A Case of Myoclonic Astatic Epilepsy with Autoantibody for Glutamate Receptor .EPSILON.2

DOI PubMed 1 Citations 9 References

Bibliographic Information

Other Title
  • グルタミン酸受容体ε２（ＧｌｕＲε２）抗体陽性を示したミオクロニー失立発作てんかんの男児例

Search this article

Abstract

A 3-year-old boy was admitted to our hospital with repetitive drop attacks and generalized tonic-clonic seizures. Brain MRI, SPECT and blood laboratory tests did not show any abnormalities, while antibody to glutamate receptor ε2 (GluR ε2) in spinal fluid was positive. Interictal EEG showed generalized 6-7 Hz slow, wave and ictal EEG showed 1-2 Hz high amplitude generalized spike and slow wave burst. We made a diagnosis as myoclonic astatic epilepsy (MAE). However, his seizures were refractory to almost all antiepileptic drugs, steroid pulse therapy and γ-globulin therapy. Eight months after the first attack, administration of ACTH therapy was effective. Seizures disappeared and EEG findings improved. To our knowledge, there have been no previous reports of MAE in which autoantibody to GluR a 2 was positive. It is suggested that autoimmunity in this case was associated with the pathogenesis of MAE.

Journal

  • NO TO HATATSU

    NO TO HATATSU 40 (1), 38-41, 2008

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

Citations (1)*help

See more

References(9)*help

See more

Related Projects

See more

Keywords

Details 詳細情報について

Report a problem

Back to top