Brain and behavior · 2019
Adult-onset neuronal intranuclear inclusion disease presenting with typical MRI changes.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Han Xinsheng, Han Miao, Liu Ning, Xu Jianke, Zhang Yan, Zhang Yun, Hong Daojun, Zhang Wei
Аннотация
BACKGROUND: This study aims to analyze the clinical, imaging, electrophysiological, and dermatopathological features of a patient with adult-onset neuronal intranuclear inclusion disease (NIID) and to explore the diagnostic methods of adult-onset NIID.
CASE PRESENTATION: We here report a 63-year-old male with recurrent acute encephalopathy syndrome and autonomic nervous system damage syndrome characterized by sexual dysfunction and urinary and fecal dysfunction. Cranial diffusion-weighted magnetic resonance imaging (DWI) demonstrated symmetrically distributed strip-shaped high-intensity signal in bilateral fronto-occipital-parietal cortical-medullary junction. Electrophysiological test revealed that the main site of injury was myelin sheath in both motor and sensory nerves. Skin biopsy revealed eosinophilic spherical inclusion bodies in the nucleus of sweat gland epithelial cells.
CONCLUSION: This case suggests that adult NIID is a chronic neurodegenerative disease with high clinical heterogeneity. Subcortical strip-shaped high-intensity signal on DWI has high diagnostic significance. Eosinophilic intranuclear inclusion bodies detected by skin biopsy contribute to diagnosis.
CASE PRESENTATION: We here report a 63-year-old male with recurrent acute encephalopathy syndrome and autonomic nervous system damage syndrome characterized by sexual dysfunction and urinary and fecal dysfunction. Cranial diffusion-weighted magnetic resonance imaging (DWI) demonstrated symmetrically distributed strip-shaped high-intensity signal in bilateral fronto-occipital-parietal cortical-medullary junction. Electrophysiological test revealed that the main site of injury was myelin sheath in both motor and sensory nerves. Skin biopsy revealed eosinophilic spherical inclusion bodies in the nucleus of sweat gland epithelial cells.
CONCLUSION: This case suggests that adult NIID is a chronic neurodegenerative disease with high clinical heterogeneity. Subcortical strip-shaped high-intensity signal on DWI has high diagnostic significance. Eosinophilic intranuclear inclusion bodies detected by skin biopsy contribute to diagnosis.