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• Vol.50 No.8
• CLINICA NEUROL, 50: 585−588, 2010
• Vol.50 No.8 contents

Brief Clinical Note

Anti-NMDA receptor antibody encephalitis with recurrent optic neuritis and epilepsy

Rie Motoyama, M.D.¹⁾, Kazuhiro Shiraishi, M.D.²⁾, Keiko Tanaka, M.D.³⁾, Masako Kinoshita, M.D.⁴⁾ and Masami Tanaka, M.D.¹⁾

¹⁾MS Center, Utano National Hospital
²⁾Department of Neuropediatrics, Utano National Hospital
³⁾Department of Neurology, Kanazawa Medical University
⁴⁾Department of Neurology, Utano National Hospital

A previously healthy, 10-year-old girl developed left optic neuritis that treated with oral prednisolon (PSL). During the following 8 months, the patient exhibited right optic neuritis 3 days after discontinuation of PSL therapy and three episodes of epileptic seizures 3 weeks after PSL withdrawal. Cerebrospinal fluid (CSF) examination revealed pleocytosis (mononuclear cells), increased IgG index, and positive oligoclonal IgG expression. Brain MRI showed multiple cortical, subcortical, and leptomeningeal enhanced lesions. However, spinal cord MRI revealed no lesions. Neither autoantibodies to nuclear, thyroid, α-enolase, glutamic acid decarboxylase, nor aquaporin 4 was detected. However, anti-NMDA receptor antibodies (NMDAR-Ab) were present in her CSF. This patient is the second reported case of NMDAR-related encephalitis with recurrent optic neuritis. The possibility of seronegative neuromyelitis optica (NMO) could not be ruled out for the symptom of recurrent optic neuritis. However, the presence of NMDAR-Ab in the CSF together with increased IgG index and oligoclonal IgG bands, which are usually negative in NMO suggested that this patient is NMDAR-related encephalitis combined with rare symptom of optic neuritis for this type of encephalitis, though we need to wait larger number of patients' accumulation to conclude that the optic neuritis could be one of the features of NMDAR-related encephalitis.
Full Text of this Article in Japanese PDF (569K)

(CLINICA NEUROL, 50: 585−588, 2010)
key words: Anti-NMDA receptor antibodies encephalitis, anti-aquaporin 4 antibodies, multiple sclerosis, neuromyelitis optica, optic neuritis

(Received: 21-May-10)