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Tonic Spasms in Multiple Sclerosis Anatomic Basis and Treatment LAWRENCE S * * 723 THE WESTERN JOURNAL OF MEDICINE * JUNE 1991 o* 154 * 6 723 5. Ward DJ: Disseminated granulomatous pneumocystosis in a patient receiving drawn up, and he spoke with effort. There was no headache, aerosolized pentamidine prophylaxis-Poster MBP378, In Abstracts, Vth Interna- tional Conference on AIDS. Montreal, Quebec, 1989, p 284 nausea, vomiting, tinnitus, diplopia, or visual, mental, or 6. Raviglione MC, Mariuz P, Sugar J, Mullen MP: Extrapulmonary Pneumocystis sphincteric disturbances preceding or during the attacks, nor infection (Letter). Ann Intern Med 1989; 1 1 1:339 were any residual sensory or motor deficits noted. 7. Richie TL, Yamaguchi E, Virani NA, Quinn BD, Chaisson RE: Extrapulmonary Pneumocystis infection (Letter). Ann Intern Med 1989; 111:339-340 When these attacks had first started, the patient went to an 8. Dyner TS, Lang W, Busch DF, Gordon PR: Intravascular and pleural involve- emergency department. No episodes were witnessed there, ment by Pneumocystis carinii in a patient with the acquired immunodeficiency syn- drome (AIDS). Ann Intern Med 1989; 111:94-95 and the results of an examination were normal. A diagnosis 9. Unger PD, Rosenblum M, Krown SE: Disseminated Pneumocystis carinii infec- of "muscle spasm: no neurologic disease" was made, and he tion in a patient with acquired immune deficiency syndrome. Hum Pathol 1988; 19:113-1 16 was instructed to consult a neurologist should the episodes 10. Bamett RN, Hull JG, Vortel V, Schwarz J: Pneumocystis carinii in lymph continue. The episodes were initially only a few seconds in nodes and spleen. Arch Pathol 1969; 88:175-180 duration and occurred once to three times per day, but over a 11. Berman SM, Shah B, Wylie FA, Dacosta-Iyer M, McRae DM: Disseminated Pneumocystis carinii in a patient receiving aerosolized pentamidine prophylaxis. West month's time they increased in severity and frequency, lasting J Med 1990; 153:82-86 15 to 60 seconds and occurring as often as three times per 12. Shelhammer JH, Ognibene FP, Macher AM, et al: Persistence ofPneumocystis carinii in lung tissue of acquired immunodeficiency syndrome patients treated for hour. While originally benign, they became troublesome to pneumocystis pneumonia. Am Rev Respir Dis 1984; 130:1161-1165 the patient, interfering with his driving and at one point 13. Centers for Disease Control: Guidelines for prophylaxis against Pneumocystis carinii pneumonia for persons infected with human immunodeficiency virus. MMWR causing him to drop a beverage glass. 1989; 38(Suppl S-5):1-9 His medical history included a period of several months 14. Health and Welfare Canada. Aerosol pentamidine. Dear Doctor 1989 Jul 10, of diplopia at age 12, requiring "visual training classes," but pp 1-5 15. Conte JE Jr, Golden JA: Concentrations of aerosolized pentamidine in bron- resolving without medical or surgical therapy. He had sus- choalveolar lavage, systemic absorption, and excretion. Antimicrob Agents Chemother tained minor injuries in vehicular accidents, but his only 1988; 32:1490-1493 16. Montgomery AB, Debs RJ, Luce JM, et al: Selective delivery of pentamidine operation was a tonsillectomy at age 7. His habits included to the lungs by aerosol. Am Rev Respir Dis 1988; 137:477-478 two-packs-per-day tobacco smoking and moderate daily al- 17. Salamone FR, Cunha BA: Update on pentamidine for the treatment ofPneumo- cohol use. No medications or street drugs were being taken. cystis carinii pneumonia. Clin Pharm 1988; 7:501-5 10 On general physical examination, no abnormalities were noted. His mental state was notable for anxiety, inappro- Tonic Spasms in Multiple Sclerosis Anatomic Basis and Treatment LAWRENCE S. HONIG, MD, PhD PHILIP H. WASSERSTEIN, MD Stanford, California BRUCE T. ADORNATO, MD Palo Alto, Califomia TONIC SPASMS are among several paroxysmal symptoms of multiple sclerosis. Although first described decades ago, they are frequently misdiagnosed, and they have not been well localized. We present the case ofa 31-year-old man with the sole complaint of stereotyped, left-sided, painful tonic spasms primarily affecting the arm, with less involvement of leg and face. Hyperventilation reliably precipitated the spasms. Report of a Case The patient, a 31-year-old right-handed man, was seen because for six weeks he had had intermittent "muscle spasms" involving the left side of his body. They typically began in the left shoulder and progressed distally to the arm, hand, and fingers. There was simultaneous involvement of his left hip and leg. The feeling of muscle tension was ac- companied by uncontrollable shaking and spasmodic move- ments of the affected parts, with the fingers "going every which wayautomatically." In addition, apins-and-needles tin- gling sensation that was unpleasant and painful developed in the left limbs. The left side of the face was involuntarily (Honig LS, Wasserstein PH, Adornato BT: Tonic spasms in multiple sclero- Figure 1.