重篤な末梢神経障害での加療中に死亡した衝心脚気の若年例

Shoshin Beriberi in an Adult Patient following Severely-impaired Peripheral Neuropathy

要旨　患者は26歳，男性．貧困のため不規則な食生活を続けていた．2003年3月下旬より上気道炎症状を伴う四肢脱力が出現．近医にてギランバレー症候群の診断で経過観察されていたが，4月20日突然の心原性ショックを来し，当科に紹介された．高度左室機能低下と心室内伝導障害の急激な進行を認め，人工心肺補助を含む全身管理を行ったが，全身血管抵抗の著減により血圧保持が困難であり，入院5日目に死亡した．剖検では，心筋は変性像のみで冠動脈病変や心筋炎所見を認めなかった．一方，大脳乳頭体の出血と炎症非随伴性の高度末梢神経障害像が存在した．以上より，ウェルニッケ脳症および末梢神経障害を随伴する衝心脚気と診断された．現代においても，患者背景や臨床経過から脚気を鑑別診断として拠出することが必要である．

Summary

　A 26-year-old man, living in poverty on an unbalanced diet, had flu-like symptoms following weakness of his limbs at the end of March, 2003. His neurological dysfunction gradually improved under in-hospital management according to the initial diagnosis of Guillain-Barre syndrome. On April 21, he suddenly suffered cardiogenic shock. Cardiological examination showed severely-impaired left ventricular contractions together with rapid progress of intraventricular conduction disturbance. He died on April 25 due to sustained low blood pressure accompanied by decreased systemic vascular resistance despite artificial hemodynamic supports. The autopsy demonstrated myocardial degeneration without any stenotic coronary arteries and myocardial inflammation in addition to noninflammatory axonal degeneration in peripheral nerves together with petechiae in mammillary bodies. These clinical and pathological findings led to the final diagnosis of Shoshin beriberi and Wernicke encephalopathy with preceding severely-impaired peripheral neuropathy. It is important even nowadays to bear in mind the possibility of beriberi for the differential diagnosis of heart failure.