Abstract Partial motor status epilepticus (PMSE) is characterized by motor seizures that "are so frequently repeated or so prolonged as to create a fixed and lasting condition."1 These epilepsies are associated with different types of diseases, including infectious, vascular, neoplastic, demyelinating, and traumatic ones as well as those of metabolic origin.2 We report a case of simple PMSE characterized by alternating or bilateral tonic contraction preceded by brief hallucinatory and forced-thinking episodes, which revealed Waldenström macroglobulinemia (WM). Although this lymphoproliferative disorder frequently involves the nervous system,3,4 this is apparently the first report of an association with PMSE and of complete recovery after chemotherapy. Moreover, the presence of antiglycolipid antibodies that have been reported to be potentially epileptogenic in rats5 and humans6 suggested that an autoimmune mechanism was responsible for status epilepticus occurrence. A 64-year-old, right-handed man with an unremarkable medical history was first evaluated in January References 1. Gastaut H. Classification of status epilepticus . In: Delgado-Escueta AV, Wasterlain CG, Treiman DM, Porter RJ, eds. Status Epilepticus . New York, NY: Raven Press; 1983:15-35. 2. Schomer DL. Focal status epilepticus and epilepsia partialis continua in adults and children . Epilepsia . 1993;34( (suppl 1) ):S29-S36.Crossref 3. Logothetis J, Silverstein P, CoeJ. Neurologic aspects of Waldenström's macroglobulinemia: report of a case . Arch Neurol . 1960;5:564-673.Crossref 4. MacKenzie MR, Fudenberg HH. Macroglobulinemia: an analysis of forty patients . Blood . 1972;39:874-889. 5. Karpiak SE, Mahadik SP, Graf L, Rapport M. An immunological model of epilepsy: seizures induced by antibodies to GM1 ganglioside . Epilepsia . 1981; 22:189-196.Crossref 6. Bartolomei F, Boucraut J, Barrie M, et al. Cryptogenic partial epilepsies with anti-GM1 antibodies: a new form of immune-mediated epilepsy? Epilepsia . 1996;37:922-926.Crossref 7. Binnie CD, Prior PF. Electroencephalography . J Neurol Neurosurg Psychiatry . 1994;57:1308-1319.Crossref 8. Stern TA, Purcell JJ, Murray GB. Complex partial seizures associated with Waldenström's macroglobulinemia . Psychosomatics . 1985;26:890-892.Crossref 9. Steck AJ, Murray N, Dellagi K, Brouet JC, Seligmann M. Peripheral neuropathy associated with monoclonal IgM autoantibody . Ann Neurol . 1987;22:764-767.Crossref 10. Aarli JA. Immunological aspects of epilepsy . Brain Dev . 1993;15:41-49.Crossref 11. Rogers SW, Andrews PI, Gahring LC, et al. Autoantibodies to glutamate receptor 3 in Rasmussen's encephalitis . Science . 1994;265:648-651.Crossref 12. Pestronck A. Motor neuropathies, motor neuron disorders, and antiglycolipid antibodies . Muscle Nerve . 1991;14:927-936.Crossref 13. Rowland LP, Sherman WL, Hays AP, et al. Autopsy-proven amyotrophic lateral sclerosis, Waldenström's macroglobulinemia, and antibodies to sulfated glucuronic acid paragloboside . Neurology . 1995;45:827-829.Crossref 14. Nobile-Orazio E, Manfredini E, Carpo M, et al. Frequency and clinical correlates of anti-neural IgM antibodies in neuropathy associated with IgM monoclonal gammapathy . Ann Neurol . 1994;36:416-424.Crossref
Archives of Neurology – American Medical Association
Published: Oct 1, 1997
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