急性に経過した進行性筋萎縮症の1剖検例—臨床的にはSPMAを思わせ，剖検において知覚障害を示した1例

A CASE OF RAPIDLY COURSED PROGRESSIVE MUSCUSAR ATROPHY

I．はじめに

　脊髄の系統的変性疾患のうち，上位および下位運動ニューロンの障害される疾患として，わが国では従来よりドイツ学派の分類により，脊髄性進行性筋萎縮症（SPMA，下位ニューロンの障害），痙性脊髄性麻痺（上位ニューロンの障害），筋萎縮性側索硬化症（ALS）をあげ，それぞれ独立疾患として扱つてきた。これは一見，臨床的にも病理的にも非常に理解しやすい分類であるが，現実には，ALSおよびSPMAには種々移行や重複があるため米英および仏学派はこれらを一括して運動ニューロン疾患（Motor neuron disease）と呼んでいる現状である。

　われわれは最近，この運動ニューロン疾患患者を剖検し，興味ある知見を得たので報告する。

There are various overlaps and transitions among motor neuron diseases. It is accepted that there are no marked changes in sensory tracts in This disease and that the course of the SPM is more chronic than that of ALS.

A man aged 52, showed a history of marked muscular weakness and atrophy of extremities associated with fibrillary twitchings and bul-bar symptoms. The condition was progressed gradually and he died of suffocation ten mo-nths after the onset of the disease.

Neurologic examination demonstrated bilat-eral muscular atrophy including small hand muscles with fibrillary twitchings and mark-edly reduced power of extremities and body. All tendon reflexes were markedly reduced. No pathological reflex or clonus was observed. The masseter reflex was normal. Sensetion and coordination were normal. There was difficulty of swallowing and speech.

Autopsy showed degenerative changes of anterior coloumn, various kinds of degeneration in muscles and peripheral motor nerves and, in part, regeneration of muscles and end-mus-cular junctions. No changes in the pyramidal tract were observed. These findings seemed to coincide with those described in spinal pro-gressive muscular atrophy. However, there were degenerations of dorsal-and ventral spi-no-cerebellar tracts and slightly reduced cells of the posterior column with degeneration of that in all segments.

In our case, the disease progressed rather rapidly and showed apparent changes of the posterior column in postmortem examination, while it showed typical findings of SPM cli-nically. It is unexplainable that, the change of anterior column was combined with the change of the posterior column.