Neuronal nuclear inclusion disease (NIID) is a rare and chronic progressive neurological degenerative disease. We presented a 68-year-old man with paroxysmal orientation disorder 1 year prior, mental and behavioral disorders for 2 days, and confirmed the diagnosis of NIID with skin biopsy. We suggest that patients with atypical clinical symptoms showed characteristic high signal in the dermatomedullary junction on DWI; NIID should be considered.
Keywords: genetic analysis; mental and behavioral disorders; neuronal nuclear inclusion disease; p62 staining; skin biopsy.
© 2022 The Authors. Aging Medicine published by Beijing Hospital and John Wiley & Sons Australia, Ltd.