痴呆・滞続症・Klüver-Bucy症候群を呈した両側側頭葉梗塞の一剖検例

BILATERAL TEMPORAL LOBE INFARCTS CAUSING DEMENTIA, STEHENDE REDENSARTEN AND KLÜVER-BUCY SYNDROME

はじめに

　痴呆をきたす疾患には，多発梗塞性痴呆，老年痴呆（Alzheimer病），Pick病，Creutzfeldt-Jacob病など種々のものがあり，それぞれに特徴的臨床症状が知られている。たとえば多発梗塞性痴呆（Multi-infarct dementia，MIDと略）ではまだら痴呆で末期まで病識があり，人格が保たれ，卒中発作を伴い，種々の神経症状，巣症状，情動失禁がみられ，動揺性の経過をとるといわれている^2,4,9,20）。私共は繰返す卒中発作のほかは通常のMIDにみる臨床像とは全く異なり，初期には滞続症が目立つ特異な痴呆と末期にはKlüver-Bucy症候群を呈した1例を剖検し，両側側頭葉に軟化巣を認めた。MIDで滞続症やKlüver-Bucy症候群をきたすことは非常に稀であるので，症例を報告し若干の考察をしたい。

A 65 year old woman became restless and de-mented on one day and was brought to our hos-pital. She had a history of stroke with slight right hemiparesis at age 55 and had been treated for hypertension since then. On admission there were no neurological abnormalities in her cranial nerves, motor, sensory and cerebellar system nor in deep tendon reflexes. She could give her name, date and place of birth, her husband's name and present season correctly. However, she did not know her age nor remember the last war and the places where atomic bombs were dropped. She could not perform simple mathematics nor could she tell the meanings of cousin, tax, law and the difference be-tween ice and water. Most significant symptom was a stereotyped repetition of words and brief sentences (stehende Redensarten). She continuously repeated same few sentences all day long. Also seen was bulimia and hypermetamorphosis, paying attention to every person in sight and saying the same things. She became exhibitionistic and spent in nude for several months.

At age 67, she again had a stroke with right hemiplegia and speech disturbance. At age 68, she started to eat indiscriminately everything at hand, such as dust, clothes and quite of ten feces of her own and other patient's. She expired of broncho-pneumonia, 4 years after the onset.

At autopsy the brain weighed 1,120 grams. The arteries at the base of the brain showed severe arteriosclerosis. The left posterior temporal artery originating from the middle cerebral artery was completely occluded by atheromatous plaque. The right posterior temporal artery also showed pin-point lumen. There were multiple infarcts in both temporal lobes and left basal ganglia, some being cystic and others incomplete softening. The lesions involved primarily the white matter, how-ever portions of the overlying cerebral cortex also exhibited changes of infarct. There were no his-tological changes of Alzheimer's disease or Pick's disease. The hippocampal gyri, the fornix and the mamillary bodies were intact.

Discussions were done in regard to the vascular lesions of the temporal lobes and the occurrence of stehende Redensarten and Klüver-Bucy syndrome.