特異な精神神経学的経過を示した栄養障害の1剖検例

An Autopsy Case of Nutritional Disorder with Unusual Neuropsychiatric Course

Ⅰ．緒言

　生体は常に栄養を摂取し，体内で新陳代謝を営んで生命を維持している。もしその摂取が不十分であったり，個体の身体的原因によって栄養の吸収利用が妨げられることがあると新陳代謝障害によるいろいろな症状が発現する。たとえば，慢性アルコール中毒者は食餌摂取の不十分，胃腸障害による栄養不足ことにビタミン欠乏を来しやすく，Wernicke脳症，Korsakoff精神病，ペラグラ，索性脊髄疾患などの中枢神経疾患を生ずることがある。これらの疾患は胃切除後にも生ずることがある。

　このたび，われわれはアルコール嗜癖，胃全摘既往の55歳男性で性格変化，痴呆，錐体路症状，せん妄，ミオクローヌス，上肢屈曲下肢伸展位，さらに下痢・発熱を進行性に順次生じて全経過4カ月で死亡した症例の臨床を観察し，さらに脳の病理学的検索の機会を得た。しかし病理学的にはBetz細胞，橋核神経細胞のcentral chromatolysis，大脳・小脳の白質のgliosisを軽度に認めるのみで重篤な臨床経過の割には脳の神経細胞変化は軽微であった。そこでこの症例の臨床経過と脳の組織像を報告するとともに，若干の考察を加える。

　We reported one autopsy case in which multiple nutriment deficiency attributable to chronic alcoholism and total gastrectomy appears to have caused nutritional disorder centering around pellagra.

　The case was a 55-year old who established and was running a relatively large musical instrument shop. At age 51 he had cancer of the stomach and underwent total gastrectomy and splenectomy. He had been drinking 500ml of sake a day since 20 years of age. From the beginning of April, 1975, he lost vigor and started nasal dysarthria. On April 22, he had fracture of left elbow and was admitted to a hospital. With this as a turning point, he showed disorientation, retardation of intellectual activities, apathy, lethargy and irritability, and on May 26 was admitted to our Department. On admission, the patient had an apathic look and was in the cachetic state; mandibular reflex and tendon reflex of the limbs showed spastic accentuation, while pathologic reflex of the upper and lower limbs was also positive. From the end of June, he was no longer able to walk and with urinary and fecal incontinence, became bed-ridden. From the middle of July, he became delirious and began to have bedsore and diarrhea. From the end of July, there appeared fever at the level of 38℃, myoclonus of the upper limb bilaterally, bending of the upper limb and extension of the lower limb. Myoclonus gradually came to stay all over the body. From August 3, however, it became mild in the limbs and that on the face was found only on the left side. On August 8, 1975, he died of emaciation. The total clinical course was about four months.

　Examinations of the blood showed no evidence of abnormalities other than macrocytic normochromic anemia, decrease in serum iron, hypoproteinemia, hyponatremia and hypocalcemia. Electroencephalograms showed irregular slow waves occasionally with 8~9Hz activity as the background rhythm on June 22, fast wave of low amplitude on July 31 and fast wave of low amplitude and on rare occasion a series of 4~5Hz slow waves and/or spike and wave complex in the right hemisphere on August 8.

　General pathological findings were normal. Neuropathologically, the brain weighted 1450g, being not remarkable in appearance and cut sur-face except slightly cloudy in the central and parietal meninx. Stained specimens were normal except that diffuse gliosis and rarefaction of myelin, slight as it was, were observed in the white substance of the cerebrum and cerebellum and that Betz cells of praecentral convolution and nerve cells of nucleus pontis showed central chromatolysis.

　Making a review mainly on clinico-pathology of pellagra, we considered the multifaceted clinical picture of this case to be the results of metabolic disturbance of the nervous system due to multiple nutriment deficiency.