Toshio Terao

ENCEPHALOMYELOPATHY DUE TO AN ORGANIC MERCURY COMPOUND.

OUR INTEREST in the toxicity of organic metal compounds was stimulated by reports of organic tin intoxication in 19551 and the “Minamata disease,” presumably caused by organic mercury compound,2 which was reported in 1957.3 An excellent review on the clinical, histopathologic, and epidemiologic aspects of this disease has been published.4 A few reports concerning the intoxication of man by organomercury compounds indicate the cause to be the inspiration of the fumes of agricultural drugs in most cases. In 1940, Hunter and associates5 reported 4 cases of intoxication by methylmercury phosphate or nitrate. Fourteen years later, Hunter and Russell6 described detailed histopathologic findings in the brain of one of the patients in the previously reported cases. The microscopic changes in the brain were most pronounced in the occipital pole region of the cerebrum and palleocerebellum. Constriction of the visual fields may be attributed to atrophy of the area striata and, according to their statement, the ataxia could be correlated with changes in the cerebellar cortex. Brown7 reported clinical and autopsy findings in a case of phenylmercury acetate intoxication which showed a clinical picture resembling amyotrophic lateral sclerosis. Microscopic findings showed degeneration of motor cells in the anterior horn and the lateral column of the spinal cord, without changes in the brain. Kantarjians also pointed out the clinical resemblance to amyotrophic lateral sclerosis in 11 cases of definite ethylmercury ptoluene sulfonanilide intoxication. This paper is a report of 3 cases of intoxication caused by the application of a solution containing organic mercury compound for the treatment of a widespread fungus skin infection.

Studies on hepatocerebral disease: III. Hepatolenticular degeneration in Japan, with studies on copper metabolism

Age in Years Fig. 1. Age and sex of patients in Japan with Wilson’s disease in accord with American and European studies, that a disturbance of copper metabolism also plays a major role in the pathogenesis of Wilson’s disease in Japan. Similar but rare cases with clinicopathologic findings suggestive of Wilson’s disease but without an abnormality of copper metabolism have also been seen. Wilson’s disease used to be considered a very rare disease in Japan. However, much interest was aroused in this disease recently, and 62 cases have been reported in the past ten years. The inference is well grounded that many more unreported cases must be present in this countrv. The distribution of the patients showed no pnrticular grouping, and the cases were found in almost all sections of Japan. The heredity of 43 families was investigated in regard to this condition. Familial occurrence of the disease or blood relationship between father and mother of the patient was demonstrated in 15 families, or 35%. In the latter cases, most marriages were between cousins.

MYOTONIC DYSTROPHY ASSOCIATED WITH THYMOMA -REPORT OF AN AUTOPSY CASE-

筋強直性ジストロフィーに胸腺腫を合併した極めてまれな剖検例を経験したので報告する.患者は46才の女性で,家族歴では姉と妹に筋強直性ジストロフィーを認める.既往歴では流産,死産があり,現病歴では小児期からgrasping myotoniaがあり, 37才の時myotonic dystrophyと診断された.昭和49年9月呼吸困難を主訴として入院した.患者は, hatchet faceで,甲状腺腫があり,四肢は著明な筋萎縮を呈していた.左肺野で呼吸音を聴取できず,脾臓は腫大していた.胸部レントゲン検査で前縦隔腫瘍と左側胸水貯留が明らかにされ,著明な呼吸機能障害が認められた.免疫学的にabsolute lymphocytopenia,血清Ig-G, Ig-A, Ig-Dに低値の傾向, DNCB感作試験陰性などpan-immunosuppressionの所見を呈した.また糖尿病をはじめとする内分泌機能の異常も認められた.全身麻酔のもとで縦隔鏡検査が施行されたが,その際に原因不明の心停止を起こして死亡した.剖検により上皮型細胞成分優位の混合型胸腺腫があることが判明し,甲状腺右葉にはfollicular adenomaを認めた.筋強直性ジストロフィーに胸腺腫を合併した剖検例としては,後藤らの報告1)に次ぐ第2番目の記載となる.

SEPARATION OF AMINO ACIDS BY GAS CHROMATOGRAPHY I

ガスクロマトグラフィーによる血中アミノ酸の微量分析法を確立すべく,その基礎的検討を行なつた. N-トリフロロアセチルメチルエステル誘導体について行なつたが,この誘導体はアミノ酸のみの混合物の分析には適しているが,それ以外のものが混在する場合には厳密にアミノ酸のみを分離しておく必要があるので,血中アミノ酸の測定には適当でない. DNP誘導体のメチルエステルをSE30およびQF-1の充填薬を用い分析条件を検討したところ, alanine, glycine, valine, leucine, proline, aspartic acid, glutamic acid, methionineおよびphenylalanineの9種のアミノ酸についてはシャープな良く分離したピークが得られた.この誘導体は血中から遊離アミノ酸を分離するのにも適当である.ガスクロマトグラフィーによれば少量の試料(血液約1cc)で短時間に分析できるので,微量定量法として充分用いうることが明らかになつた.

PYRUVATE KINASE ACTIVITY IN SERUM OF PATIENTS WITH HEPATIC DISEASE

Pyruvate kinase (PK)は解糖系に関与する酵素であり,人体の解糖を行なつている部位にあまねく分布しているものと考えられている.赤血球中におけるPKは種々の溶血性疾患について報告されているが,血清中のPKについての報告は殆どみられない.著者は正常者および諸種肝疾患患者血清中の本酵素活性値を測定し,これと平行して諸種肝機能検査,諸酵素活性値を測定し,その変動ならびに相関について観察した。PK活性値は急性肝炎発黄期にトランスアミナーゼ活性値の上昇よりはやゝ遅れて上昇し,回復期に再び一時的に上昇する例が多かつた.肝硬変のPK値は肝炎より高い値を示したものもあるが,他の肝機能との相関はみられなかつた.閉塞性黄疸では閉塞の進行するにつれてPK値の上昇がみられ,黄疸指数・アルカリフォスファターゼ(al-P-ase)とある程度の相関が認められたが,長期間の閉塞を示した症例では,かえってPK値が低下し,黄疸指数・al-P-aseと逆相関を示したものも認められた.