CSF leak, infarction, hematoma </li></ul><ul><li>11% aseptic meningitis </li></ul><ul><li>10% long term hearing loss </li></ul><ul><li>7% sensory loss </li></ul>
  25. 25. Treatment: Interventional Rhizotomy <ul><li>Percutaneous procedures via foramen ovale </li></ul><ul><li>RF thermocoagulation, mechanical balloon compression, chemical neurolysis (0.1-0.4% glycerol) </li></ul><ul><li>2008 AAN/EFNS: initial pain relief 90%, 1 yr. (68-85%), 3 yr. (54-64%), 5 yr. (50%) </li></ul><ul><li>0.2% aseptic meningitis </li></ul><ul><li>12 % dysesthesia (burning, aching, heavy) </li></ul><ul><li>50% sensory loss </li></ul><ul><li>4% anesthesia dolorosa </li></ul><ul><li>4% corneal numbness with risk of keratitis </li></ul>
  26. 26. Treatment: Gasserian Ganglion Block Submental oblique image of foramen ovale Yin W. Radiofrequency gasserian rhizotomy: The Role of RF Lesioning in the Management of Facial Pain. Techniques in Regional Anesthesia and Pain Management 2004; 8(1): 30-34.
  27. 27. Treatment: Interventional Gamma Knife Radiosurgery <ul><li>Beams aimed at proximal trigeminal root causing axonal degeneration and necrosis </li></ul><ul><li>Pain relief lags by a month </li></ul><ul><li>1 yr. (69%), 3 yr. (52%) </li></ul><ul><li>9-37% new or worsened facial sensory impairment </li></ul><ul><li>Anesthesia dolorosa very rare </li></ul><ul><li>May be more effective as a first intervention rather than second </li></ul>
  28. 28. Treatment: Interventional Peripheral Neurectomy <ul><li>Branches of trigeminal – supraorbital, infraorbital, alveolar, lingual. </li></ul><ul><li>Incision, alcohol, RF, cryotherapy </li></ul><ul><li>AAN/EFNS – evidence is negative or inconclusive </li></ul>
  29. 29. Treatment Algorithm
  30. 30. References <ul><li>UpToDate 2010: Trigeminal Neuralgia </li></ul><ul><li>Han I, Shin D, Chang J, Kim K, Chang J, Huh R, Chung Effect of Various Surgical Modalities in Recurrent or Persistent Trigeminal Neuralgia. Stereotact Funct Neurosurg 2010;88:156-162 </li></ul>