急性経過をとつた脊髄性進行性筋萎縮症の1剖検例

A CASE OF ACUTELY PROGRESSED SPINAL PROGRESSIVE MUSCULAR ATROPHY

I．はじめに

　1849年Duchenne,1850年Aranにより記載された進行性筋萎縮症は，その後種々の研究，報告が行なわれ1860年Luysにより病理学的に脊髄前角細胞の萎縮が証明され，脊髄性進行性筋萎縮症と呼ばれるようになつた。

　その後進行性筋ジストロフィー症，筋萎縮性側索硬化症が本疾患から分離されるようになつた。

Patient was 51 year old Japanese female with chief complainment of weakness of ext-remities, In November of 1959, she developed cyanosis and pain of fingers and in January of 1960, began to complain weakness of ext-remities and disturbance in gait.

Those complaint gradually became worse and in begin of march, she developed dys-phagia and was admitted in our clinic on 16 th of March. Physical examination on admittion revealed as followed.

Urinalysis, hematological study and exa-mination of cerebrospinal fluid were not re-markable.

Patellar reflex was increased in both sides and Babinski's sign was positive in left side.

In electromyogram, giant spike was observ-ed in many muscles of extremities and react-ion of degeneration due to electrical stimulus was observed as incomplete reaction in many extremity muscles.

Serological test for syphilis done in serum and cerebro-spinal fluid were negative.

At the end of March, patellar reflex and Babinski's sign disappeared. Symptoms of bul-bar paralysis were gradually advanced.

In middle of April, she was complicated by acute pneumonia and died on 16th of April.

〔Pathological findings〕

a) Gross anatomic finding.

Extensive atrophy of all skeletal mus-cles. Spinal cord appeared to be slightly thin.

b) Microscopic finding.

Atrophy, degeneration and decreased num-ber of nerve cells of nucleus hypoglossis and cornus anterior.

Acutely progressed disease process and re-markable changes of reflexes during course of disease suggested many diseases such as spinal tumor, amyotrophic lateral sclerosis, subacute or chronic polymyositis, carcinoma-tous myopathy.

However, the diagnosis of SPM was made because of clinical findings and signs of later disease process and it was confirmed by au-topsy.