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• Vol.58 No.6
• CLINICA NEUROL, 58: 407−410, 2018
• Vol.58 No.6 contents

Brief Clinical Note

Sporadic adult-onset neuronal intranuclear inclusion disease with abnormal electroretinogram, nerve conduction studies and somatosensory evoked potential

Bungo Hirose, M.D.¹⁾, Shin Hisahara, M.D., Ph.D.¹⁾, Haruo Uesugi, M.D., Ph.D.²⁾, Jun Sone, M.D., Ph.D.³⁾, Gen Sobue, M.D., Ph.D.³⁾ and Shun Shimohama, M.D., Ph.D.¹⁾

¹⁾Department of Neurology, Sapporo Medical University School of Medicine
²⁾Department of Neurology, Sapporo Yamanoue Hospital
³⁾Department of Neurology, Nagoya University Graduate School of Medicine

A 70-year-old man, a urinary retention of unknown origin from 10 years ago, decreased cognitive function from 4 years ago, vision impairment advanced a year ago. Brain MRI with DWI showed high intensity erea in the corticomedullary junction. We diagnosed as intranuclear inclusion body disease (NIID) because of p62-positive intranuclear inclusion bodies by skin biopsy. Electroretinogram revealed amplitude reduction in the cone response superiority. Nerve conduction test showed mild conduction velocity reduction. Furthermore, in the somatosensory evoked potential of the lower limb, latency of the first cortical component was prolonged. These electrophysiological abnormalities were considered to be associated with the pathological features of NIID.
Full Text of this Article in Japanese PDF (479K)

(CLINICA NEUROL, 58: 407−410, 2018)
key words: neuronal intranuclear inclusion disease, nerve conduction studies, sensory evoked potential, electroretinogram, cone cell

(Received: 9-Feb-18)