亜急性脳症を呈した神経核内封入体病の2例

Two cases of neuronal intranuclear inclusion disease with subacute encephalopathy

神経核内封入体病（neuronal intranuclear inclusion disease：NIID）は多臓器に好酸球性のヒアルロン酸核内封入体を生じる緩徐進行性の神経変性疾患である。神経症状は認知機能低下，筋力低下，縮瞳，失調，末梢神経障害など多岐にわたるが，発熱，頭痛，嘔吐，意識障害などの亜急性脳症を発症することがある。今回，亜急性脳症を呈した2例を報告する。1例は経時的に皮質から皮質下白質，皮髄境界にMR所見が変化し，もう1例は造影MRIにて，脳回と硬膜に増強効果を示した。

We reported two cases of neuronal intranuclear inclusion disease（NIID）with subacute encephalopathy. NIID is a slowly progressive neurodegenerative disease and may sometimes present with symptoms of subacute encephalopathy, including fever, headache, vomiting, and loss of consciousness. NIID should be considered in the presence of encephalopathy with cortical swelling, contrast enhancement in the unilateral temporooccipital lobes in adult patients. The characteristic hyperintense lesions in the corticomedullary junctions and paravermal sign are useful to the diagnosis of NIID, but occasionally they are not present.