無動無言と緊張病性興奮から完全に回復したCNSループスの1例

Complete Recovery from Akinetic Mutism and Catatonic Excitation in a Patient with CNS Lupus

【抄録】　37歳の女性で，抑うつ，発動性・感情反応性の低下，健忘を主徴とする精神症状で発症。抗核抗体陽性，脳MRIのT2WIにて可逆性高信号域，髄液所見にてIgG indexとinterleukin-6の上昇を認め，全身性エリテマトーデスに特徴的な他の身体所見は認めなかったが，中枢神経ループスと診断した。ステロイドパルス療法により完全寛解したが，再発して無動無言と間欠性の緊張病性興奮を呈した。ステロイドパルス療法により急性期は脱したが，残存した発動性・感情反応性の低下と健忘にはシクロホスファミドの経口投与が著効，町び完全な回復に至った。症候的にも稀な中枢神経ループスの1例を画像所見，免疫学的検査所見の推移とともに報告した。

　We report a case of severe central nervous system (CNS) lupus in which the symptoms presented were akinetic mutism and catatonic excitation, which were successfully managed by corticosteroid pulse therapy and additional oral cyclophosphamide. The patient, a 37-year-old woman, suffered temporarily dizziness and diplopia in May, 1996. She was admitted to our hospital in December, 1996, because of depression, lack of spontaneity and amnesia. She also had generalized convulsions. On the basis of reversible abnormal intensity lesions on brain MRI, positivity for serum anti-nuclear antibody and low complement levels, as well as elevation of the IgG index and interleukin-6 (IL-6) level in her cerebrospinal fluid (CSF), she was diagnosed as having CNS lupus, although the diagnostic criteria for systemic lupus erythematosus were not met because the only symptoms were neuropsychiatric. Methylprednisolone (mPSL) pulse therapy ameliorated the psychiatric symptoms, but in February, 1998, the patient was readmitted because of the development of akinetic mutism and intermittent catatonic excitation with stereotypy. The IgG index and IL-6 level were again elevated, and anti-neuronal antibody was detected in the CSF. Hypoperfusion in the right parietooccipital area was depicted by ¹²³I-SPECT. Electroencephalographic data were within the normal ranges. Haloperidol was ineffective. After two sessions of mPSL pulse therapy, the acute symptoms were resolved, but lack of spontaneity and amnesia remained. For these symptoms, another session of mPSL pulse therapy was ineffective. However, the patient showed a favorable response to additional oral cyclophosphamide and recovered completely from her residual symptoms.