プレドニソロンが著効を奏した先天性筋緊張症の2例

TWO CASES OF MYOTONIA CONGENITA (THOMSEN) TREATMENT WITH PREDNISOLONE

序言

　先天性筋緊張症のキニーネ治療は，1936年Wolf^1）2）につて導入されて以来，今日まで殆んど唯一の治療法とされてきた。キニーネ療法は，時代の試練を経たものだけに，確かに有効ではあるが，その効果持続期間が短く，長期投与を必要とするに拘らず，次第にキニーネに対する抵抗性が出現し来り，最終的には全く無効化し易いという欠点がある。勿論キニーネ投与と休止期間とを交互に交代させる方法がとられるが，この方法でもMertens und Grüttner^3）の16症例は全例共結局抵抗性になつたという。

　最近脳下垂体・副腎皮質ホルモンが筋緊張症状に対し卓効を有することが，世界各国から報告されてきた^4）−13）。私達は先天性筋緊張症の2例に対してプレドニソロン経口投与を行い，キニーネよりむしろ優れた治療効果を収めることができた。実地診療の面に於ても広く試みらるべき治療法であると信ずるのみならず，又筋緊張症の病態生理ならびに本態（萎縮性筋緊張症との関係）についても，一つの大きな示唆を与えると思われるので，報告する次第である。

The author reported two cases of myotonia congenita (Thomsen) whose clinical signs and symptoms were rather typical.

This report seems to be valuable by the fact that very remarkable effects were ob-tained by prednisolone, even superior to that of quinine.——the effects of prednisolone were consisted not only with subjective well-being, increased muscle power and motor abil-ity, but also with almost complete disappea-rance of involuntary myotonic reactions which was clearly demonstrated by electromyogra-phy.

The effects, though temporary likewise, were more prompt and more complete than that of quinine.

The mechanism of hormone action against to myotonia still remains a mystery. The following two possible ways of explanation were indicated, but both could not be consid-ered to be adequate. The first of these is from the standpoint of etiological interrelat-ionship of myotonia congenita with myotonia dystrophica, and the second from the action of hormones upon the membrane permeability via changes of electrolyte balance.