Sato Aki
Department of Neurology, Niigata City General Hospital.
No Shinkei Geka. 2021 Mar;49(2):419-424. doi: 10.11477/mf.1436204408.
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.
神经元核内包涵体病(NIID)是一种进行性神经退行性疾病,其特征是神经元和其他体细胞中存在嗜酸性透明核内包涵体。自2011年皮肤活检被证明是一种诊断工具以来,NIID的发病率一直在上升。其症状包括痴呆、肌肉无力以及感觉或自主神经功能障碍。MRI显示皮质下白质高信号病变,尤其是在U纤维上。这些表现持续存在并在数年内不断恶化。