Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changes
Abstract 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‐y...
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doaj-703257149c884f6991e0087573f296772020-11-25T03:21:40ZengWileyBrain and Behavior2162-32792019-12-01912n/an/a10.1002/brb3.1477Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changesXinsheng Han0Miao Han1Ning Liu2Jianke Xu3Yan Zhang4Yun Zhang5Daojun Hong6Wei Zhang7Department of Neurology Kaifeng Central Hospital Kaifeng ChinaDepartment of Neurology Kaifeng Central Hospital Kaifeng ChinaDepartment of Neurology Kaifeng Central Hospital Kaifeng ChinaDepartment of Neurology Kaifeng Central Hospital Kaifeng ChinaDepartment of Neurology Kaifeng Central Hospital Kaifeng ChinaDepartment of Neurology Kaifeng Central Hospital Kaifeng ChinaDepartment of Neurology Peking University People's Hospital Beijing ChinaDepartment of Neurology Peking University First Hospital Beijing ChinaAbstract 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.https://doi.org/10.1002/brb3.1477acute encephalopathy syndromemagnetic resonance imagingneuronal intranuclear inclusion disease |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Xinsheng Han Miao Han Ning Liu Jianke Xu Yan Zhang Yun Zhang Daojun Hong Wei Zhang |
spellingShingle |
Xinsheng Han Miao Han Ning Liu Jianke Xu Yan Zhang Yun Zhang Daojun Hong Wei Zhang Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changes Brain and Behavior acute encephalopathy syndrome magnetic resonance imaging neuronal intranuclear inclusion disease |
author_facet |
Xinsheng Han Miao Han Ning Liu Jianke Xu Yan Zhang Yun Zhang Daojun Hong Wei Zhang |
author_sort |
Xinsheng Han |
title |
Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changes |
title_short |
Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changes |
title_full |
Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changes |
title_fullStr |
Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changes |
title_full_unstemmed |
Adult‐onset neuronal intranuclear inclusion disease presenting with typical MRI changes |
title_sort |
adult‐onset neuronal intranuclear inclusion disease presenting with typical mri changes |
publisher |
Wiley |
series |
Brain and Behavior |
issn |
2162-3279 |
publishDate |
2019-12-01 |
description |
Abstract 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. |
topic |
acute encephalopathy syndrome magnetic resonance imaging neuronal intranuclear inclusion disease |
url |
https://doi.org/10.1002/brb3.1477 |
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