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• Vol.47 No.10
• CLINICA NEUROL, 47: 629−634, 2007
• Vol.47 No.10 contents

Original Article

Non-herpetic acute limbic encephalitis-like manifestation in a case of Hashimoto's encephalopathy with positive autoantibodies against ionotropic glutamate receptor ε2

Akihiro Shindo, M.D.¹⁾, Yuichiro Ii, M.D.¹⁾, Ryogen Sasaki, M.D.¹⁾, Yukitoshi Takahashi, M.D.²⁾, Makoto Yoneda, M.D.³⁾ and Shigeki Kuzuhara, M.D.¹⁾

¹⁾Department of Neurology, Mie University Graduate School of Medicine
²⁾National Epilepsy Centre, Shizuoka Institute of Epilepsy and Neurological Disorders
³⁾Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui

A 37-year-old woman developed memory disturbance and seizures preceded by headache and high fever. Physical examination revealed persistent high fever, confusion, neck stiffness, anterograde and retrograde amnesia and disorientation. CSF showed pleocytosis and normal glucose level, and negative herpes simplex virus DNA on PCR. The fluid attenuated inversion recovery (FLAIR) MRI of the brain demonstrated nearly symmetric high signal intensity areas in the bilateral mesial temporal lobes. The tentative clinical diagnosis was non-herpetic acute limbic encephalitis (NHALE), and administration of methylprednisolone improved her conditions. Laboratory tests revealed mild hypothyroidism with high titers of serum antibodies against TPO and thyroglobulin, consistent with Hashimoto's disease. In addition, antibodies against amino terminal of α-enolase in the serum and those against glutamate receptor (GluR) ε2 in the serum and CSF were positive. A final diagnosis of Hashimoto's encephalopathy associated with GluRε2 antibody was made. The present case suggests that NHALE-like clinical manifestation can be produced by autoimmune-mediated encephalopathies.

(CLINICA NEUROL, 47: 629−634, 2007)
key words: non-herpetic acute limbic encephalitis, Hashimoto's encephalopathy, anti-GluR antibody, anti α-enolase antibody, autoimmune encephalitis

(Received: 18-Jun-07)