Journal
NEUROLOGICAL SCIENCES
Volume 41, Issue 5, Pages 1019-1025Publisher
SPRINGER-VERLAG ITALIA SRL
DOI: 10.1007/s10072-019-04195-6
Keywords
Neuronal intranuclear inclusion disease; Biopsy; Diffusion-weighted-imaging; Dementia; Ubiquitin
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Objective To summarize the current understanding of neuronal intranuclear inclusion disease (NIID) and improve the understanding of the physician about this condition. Methods We searched PubMed with keywords related to NIID and selected publications which seemed appropriate. We analyzed its clinical features, pathogenesis, evaluation methods, treatment options, and research prospectives. Results NIID is a degenerative condition which can affect multiple organ systems especially central nervous system. Its clinical features greatly vary, and making the exact diagnosis is often difficult. There are several genes which have been associated with this disorder. Some specific signs on diffusion-weighted-imaging (DWI) sequence of magnetic resonance (MR) imaging are characteristics to NIID. Conclusion Intranuclear inclusions have been found in various nonneural cells of the body; therefore, the term systemic intranuclear inclusion disease is, perhaps, better suited to explain this disorder. There are several disorders which need to be ruled out before making the diagnosis, and neuroimaging and biopsy analysis should be combined to support the diagnosis.
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