title: Memory Loss and Frontal Cognitive Dysfunction in a Patient with Adult-onset Neuronal Intranuclear Inclusion Disease.
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: Department of Neurology, Nagoya University Graduate School of Medicine, Japan.
authors: Kunihiko
authors: K
authors: Araki
authors: Jun
authors: J
authors: Sone
authors: Yusuke
authors: Y
authors: Fujioka
authors: Michihito
authors: M
authors: Masuda
authors: Reiko
authors: R
authors: Ohdake
authors: Yasuhiro
authors: Y
authors: Tanaka
authors: Tomohiko
authors: T
authors: Nakamura
authors: Hirohisa
authors: H
authors: Watanabe
authors: Gen
authors: G
authors: Sobue
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology; D060825:Cognitive Dysfunction / Q000209:etiology; D038524:Diffusion Magnetic Resonance Imaging; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D045586:Intranuclear Inclusion Bodies / Q000473:pathology; D008297:Male; D008569:Memory Disorders / Q000209:etiology; D019636:Neurodegenerative Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*