慢性炎症性脱髄性多発神経炎に対する大量ヒト免疫グロブリン療法

High-dose Intravenous Immunoglobulin Treatment in Chronic Inflammatory Demyelinating Polyneuropathy

　慢性炎症性脱髄性多発神経炎（CIDP）に対するヒト免疫グロブリン（HIG）の効果を臨床的および電気生理学的に経時的に検討した。対象はCIDP患者2例で症例1は2回のステロイド治療が無効だったもの，症例2には初回治療としてHIGを用いた。HIGは0.4g／kg／dayを経静脈的に5日間投与した。症例1は投与1週後には臨床的に改善を認め，電気生理学的には誘発筋電位の増大とF波の出現をみ，伝導ブロックの改善と考えられた。誘発筋電位は投与後4週間は増大を続けたが，以後悪化した。投与中に軽度の頭痛と皮疹を認めたが，対症療法にて軽快した。症例2は臨床的，電気生理学的に全く改善せず，HIG投与後4週後から開始したステロイドが著効した。CIDPの治療にステロイドが第1選択薬であることは異論のないところであろう。しかし，CIDP患者でステロイドが無効な例，あるいは合併症のためステロイドが投与できない例がある。そのような例ではHIG療法を試みる価値がある。

We treated two patients with chronic inflam-matory demyelinating polyneuropathy (CIDP) with high - dose intravenous immunoglobulin (HIG). The patients received 400mg/kg of immunoglo-bulin a day for five days. One patient, who had failed to respond to prednisolone before, was trea-ted with HIG, 18 months after the onset. His moter symptoms resolved immediately after the com-mencement of HIG. Electrophysiologically, thecompound muscle action potentials increased in amplitude in all nerves examined and F wave reappeared in the left median nerve. The electro-physiological changes were compatible with improvement of conduction blocks. This patient had headache and exanthema during the HIG ther-apy, but they settled after cessation of the infusion. The other patient was administered HIG as an initial treatment, four months after the onset. HIG was of no effect in this case, but he showed remark-able recovery during the following prednisolone therapy. Although corticosteroid therapy is the first choice for CIDP, there are CIDP patients who do not respond to steroid or can not complete the steroid therapy because of adverse effects. HIG is an expectative and recommendable treatment for the steroid-resistant CIDP patients.