-Brain magnetic resonance imaging: Spin-echo sequences include a sis-Anatomic basis and treatment. West J Med 1991 Jun; 154:723-726) proton-weighted first-echo (TR = 2,000, TE = 20 milliseconds) image (A) that shows a right internal capsule abnormality with greatly increased signal inten- From the Department of Neurology and Neurological Sciences, Stanford Univer- sity on a second-echo = 2,000, TE = 80 milliseconds) image (B) Medical Center. California. T2-weighted (TR sity Stanford, but no signal abnormality on a Ti-weighted (TR = 800, TE = 20 milliseconds) Reprint requests to Lawrence S. Honig, MD, Department of Neurology and Neuro- logical Sciences (H-3 160), Stanford University Medical Center, Stanford, CA 94305- image (C). Two months later, the lesion size and signal intensity are decreased, 5235. as shown in a T2-weighted image (D). 724 ALERTS, NOTICES, AND CASE REPORTS At the initial presentation, brain x-ray computed tomog- ABBREVIATIONS USED IN TEXT raphy showed a small region of lucency in the posterior limb CSF = cerebrospinal fluid of the right internal capsule that failed to enhance with intra- Ig = immunoglobulin MRI = magnetic resonance imaging venous contrast dye. Magnetic resonance imaging (MRI) showed this area, measuring about 2 cm in diameter, as abnormal signal centered in the posterior limb of the right priate affect, social disinhibition, and increased psychomo- internal capsule extending into adjacent thalamus. The lesion tor activity. A cranial nerve evaluation, including fundi, elic- displayed increased signal on the proton-weighted (TR = ited entirely normal findings, as did the motor system 2,000, TE = 20 milliseconds) spin-echo image (Figure 1-A), examination, with normal bulk, tone, strength, gait, and co- and more intense signal on the T2-weighted (TR = 2,000, ordination throughout. Sensory testing revealed mild hypes- TE = 80 milliseconds) image (Figure 1-B), but on a Tl - thesia to light touch, pinprick, and vibration on the left hemi- weighted (TR = 800, TE = 20 milliseconds) pulse sequence body with respect to the midline; proprioception and (Figure 1-C), it possessed normal signal and was not identifi- stereognosis were normal. Muscle stretch reflexes were able. Follow-up imaging two months later, when the patient present and symmetric. No pathologic reflexes were found. was asymptomatic, showed decreased lesion size and signal Laboratory studies included normal serum electrolytes, intensity on proton (not shown) and T2-weighted (Figure 1- general chemistry panels, urinalysis findings, and hemato- D) images. A third MRI study (no figure) done eight months logic studies, with a sedimentation rate of 2 mm per hour. after the onset of facial palsy, when the patient was without Serum was negative for fluorescent treponemal antibody ab- spasms offmedical therapy, revealed some further resolution sorption, VDRL, fluorescent antinuclear antibody, and rheu- of the capsular abnormality, without the appearance of new matoid factor; serum angiotensin-converting enzyme level, lesions. protein electrophoresis, chest roentgenogram, and electro- cardiogram were normal. A lumbar puncture yielded clear, Discussion colorless cerebrospinal fluid (CSF) with an opening pressure This 31-year-old man had the subacute development of of 150 mm of water. Cell counts were erythrocytes, 10 x 106 stereotyped, painful tonic spasms involving the left upper per liter, and leukocytes, 13 x 106 per liter, with 0.98 lym- extremity and the left side of his face. He had a history of a phocytes and 0.02 monocytes; the glucose level was 3.4 childhood period of diplopia and a long-standing behavioral mmol per liter (61 mg per dl), protein 440 mg per liter, and disorder. The spasms could be brought on reliably by hyper- VDRL negative. A CSF protein analysis by agarose electro- ventilation. When carbamazepine therapy was instituted, phoresis showed a normal albumin level of 168 mg per liter both spontaneous and induced attacks disappeared. (normal, 130 to 240) and CSF:serum albumin ratio of 0.004 The differential diagnosis of stereotyped unilateral body (normal, <0.009), reflecting an intact blood-brain barrier. movements without alteration of consciousness includes epi- Elevated levels, however, ofimmunoglobulin (Ig) G of72 mg leptic seizures, psychogenic attacks, carpopedal spasm (tet- per liter (normal, <55), fraction IgG of 0.164 (normal, any), paroxysmal kinesogenic choreoathetosis, and paroxys- <0.105), IgG index of 1.87 (normal, <0.7), and a calcu- mal dystonic choreoathetosis, in addition to the tonic spasms lated IgG synthesis rate of 24 mg per day (normal,
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