大脳皮質にLewy小体の多発を認め，痴呆と無動・無言症を呈したParkinson病の一剖検例—痴呆の機序に関する考察

AN AUTOPSY CASE OF PARKINSON'S DISEASE ASSOCIATED CLINICALLY WITH DEMENTIA TERMINATING IN AKINETIC MUTISM AND PATHOLOGICALLY WITH MULTIPLE LEWY'S BODIES IN THE CEREBRAL CORTEX

　抄録　24歳で被害妄想的言動と易興奮性で発病し，37歳より進行性痴呆，44歳よりパーキンソン徴候を生じ，以後50歳で死亡するまで無動・無言症を呈した1男性を剖検し，その脳にはParkison病（P病）の所見に加えて，大脳皮質の策5・6層に明暈のないLewy小体（L体）の散在を認めた。L体は特に帯状回および周囲の前頭葉皮質に多く，そこには更に第3,5,6層に神経細胞の萎縮と著明なリポフスチン沈着，原発刺激像等の非特異的変性像を認め，その数はL体含有神経細胞をはるかに上回っていた。又，海馬回の少数の神経原線維変性以外に，進行性痴呆を説明しうる老人性変化は認めなかつた。以上の剖検所見と本例の臨床症状の進展様式並びに文献的考察から，本例の痴呆は両側帯状回と周囲の前頭葉皮質に分布するL体並びにそれに随伴した上述の非特異的神経細胞変性による可能性を示した。又，黒質と帯状回のL体を組織化学的並びに電顕的に比較した結果，両者の性状と電顕上の構成成分に差異はなく，黒質のL体の明暈部は細線維が放射状に配列しているのに対し皮質のそれにはその様な配列がない点が異なつていた。以上の所見から，大脳皮質にL体の多発するP病をP病とは別個の独立疾患単位とみなすのは慎重を要することを述べた。

An autopsy case of a 50 year-old male with Par-kinson's disease associated with multiple Lewy bodies in the cerebral cortex was reported. His clinical symptoms began at the age of 26 with the speech and actions indicative of a persecution complex accompanied by irritability and were followed by progressive dementia from the age of 37 and Perkinsonism since the age of 41. He was in a state of akinetic mutism thereafter till his death at the age of 50.

Autopsy disclosed in addition to the typical findings of Parkinson's disease in the brain stem multiple intracytoplasmic Lewy bodies in medium-sized neurons of the fifth and sixth layers of the cerebral cortex. They were atypical in the sense that they did not have any haloes. They were especially numerous in the cingulate gyrus. In addition, findings of non-specific neuronal dege-neration were obtained in the cerebral cortex such as cellular atrophy with massive deposition of lipofuscin pigments, central chromatolysis, cell loss and cellular gliosis in the third, fifth and sixth layers. These neuronal findings were also pro-minent in the cingulate gyrus. Such senile changes as senile plaques or granulo-vacuolar degeneration were not found, although there were a few foci of neurofibrillary degeneration in the hippocampal gyrus. Histochemically and electron microscopically, no difference was observed in the constituents of Lewy bodies between the brain stem and the cerebral cortex.

Such autopsy findings and a review of the literature indicate that the dementia in this case may be related not only to the presence of Lewy bodies but also to the above-described, non-specific neuronal degeneration in the bilateral cingulate gyri and surrounding frontal gyri. The standpoint of regarding a Parkinson's disease with multiple Lewy bodies in the cerebral cortex as an inde-pendent disease entity was criticized.