[Autoimmune encephalitis with anti-glutamate receptor antibody presenting as epilepsia partialis continua and action myoclonus: a case report].

Abstract

A 19-year-old man was admitted to our hospital with tremor and myoclonus that appeared after several episodes of consciousness disturbance and generalized convulsions. While steroid therapy resolved these symptoms, epilepsia partialis continua (EPC) and action myoclonus developed. Clobazam improved the EPC, but action myoclonus persisted. Oral tandospirone (30 mg/day) was given because 5-hydroxyindole acetic acid (5-HIAA) was markedly decreased in the cerebrospinal fluid (CSF). After 10 days of this therapy, most action myoclonus disappeared and he could perform fine motor skills. Although the MR structural images were unremarkable, cerebral SPECT showed decreased uptake in the left thalamus and bilateral frontal lobes. The antibody against glutamate receptor subunit epsilon2 was positive in the CSF. This is the first report of autoimmune encephalitis with anti-glutamate receptor antibody presenting as low level of 5-HIAA in the CSF. Tandospirone was effective for action myoclonus.

• Authors: Yuji Kato, Yoshihiko Nakazato, Naotoshi Tamura, Ryo Tomioka, Yukitoshi Takahashi, Kunio Shimazu
• Journal: Rinsho shinkeigaku = Clinical neurology (Rinsho Shinkeigaku) Vol. 47 Issue 7 Pg. 429-33 (Jul 2007) ISSN: 0009-918X [Print] Japan
• PMID: 17710887 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Autoantibodies
• Immunoglobulin G
• Receptors, Glutamate

Topics

• Adult
• Autoantibodies (cerebrospinal fluid)
• Autoimmune Diseases (complications, immunology)
• Diagnosis, Differential
• Encephalitis (complications, immunology)
• Epilepsies, Partial (etiology)
• Humans
• Immunoglobulin G (cerebrospinal fluid)
• Male
• Myoclonus (etiology)
• Receptors, Glutamate (immunology)