脊髄性進行性筋萎縮症（Lumbosacraler Unterschenkel-Oberschenkeltyp）の1例

A CASE OF SPINAL PROGRESSIVE MUSCULAR ATROPHY (PROXIMAL TYPE)

I．まえがき

　脊髄性進行性筋萎縮症（以下SPMと略す）を筋萎縮性側索硬化症（以下ALSと略す）の1亜型となすか，あるいは他の独立疾患とみるかについては学者の見解が一致していない。周知のごとくドイツ系は後者の立場をとり，英米系は前者である。わが国においてもある者は前者の立場をとり，ある者は後者をもつて正しいとする。われわれは経験例も少なく，いずれの学説に軍配をあげる資格もないが，最近表題のごとく診断すべき1例を診たのでここに報告する。

A 75 year-old man, who showed very sev-ere generalized muscle atrophy, except face muscles. The muscle atrophy had begun in lower extremities and elevated higher and higher with spontaneous muscular fasciculat-ions. He was not able to walk and had diffi-culty in lifting his upper exremities.

The course of this disease was very rapid, and patient died of bulbar palsy after 7 months clinical course. Sensitivity was normal, and pyramidal signs were not found. Electromyo-gram and biopsy specimens showed changes typical of lower moter neuron damage.

Pathological examination revealed, that there was a degeneration of anterior horn cells with a marked decrease in their numbers and dem-yelination of anterior roots. The giant pyra-midal cells of Betz and pyramidal pathways were, however intact. The diagnosis of atypical spinal progressive muscular atrophy was made.