筋萎縮を伴つた高度痴呆の1剖検例

AN AUTOPSY CASE OF PRESENILE DEMENTIA WITH MOTOR NEURON DISEASE

はじめに

　我々は本例を「筋萎縮を伴つた高度痴呆の一症例」として，その臨床を既に報告した1）。その際神経学的症状からは，筋萎縮性側索硬化症（以下ALSと記す）を，精神症状からはPick病・Alzheimer病・Gruetzfeldt—Jakob病（以下C-J．病と記す）等の初老期脳器質疾患を考察の対象にしたが，特定診断を下すことはさけた。その後組織学的検索の機会を得たので，今回は組織学的所見を中心にして本例を考察したい。

Clinical History :

A 60-year-old house wife at the age of 58 years developed an illness consisting of retention defect, preserved language and personality defect. Later muscular atrophy in the arms, thenar and hypothenar was observed. Neurologically deep tendon reflexes of the limbs were normal with no pathological re-flex. Pneumoencephalography revealed a diffuse cerebral atrophy and EMG showed lower motor neuron damage. The illness was aggravated pro-gressively and she died about two years after the onset of the disease.

Gross Anatomical Findings :

1. Atrophies of intra-abdominal organs.

2. Chronic emphysema (senile).

3. Cholelithiasis with chronic cholecystitis.

4. Petechial hemorrhage (stomach, kidney pelvis, and urinary bladder).

Neuropathological Findings :

Brain : weight 1,070 g, diffuse cerebral atrophy which was maked in the frontal lobes.

Microscopic Anatomical Findings :

The cerebral cortex in all areas showed a moderate degree of nerve cell degeneration and some loss of neurons with an astroglial reaction. A mild spon-giose state was present between the second and third layers in the frontal, temporal and central cortices. There was a mild rarefaction of the myelin sheath in the deep white substance of the cerebral hemispheres and in the lateral tract of the spinal cord, but gliosis and fatty granular cells were not observed. In the spinal cord, the loss of anterior horn cells was marked in the cervical cord and mild in the lumbar cord. There were many torpedos in the cerebellum. It is considered that this is an un-classified presenile dementia case assumed to be one of the systemic degenerative diseases of CNS. This group is characterized by an onset of presenility with mental changes, an incomplete type of ALS and by advancement to death within a few years. Pathologically, nonspecific mild degenerative changes are observed throughout the central nervous system.