筋ヂストロフィー症を合併せる周期性四肢麻痺の本邦における第1家系について

The first report of periodic paralysis with progressive muscular dystrophy in Jasan.

緒論

　吾々は最近父子二代にわたる累代發生を來した周期性四肢麻痺を有する一家族を經驗し，その家族員の1名を入院せしめて検索する機會を得た。この入院せしめた患者は進行性筋ヂストロフイー症を合併せる極めて稀な症例であり，外國においてはBernhardt，Oppenhein，Wexyberg，Mankowsky等の報告があるが，本邦においては未だその報告をみない。吾々はこの症例に關して興味ある事項を報告し，且つ家族についても併せて調査を行つたので，注目すべき諸點について報告したい。又周期性麻痺の本態に關し，從來種々議論されているところであるが，吾々はD. O. C. A. による周期性麻痺發作の誘發に成功し，本症の原因，發生機轉について一つの有力な示唆を得たので併せてこれを報告する。

　家系　家系圖は第1圖に示す如くであるが本患者の家系においては，父において始めて周期性麻痺を發生したものと考えられる。本家族においては周期性麻痺發作は父子二代にわたり，子供は7人中5人も發作を有している。發作のない第6子に先天性白内障がある。本家系には血族結婚はみとめられない。次に入院患者19歳♂についてその臨床検査成績の結果を記せば次の如くである。

　The reports of periodic paralysis with progressive Muscular dystrophy are meagre except only four caseses by Bernhardt, Oppenheim, Wexberg ane Mankowsky. Recently we experienced one family of periodic paralysis in which father and his children (five of seven) were suffering from it. In that family, one patient (in the age of 19) was accompanied by progressive muscular dystrophy. His dystrophic syndrome was disclosed in entering to the elementary school, and the type of dystrophy was scapulo-humeral one. The other patients suffering from periodic paralysis also had tendency to progressive muscular dystrophy. We observed hypervolume of M. gastrocnemius, but could not find muscular atrophy. Onset of periodic paralysis in that family was in a year and three months after their birth, and that condition was worst in their puberty. Almost patienets suffering from periodic pasalysis hadGoiter, convergence-insufficiency, exohpthalmos, mydriasis and reacted remarkably on adrenalin. With various investigations we recognized the distinct difference between the patients of periodic paralysis and the rests of family. We succceeded in provocation of periodic paralysis using D.O.C.A. We examined the X-Ray of turky saddle (three cases of six patients produced bridge), bridge-Syndrome, and Thorn's test (-33%). From the results of these experiments adreno-cortical insufficiency or disorder of pituitary-adrenal system to have close relation with mechanism of periodic paralysis.