Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

Adult-onset neuronal intranuclear inclusion disease presented transient global amnesia-a case report

Jun Takeshita, M.D.1), Hiromitsu Kobayashi, M.D.2), Yutaka Shimoe, M.D., Ph.D.1), Jun Sone, M.D., Ph.D.3), Gen Sobue, M.D., Ph.D.3) and Masaru Kuriyama, M.D., Ph.D.1)

1)Department of Neurology, Brain Attack Center Ota Memorial Hospital
2)Department of Radiology, Brain Attack Center Ota Memorial Hospital
3)Department of Neurology, Nagoya University

A 65-year-old man had a transient amnesia for about 3 hours. Similar symptoms appeared three years ago. He did not manifest dementia, cerebellar ataxia and involuntary movements. Peripheral neuropathy was observed by the neurophysiological examinations. Diffusion weighted image showed high intensity signal in the area of the corticomedullary junction of the frontal to parietal lobes and immunohistochemical studies of biopsied skin revealed many intranuclear inclusion bodies. Adult-onset neuronal intranuclear inclusion disease was diagnosed. As there was no similar member in his family, he was a sporadic case. Clinical characteristics of his amnesia was fulfilled with the criteria of transient global amnesia (TGA). The transient disturbance of limbic system was suspected.
Full Text of this Article in Japanese PDF (530K)

(CLINICA NEUROL, 57: 303|306, 2017)
key words: neuronal intranuclear inclusion disease, adult-onset, transient global amnesia, skin biopsy, DWI

(Received: 22-Dec-16)