凍結乾燥硬膜が原因と考えられたCreutzfeldt-Jakob Diseaseの1例

Creutzfeldt-Jakob Disease Transmitted by Cadaveric Dural Graft: A case report

I．はじめに

　Creutzfeldt-Jakob disease（以下CJD）は非定型ウイルスによる遅発性感染症で発症後1年以内に死亡ないし重篤な状態に陥る疾患である．乾燥凍結硬膜を介しての感染と考えられうるCJD列は1988年にThadaniが初めて報告して以来これまで4例が報告されている^6,7,9,11）．われわれも最近同様の1例を経験したので報告する．

We report a case of Creutzfeldt-Jakob disease in a 38-year-old man, transmitted by a cadaveric chiral graft.

In August 1985, he underwent cranial nerve decompres-sion for hemifacial spasm and received a cadaveric clural graft for chiral closure. He had been well until he began to complain of blurred vision and headache in May, 1990. He developed dementia, myoclonus and urinary inconti-nence over the subsequent 3 months. He was admitted to our hospital in August, 1990.

On admission, he was somnolent and showed gait dis-turbance, myoclonus in extremities and elevated deep ten-don reflexes symmetrically. The results of analysis of blood, urinary and cerebrospinal fluid were normal. The initial computed tomography (CT) and magnetic reso-nance imaging detected no abnormality. Electroencepha-lography showed typical periodic synchronous discharge (PSD). There was progressive worsening of his neurolo-gical symptoms, and this developed into mutism in September, 1990. CT, 11 months after clinical onset, showed marked enlargement of the ventricles and the sulci.

In view of his rapid worsening clinical course, PSD findings on electroencephalography, and delayed pro-gressive changes of CT findings, the diagnosis of CJD dis-ease was made. The cadaveric clural graft was suspected as the cause of the patient's condition. Since Thadani et al reported the first case of CJD transmitted by cadaveric clural graft in 1988, 3 other cases have been reported.

This is most likely the 5th reported case of Creutz-feldt-Jakob disease transmitted by cadaveric clural graft.