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• Vol.54 No.9
• CLINICA NEUROL, 54: 715−720, 2014
• Vol.54 No.9 contents

Case Report

Acute encephalitis with refractory partial status epilepticus treated with early immunotherapies including plasma exchange: a case report

Daisuke Yamamoto, M.D.¹⁾, Tsuyoshi Uchiyama, M.D.¹⁾, Tomoyasu Bunai, M.D.1⁾, Keishiro Sato, M.D.¹⁾, Takako Shimizu, M.D.¹⁾, Keiko Tanaka, M.D.²⁾ and Toshihiko Ohashi, M.D.¹⁾

¹⁾Department of Neurology, Seirei Hamamatsu General Hospital
²⁾Department of Neurology, Kanazawa Medical University Hospital

We describe a patient of acute encephalitis with refractory partial status epilepticus who was successfully treated with early immunotherapy. A 35-year-old male presented with generalized seizures a week after febrile upper respiratory illness. He developed refractory multifocal and generalized seizures despite multiple antiepileptic drug therapies, thereby requiring intubation on the 8^th day after admission. No significant improvement was observed after steroid pulse and intravenous immunoglobulin (IVIG) therapies. On the 18^th day, he received plasma exchange (PE) therapy in combination with intravenous thiamylal and lidocaine to achieve burst-suppression coma. This multidisciplinary treatment led to remission of refractory status epilepticus and subsequent withdrawal from general anesthesia. Although anti-N-methyl-D-aspartate receptor antibodies in cerebrospinal fluid were negative, other neural surface antibodies may responsible for the development of status epilepticus in this case. Clinical features in this case, including previous good health, an antecedent febrile illness, and prolonged treatment-resistant status epilepticus, were similar to those of the acute phase of new-onset refractory status epilepticus (NORSE) syndrome. Consecutive use of glucocorticoids, IVIG, and PE in the early phase was speculated to ameliorate seizures by suppressing abnormal activation of humoral immunity. This indicates that early aggressive immunotherapy may prevent complications resulting from immune-mediated treatmentresistant status epilepticus.
Full Text of this Article in Japanese PDF (2837K)

(CLINICA NEUROL, 54: 715−720, 2014)
key words: autoimmune epilepsy, immunotherapy, NORSE (new-onset refractory status epilepticus)

(Received: 22-Oct-13)