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signal of white matter and cerebellar atrophy. Diffusion-weighted image(E) shows linear high intensity signals of corticomedullary junction, which shows iso-intensity of apparent diffusion coeffi cient map(F).
Fig. 2. Brain 99Tc-ECD SPECT Cerebral blood fl ow shows decreased blood fl ow in the bil-frontotemporal lobes.
Fig. 3. Immunohistological study of skin biopsy samples with anti-ubiquitin antibodies The upper row demonstrates immunostaining with DAB. The lower row shows
immunofl uorescence staining with DAPI (blue) and ubiquitin (green). Arrow: ubiquitin. Scale bars = 10 μm.
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A Case of Adult-onset Neuronal Intranuclear Inclusion Disease Associated with
Repeated Stroke-like Episode; A Case Report
Takahiro NATORI1), Kazumasa SHINDO1), Mai TSUCHIYA1), Megumi FUKUMOTO1), Ryusuke TAKAKI1), Nobuo YAMASHIRO1), Takamura NAGASAKA1), Jun SONE2),
Gen SOBUE2) and Yoshihisa TAKIYAMA1)
1) Department of Neurology, University of Yamanashi2) Department of Neurology, Nagoya University Graduate School of Medicine
Abstract: A 56-year-old man had a transient symptom of left upper limb paralysis in 2010 but recovered within one day. After that, he experienced similar stroke-like episodes such as right upper limb paralysis, dysarthria and right hand clumsiness, which disappeared within 1 day and repeated twelve times. Brain diffusion-weighted(DWI) MRI shows linear high intensity area of corticomedullary junction in bilateral parietal areas. Therefore, we suspected a diagnosis of neuronal intranuclear inclusion disease(NIID) and performed skin biopsy at right ankles. Intranuclear inclusion bodies were observed in fi broblasts, adipocytes and sweat glands, which were positive for an anti-ubiquitin antibody. We diagnosed him with NIID. Although NIID is reported to show various neurological symptoms, a few patients with stroke-like episodes have been reported. When we examine patients showing transient and repeated stroke-like episodes with linear high intensity areas on DWI of the brain, skin biopsy should be considered to inves-tigate the possibility of NIID.