古典的Pelizaeus-Merzbacher病の1剖検例

An autopsy case of classical Pelizaeus-Merzbacher disease

Ⅰ．緒言

　わが国における脱髄疾患の研究は，1958年白木^1），横井^2）らによる報告がなされており，その中でも表題のPelizaeus-Merzbacher病（以下P-M病と略す）は，報告例がきわめて稀なもので，本邦では1970年の藤沢^3）の成人男子例があるにすぎない。

　この疾患の剖検は，Merzbacher^4）が1910無こ最初の詳細な報告をしてから，外国例を含めて文献上で認定されているものは，約20例できわめて少ない。

　An 8 year 10 month old Japanese girl with classical Pelizaeus-Merzbacher disease was reported.

　The patient seemed to be normal until 10 months of age, when she began to have bilateral horizontal nystagmus. At about 1 year, spastic paresis of lower extremities appeared, and increased progressively. Mental deterioration was also very slowly progressive, and she was considereu to be an idiot. She died of asphyxiation due to vomiting.