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Adult-onset neuronal intranuclear inclusion disease presenting with typical MRI changes.
Brain Behav. 2019 12; 9(12):e01477.BB

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.

Authors+Show Affiliations

Department of Neurology, Kaifeng Central Hospital, Kaifeng, China.Department of Neurology, Kaifeng Central Hospital, Kaifeng, China.Department of Neurology, Kaifeng Central Hospital, Kaifeng, China.Department of Neurology, Kaifeng Central Hospital, Kaifeng, China.Department of Neurology, Kaifeng Central Hospital, Kaifeng, China.Department of Neurology, Kaifeng Central Hospital, Kaifeng, China.Department of Neurology, Peking University People's Hospital, Beijing, China.Department of Neurology, Peking University First Hospital, Beijing, China.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

31749292

Citation

Han, Xinsheng, et al. "Adult-onset Neuronal Intranuclear Inclusion Disease Presenting With Typical MRI Changes." Brain and Behavior, vol. 9, no. 12, 2019, pp. e01477.
Han X, Han M, Liu N, et al. Adult-onset neuronal intranuclear inclusion disease presenting with typical MRI changes. Brain Behav. 2019;9(12):e01477.
Han, X., Han, M., Liu, N., Xu, J., Zhang, Y., Zhang, Y., Hong, D., & Zhang, W. (2019). Adult-onset neuronal intranuclear inclusion disease presenting with typical MRI changes. Brain and Behavior, 9(12), e01477. https://doi.org/10.1002/brb3.1477
Han X, et al. Adult-onset Neuronal Intranuclear Inclusion Disease Presenting With Typical MRI Changes. Brain Behav. 2019;9(12):e01477. PubMed PMID: 31749292.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Adult-onset neuronal intranuclear inclusion disease presenting with typical MRI changes. AU - Han,Xinsheng, AU - Han,Miao, AU - Liu,Ning, AU - Xu,Jianke, AU - Zhang,Yan, AU - Zhang,Yun, AU - Hong,Daojun, AU - Zhang,Wei, Y1 - 2019/11/20/ PY - 2019/03/15/received PY - 2019/09/11/revised PY - 2019/10/15/accepted PY - 2019/11/22/pubmed PY - 2020/6/20/medline PY - 2019/11/22/entrez KW - acute encephalopathy syndrome KW - magnetic resonance imaging KW - neuronal intranuclear inclusion disease SP - e01477 EP - e01477 JF - Brain and behavior JO - Brain Behav VL - 9 IS - 12 N2 - 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. SN - 2162-3279 UR - https://www.unboundmedicine.com/medline/citation/31749292/Adult_onset_neuronal_intranuclear_inclusion_disease_presenting_with_typical_MRI_changes_ L2 - https://doi.org/10.1002/brb3.1477 DB - PRIME DP - Unbound Medicine ER -