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1歳時より100mg/kgのガンマグロブリン(γ-gl)を月1回静注されていたBruton型無γ-gl血症の患者が,慢性エンテロウイルス髄膜炎のため,10歳時に水頭症を発症し当科に入院した。入院後は脳室ドレナージを行い,400mg/kgのγ-glを月1回静注しながら,125〜250mgのγ-glを週1回(8週間で総投与量1.5g)脳室内に投与したところ髄膜炎症状は改善した。Bruton型無γ-gl血症のエンテロウイルス髄膜炎は,慢性化すると水頭症を合併し治療が困難になるため,髄膜炎の発症後はできるだけ早期に,γ-glの脳室内投与を開始すべきと考えられた。
We report a 10-year-old boy with chronic enter-oviral meningitis associated with agamma-globulinemia (CEMA) and hydrocephalus. He was treated with a low-dose intravenous administration (100mg/kg/4 weeks) of gammaglobulin (γ-gl) since he was diagnosed as having Bruton type agam-maglobulinemia at 1 year of age. At this admis-sion, neurological examination revealed meningeal signs, Babinski sign, frontal signs, urinary inconti-nence, and mental retardation (IQ=48) which was considered to be a sequela of the enteroviral enceph-alitis which had occurred in his first year of life.
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