Memory Loss and Frontal Cognitive Dysfunction in a Patient with Adult-onset Neuronal Intranuclear Inclusion Disease

Kunihiko Araki; Jun Sone; Yusuke Fujioka; Michihito Masuda; Reiko Ohdake; Yasuhiro Tanaka; Tomohiko Nakamura; Hirohisa Watanabe; Gen Sobue

doi:10.2169/internalmedicine.55.5544

CASE REPORTS

Memory Loss and Frontal Cognitive Dysfunction in a Patient with Adult-onset Neuronal Intranuclear Inclusion Disease

Kunihiko Araki, Jun Sone, Yusuke Fujioka, Michihito Masuda, Reiko Ohdake, Yasuhiro Tanaka, Tomohiko Nakamura, Hirohisa Watanabe, Gen Sobue

Author information

Kunihiko Araki
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Jun Sone
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Yusuke Fujioka
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Michihito Masuda
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Reiko Ohdake
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Yasuhiro Tanaka
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Tomohiko Nakamura
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Hirohisa Watanabe
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Gen Sobue
Department of Neurology, Nagoya University Graduate School of Medicine, Japan

Keywords: executive function, leukoencephalopathy, memory loss, neuronal intranuclear inclusion disease, skin biopsy

JOURNAL OPEN ACCESS

2016 Volume 55 Issue 16 Pages 2281-2284

DOI https://doi.org/10.2169/internalmedicine.55.5544

Details

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.

Corresponding author

Register with J-STAGE for free!