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Memory Loss and Frontal Cognitive Dysfunction in a Patient with Adult-onset Neuronal Intranuclear Inclusion Disease.
Intern Med. 2016; 55(16):2281-4.IM

Abstract

Neuronal intranuclear inclusion disease (NIID) is an uncommon progressive neurodegenerative disorder. Adult-onset NIID can result in prominent dementia. We herein describe the case of a 74-year-old man who presented with dementia, cerebellar ataxia, neuropathy, and autonomic dysfunction. Diffusion-weighted imaging showed hyperintensity of the corticomedullary junction. Fluid-attenuated inversion recovery images showed frontal-dominant white matter hyperintensity. NIID was diagnosed from the presence of intranuclear inclusions in a skin biopsy sample. Neuropsychological testing revealed memory loss and frontal cognitive dysfunction, especially in relation to language and executive functions. We were therefore able to confirm the association of NIID with cognitive dysfunction.

Authors+Show Affiliations

Department of Neurology, Nagoya University Graduate School of Medicine, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

27523009

Citation

Araki, Kunihiko, et al. "Memory Loss and Frontal Cognitive Dysfunction in a Patient With Adult-onset Neuronal Intranuclear Inclusion Disease." Internal Medicine (Tokyo, Japan), vol. 55, no. 16, 2016, pp. 2281-4.
Araki K, Sone J, Fujioka Y, et al. Memory Loss and Frontal Cognitive Dysfunction in a Patient with Adult-onset Neuronal Intranuclear Inclusion Disease. Intern Med. 2016;55(16):2281-4.
Araki, K., Sone, J., Fujioka, Y., Masuda, M., Ohdake, R., Tanaka, Y., Nakamura, T., Watanabe, H., & Sobue, G. (2016). Memory Loss and Frontal Cognitive Dysfunction in a Patient with Adult-onset Neuronal Intranuclear Inclusion Disease. Internal Medicine (Tokyo, Japan), 55(16), 2281-4. https://doi.org/10.2169/internalmedicine.55.5544
Araki K, et al. Memory Loss and Frontal Cognitive Dysfunction in a Patient With Adult-onset Neuronal Intranuclear Inclusion Disease. Intern Med. 2016;55(16):2281-4. PubMed PMID: 27523009.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Memory Loss and Frontal Cognitive Dysfunction in a Patient with Adult-onset Neuronal Intranuclear Inclusion Disease. AU - Araki,Kunihiko, AU - Sone,Jun, AU - Fujioka,Yusuke, AU - Masuda,Michihito, AU - Ohdake,Reiko, AU - Tanaka,Yasuhiro, AU - Nakamura,Tomohiko, AU - Watanabe,Hirohisa, AU - Sobue,Gen, Y1 - 2016/08/15/ PY - 2016/8/16/entrez PY - 2016/8/16/pubmed PY - 2017/3/23/medline SP - 2281 EP - 4 JF - Internal medicine (Tokyo, Japan) JO - Intern Med VL - 55 IS - 16 N2 - Neuronal intranuclear inclusion disease (NIID) is an uncommon progressive neurodegenerative disorder. Adult-onset NIID can result in prominent dementia. We herein describe the case of a 74-year-old man who presented with dementia, cerebellar ataxia, neuropathy, and autonomic dysfunction. Diffusion-weighted imaging showed hyperintensity of the corticomedullary junction. Fluid-attenuated inversion recovery images showed frontal-dominant white matter hyperintensity. NIID was diagnosed from the presence of intranuclear inclusions in a skin biopsy sample. Neuropsychological testing revealed memory loss and frontal cognitive dysfunction, especially in relation to language and executive functions. We were therefore able to confirm the association of NIID with cognitive dysfunction. SN - 1349-7235 UR - https://www.unboundmedicine.com/medline/citation/27523009/Memory_Loss_and_Frontal_Cognitive_Dysfunction_in_a_Patient_with_Adult_onset_Neuronal_Intranuclear_Inclusion_Disease_ L2 - https://dx.doi.org/10.2169/internalmedicine.55.5544 DB - PRIME DP - Unbound Medicine ER -