神経系統疾患の臨床病理学的研究—2．一種の脳炎を合併した痙性脊髄麻痺症例

CLINICO-PATHOLOGIC STUDIES ON DISEASES OF THE NERVOUS SYSTEM : 2. ACASE REPORT ON SPASTIC SPINAL PARALYSIS ASSOCIATED WITH A SORT OF ENCEPHALITIS

はじめに

　痙性脊髄麻痺（以下S．S．と略す）とは，脳或は脊髄のある局所の病変により，ある高さで錐体路の遮断が起るものを含まず，錐体路第一次ノイロンの系統的変性を来す比較的稀な疾患で，ほとんどが家族性に発現する。その病変は，理論上，錐体路起始部即ち中心前回に始まるか，脊髄内の錐体路からか，あるいは又，錐体路第一次ノイロンの種々の部位から始まるかの，いずれかであろうが，Schafferらは，中心前回に始まる，との見解をとつている（Peters^1）による）。

　この際，中心前回は，肉眼的に萎縮が著しく，組織学的には，第3・第5層の神経細胞脱落及びそこに残存する神経細胞の各種退行病変，第3層神経細胞のアルツハイマー原線維変化，皮質内の脱髄などが記載せられている^1）2）。

1. A man without familial heredity of diseases of the nervous system developed disturbances of speech and spastic paresis of the right leg when he was 36 years of age. Spastic paresis of the upper and lower extremities got worse from the right leg to the left and then from the right arm to the left. At the time of 40 years the upper and lower extremities revealed complete and par-alysis and the entire body showed marked rigidity like a board. On the other hand, disability of speech, atrophy of tongue, and difficulty of swallowing due to pseudobulbar paralysis became striking. The patient did not respond to the treatment for syphilis and these signs were gradually aggravated. After a clinical course of five years and a half, the patient (41 years and 4 moths old) became very weak and died.

2. On autopsy, symmetric demyelination of the pyramidal tract was seen mainly in the first neuron at the level of the midbrain to the lumbar spinal cord. In the areas of demyelination there was gliosis also present,and fat was poorly deposited in these areas. The second neuron, nerve cells such as nuc-leus hypoglossus and anterior column, were of no remarkable changes.