TRIGEMINAL NEURALGIA (TIC DOULOUREUX) BASICS DESCRIPTION A disorder of the sensory nucleus of the 5th cranial nerve (trigeminal

nerve), producing episodic, paroxysmal, severe lancinating pain lasting seconds to minutes followed by a pain free period in the distribution of one or more of its divisions. Often precipitated by stimulation of well-defined, ipsilateral trigger zones, usually perioral, perinasal, occasionally intraoral (eg washing, shaving). System(s) affected: Nervous Genetics: N/A Incidence/Prevalence in USA: 16/100,000 Predominant age: Over age 50, peak age 60, rare before age 35 Predominant sex: Female > Male (2:1) SIGNS & SYMPTOMS Unilateral (< 4% bilateral, rarely at the same time; bilateral mostly in MS), symptoms rarely present at night Excruciating lip pain Excruciating gum pain Excruciating cheek pain Paroxysmal facial pain Wincing Pain elicited by tickle or touch Flushing Lacrimation Salivation Pain bursts several seconds to minutes with refractory period after Right > left side preference 2nd > 3rd >> 1st (less than 5%) division trigeminal nerve most commonly affected CAUSES When present, most commonly compression of the trigeminal nerve by anomalous arteries or veins of the posterior fossa, usually the superior cerebral artery compressing the trigeminal root. Etiology classifi cation Idiopathic Secondary - disseminated sclerosis; cerebellopontine angle tumors, e.g., meningioma; tumors of the 5th nerve, e.g., neuroma, vascular malformations RISK FACTORS Unknown DIAGNOSIS LABORATORY N/A Drugs that may alter lab results: N/A Disorders that may alter lab results: N/A PATHOLOGICAL FINDINGS Semilunar ganglion - infl ammatory changes Degenerative changes DIAGNOSTIC PROCEDURES MRI or CT scan - neoplasm in cerebellopontine angle must be ruled out. Special MRA technique of collapsed MRA superimposed on routine spin echo T-1 weighted images.

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TREATMENT APPROPRIATE HEALTH CARE Outpatient GENERAL MEASURES Drug treatment is fi rst approach. Invasive procedures for patients who cannot tolerate, or fail to respond to, drug treatment. Avoidance of stimulation (air, heat, cold) of trigger zones (lips, cheeks, gums) 4% tetracaine dissolved in 0.5% bupivacaine nerve block (only a few case reports to date) 25-50% of TN patients eventually fail medical treatment Alcohol block or glycerol injection into the trigeminal cistern - unpredictable side effects (dysesthesia and anesthesia dolorosa); temporary SURGICAL MEASURES Microvascular decompression of the 5th cranial nerve at its entrance to (or exit from) the brainstem (70-90% effective) Partial sensory rhizotomy Peripheral block or section of 5th nerve proximal to the Gasserian ganglion Gamma knife radiosurgery (minimally invasive, 77% significant relief) Balloon compression of the Gasserian ganglion (especially effective for 1st division TN pain) Peripheral nerve ablation Radiofrequency thermocoagulation (possibly 90-97% partial or complete relief; recurrence rate is unknown) Neurectomy Cryotherapy - good initial results; considerable relapse rate ACTIVITY Full activity DIET No special diet PATIENT EDUCATION Instruct regarding medication dosage and side effects MEDICATIONS DRUG(S) OF CHOICE Carbamazepine (Tegretol) starting dose 100-200 mg bid; effective dose usually 200 mg qid; 1200 mg/day maximum. By 3 years, 30% no longer helped. Most common side effect is sedation. Contraindications: MAO inhibitors taken concurrently Precautions: Use with caution in presence of liver disease Significant possible interactions: Macrolide antibiotics with Carbamazepine. Oral anticoagulants, anticonvulsants, tricyclics, oral contraceptives, steroids, digitalis, INH, MAO inhibitors, methyprylon nabilone, nizatidine, other H2 blockers, phenytoin, propoxyphene, benzodiazepines, calcium, channel blockers. ALTERNATIVE DRUGS Phenytoin (Dilantin) 300-400 mg/day (synergistic with carbamazepine) Baclofen (Lioresal) 10-80 mg/day; start at 5-10 mg tid with food (as adjunct with phenytoin or carbamazepine): drowsiness, weakness, nausea, vomiting Gabapentin (Neurontin) 100 mg tid or 300 mg qhs, increased up to 300-600 mg tid-qid Lamotrigine - (10% rash) Chlorphenesin carbamate (Maolate) 800-2400 mg/day: drowsiness, (as adjunct with phenytoin and/or carbamazepine) Oxcarbazepine (Trileptal) - derivative of carbamazepine also similar to gabapentin. Faster with less drowsiness. Decreases serum sodium. Clonidine - stimulates inhibitory bulbospinal pain pathways that are norepinephrine dependent. Second line after anticonvulsants and/or antidepressants. Antidepressants, especially used with anticonvulsants. Particularly effective for atypical forms of TN - amitriptyline, fluoxetine, trazodone. Clonazepam (Klonopin): frequently causes drowsiness and ataxia Mexiletine (Mexitil). Experimental for this condition. Narcotics - controversial

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 Capsaicin cream (not standard therapy; anecdotal evidence) Pimozide (Orap) superior to carbamazepine in one study of 48 patients, but it is an antipsychotic drug with extrapyramidal affects, dystonia and tardive dyskinesia. High side effect profi le. Valproic acid (Depakene, Depakote) FOLLOWUP PATIENT MONITORING Carbamazepine and/or phenytoin serum levels If carbamazepine is prescribed: CBC and platelets at baseline then weekly for a month, then monthly for 4 months, then every 6-12 months if dose stable (regimens for monitoring vary) CBC as needed PREVENTION/AVOIDANCE Reduce drugs after 4-6 weeks to determine if condition is in remission, resume at previous dose if pain recurs. Withdraw drugs slowly after several months again to check for remission or if lower dose of drugs can be tolerated. POSSIBLE COMPLICATIONS Mental and physical sluggishness, dizziness with carbamazepine EXPECTED COURSE/PROGNOSIS Exacerbations in fall and spring; otherwise good MISCELLANEOUS AGE-RELATED FACTORS Pediatric: Unusual in childhood Geriatric: N/A Others: N/A PREGNANCY N/A REFERENCES Vaillancourt PD, Largevin HM. Painful peripheral neuropathies. Med Clin NA 1999;83(3):627-42 Bell WE: Orofacial Pain. 4th Ed. Chicago, Year Book Medical Publishers, 1989 Adams RD, Victor M: Principles of Neurology. 5th Ed. New York, McGraw-Hill, 1993 Sweet WH: The treatment of trigeminal neuralgia (tic douloureux). New Engl J Med 1986;174-177 Moller AR: The cranial nerve vascular compression syndrome: A review of treatment. Acta Neurochirurgica 1991;113:18-23 Merrill R, Graff-Radford SB: Trigeminal neuralgia: How to rule out the wrong treatment. JADA 1992;123:63-68 Smith LH Jr, Bennett C: Trigeminal neuralgia. In: Wyngaarden JB, ed. Cecil Textbook of Medicine. Philadelphia, W.B. Saunders Co., 1992 Fields HL: Treatment of trigeminal neuralgia (editorial). NEJM 1996;334(17):11251126 Web references: 2 available at www.5mcc.com

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