老年斑様小体，原線維変化，動脈病変，ALS様変化などを呈する脳症の1例

A Case of Encephalopathy with Plaque-like Bodies, Neurofibrillary change, Angiopathy and Amyotrophic Lateral sclerosis-like Lesions

　一卵双性双生児に発生した，臨床的に重篤な神経諸症状と進行性痴呆を示した疾患の，妹例の臨床，病理所見について報告した。28才で発病，初発症状は構音障害と左下肢の鈍痛を伴う運動障害で，ついで運動障害は四肢，脳神経文領配域および．に小脳・錐体外路症状も加わり，同時に人格変化，痴呆も進行し，36才で死亡した。身体臓器では骨格筋萎縮が特記すべき所見で，脳重1,185g，肉眼的に脳室系の拡張と，大脳脚，橋底の萎縮を認めた。顕微鏡的には，中枢神経系灰白質に広範な老年斑様小体と原線維変化を認め，後頭葉穹薩面を中心に，アミロイド変性を主体とした動脈病変を見，さらに，ALSおよび脊髄小脳変性症に類似の系統変性とみなすべき所見があつた。本例を，脳1血管アミロイドーゼを伴う非定型Alzheimer病，Parkinsonism-dementia complex on Guam，Kuruなどと比較し，脳の老化とアミロイドーゼの関係，本例の老年斑様小体のhistogenesisなどについて考察を行なつた。

The clinical and post-mortem finidings of a woman aged 36 at death, the one of the identical twins, another of which is alive and suffering from the illness similar to the proband, were reported. The onset of the illness was at the age of 28, when her speach gradually became slurred, and her gait became disturbed. The motor disturbances were propagated to the extremities and to the regions innervated by cranial nerves. Then, her personality changed and dementia progressed. At autopsy the atrophy of skeletal muscles was noted. The brain weighed 1, 185 grammes. On external survey bilateral frontal lobes and basis pontis were atrophic. On coronal sections the ventricular system was moderately dilated. Substantia nigra was depigmented. Microscopically, four kinds of conspicuous changeswere observed. 1) Senile plaques-like bodies were widely and numerously distributed in the cerebral and cerebellar corteces, basal ganglia, the grey matter of the midbrain, and only a few in grey of brainstem and spinal cord. Data in some staining methods indicated the body as to be "primitive plaque". 2) Alzheimer's neurofibrillary change was widespread in the cerebral cortex, substantia innominata, substantia nigra, locus caeruleus, Edinger-Westphal's nucleus, etc. 3) Arterial changes, complicated in nature, but mostly related to amyloidosis, were seen on the surface of the convex of the occipital lobes, and less severely in other parts of the cerebrum. 4) Some systemic degenerations consisting with demyelination and or gliosis of nerve tracts, distributions of which were similar to ALS and spinocerebellar degeneration were noted. In discussion the present case was considered to be akin to atypical case of Alzheimer's disease with amyloid degeneration of cerebral arterioles, Parkinsonism-dementia complex on Guam as well as Kuru disease.