所謂青年性家族性震顫麻痺の一家系について

1) 私は母系二代に亙つて4名(同胞3名と母の弟)の震顫麻痺患者を出した一家系を経験した. 2) これらの例は20才代に発病せるもの2例, 30才代に発病したと推定されるもの2例で所謂Willigeの青年性家族性震顫麻痺と考えられるものであつた. 3) これら症例の2例に定型的震顫麻痺症状を認めたが,他の2例は本病の不全型と思われるものであつた,且本病の発生機序に於て可成の類似点を見た.即ち各例共に震顫は下肢に始り,震顫は筋強剛よりも優位にあり,経過は慢性進行性であつた. 4) 精神症状に顕著な障碍は認められなかつたが病機の進行と共に性格気質の変化を証明した. 5) 本家系に於ける同胞罹患例の父母は従同胞婚であり,母の弟に1名の精神病者を見出したが老年痴呆患者は認めなかつた. 6) 本病の病因を流行性脳炎或は老年現象とする説,即ち本病を全て症候性Parkinsonismusとする説に左袒する事は出来ない,寧ろ遺伝退行性変化に基くものであると考え,且この意味に於て優性遺伝形式をとるものと思われた.

The author submits here a report on four cases of paralysis agitans that occurred extending over two generations in the mother's lineage (two brothers, their sister, and their maternal uncle. Two brothers fell ill in their twenties, and the other two in their thirties. Therefore, it may be said that this disease belongs to what is called familiar juvenil paralysis agitans by Willige. Two cases gave the typical symptoms of paralysis agitans while the rest presented atypical symptoms, that is "formes frustes". In the observation of starting mechanism of their disease, the following symptoms were revealed : Tremor began from the lower part of legs earlier than muscle rigidity. Moreover, the process of this disease was progressive-chronic. No remarkaple changes in mental state could be recognized, but the changes in temperaments and characters were noticed as this disease proceeded. The family history shows that their father was married to his cousin, and their mother's youngest brother died of psychose. The other members of their family, however, were free from psychiatric disease especially senile psychose. Today, in regard to the cause of paralysis agitans, various theories have been advanced, for instance, symptomatic disorder theory according which this disease is caused by senile process or encephalitis, and heredodegenerative theory, etc. The author is inclined to consider that paralysis agitans is due to the heredodegenerative disease and that it may be dominant heredity.