断血性病変を主とした肝脳疾患を疑わせる1例

生来健康な50才男子が,意識障害を思わす症状で発症し,原因不明の発熱があり,血中アンモニヤは軽度上昇を示した.脳機能賦活剤とともに少量のChloramphenicolの他にLincocin, Sulpyrine, Sulfonamideなどを投与後,これらの薬剤に起因すると思われる顆粒白血球減少症をきたした.輸血その他の治療で恢復したが,輸血終了後6日目に発疹につづいて黄疸が始まり,全経過70日で死亡した.剖検では島葉,前障,扁桃核,側頭葉の脳回谷部,アンモン角,小脳皮質,歯状核,下オリーブ核などに広汎,重篤な断血性変化があり,さらに星状グリヤの裸核化がびまん性にみられた.これらは循環障害と肝脳疾患断血型の境界に位置するものと考えられる.さらに真菌感染を強く疑わせる組織球性浸潤が合併していた.

A 50-year old male was suffering from disturbance of consciousness. Intermittent fever of unknown origin was noticed in the early stage of the disease and blood level of ammonia went up mildly in the middle stage. After the administration of stimulants of brain metabolism, a small amount of chloramphenical, lincomycin, sulpyrine, sulfonamide etc., agranulocytosis appeared. Treatment including blood transfusion soon brought recovery from agranulocytosis, but exanthem developed on the 6th day after the end of blood transfusion, followed by jaundice. He died on the 70th day after the onset of the disease.　Neuropathological examination revealed widespread ischemic changes bilaterally in the insular cortex, claustrum, amygdala, in the bottom of sulci of the temporal convolutions, ammon's horn, cerebellar cortex, dentate nucleus and inferior olive. Besides large amount of gemistocytic astrocytes, many enlarged astrocytic nuclei, belonging to Alzheimer's type II of glial cells, were observed diffusely throughout the central nervous system. The above clinico-pathological findings suggest that this case locates on the borderline between the "ischemic type" of hepatocerebral disease and circulatory disorders. Furthermore, perivascular cuffing of histiocytes was also observed, suggesting concomitant fungus infection.