神経核内封入体病（NIID）

Neuronal Intranuclear Inclusion Disease（NIID）

Point

・神経核内封入体病（NIID）は，認知症や筋力低下など多様な症状を呈する疾患で，2011年に皮膚生検でも診断可能とされたことにより，報告数が増加している．

・DWIではU-fiberに強い大脳白質高信号病変が遷延することが特徴である．

　Neuronal intranuclear inclusion disease（NIID）is a progressive neurodegenerative condition characterized by eosinophilic hyaline intranuclear inclusions in neuronal and other somatic cells. Since 2011, when skin biopsy was proven to be a diagnostic tool, the incidence of NIID has been increasing. Its symptoms include dementia, muscle weakness, and sensory or autonomic disturbance. MRI shows hyperintense lesions of the subcortical white matter, especially on the U-fiber. These findings persist and continue to worsen for years.