症候的に特異と思われるMotor Neuron Diseasesの4例

ATYPICAL CASES OF MOTOR NEURON DISEASES

I．はじめに

　われわれは最近，筋萎縮性側索硬化症の特殊型ともいうべき3例を経験し，また家族性発現の顕著な，進行性脊髄性筋萎縮症と考えられる特異な筋萎縮症例を経験したので，あわせて報告する。

We observed 4 male cases of motor neuron disease including 2 autopsied cases. They show-ed more or less interesting clinico-patholo-gical findings surnmerized as follows.

1) One patient complained of severe pain in shoulder joint during downhill course of ALS. In autopsy we could detect no finding explainable for pain.

2) One patient of ALS showed myasthenic symptom referable to oculmotor nerve paresis.

3) Two cases of bulbar palsy showed dege-nerative changes in olivery nuclei, which mean-ing is not clear.

4) Two cases of ALS showed muscular at-rophy begining in the shoulder girdle and the upper arm. One of them showed atrophy rema-ind in the right upper arm over 10 years.

5) One case whose younger two brothers died of SPMA showed the adult type of Wer-dnig-Hoffmann disease and transient block-age of subarachnoid space in myelography.