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In addition, these inclusions are present in the peripheral autonomic nervous system, visceral organs and skin. In NIID cases with an expansion of GGC repeats in the 5\u2032\u2010untranslated region (5\u2032\u2010UTR) of the Notch 2 N\u2010terminal like C (NOTCH2NLC) gene, these repeats are located in an upstream open reading frame (uN2C) and result in the production of a polyglycine\u2010containing protein called uN2CpolyG. Typically, patients with adult NIID show high\u2010intensity signals at the corticomedullary junction on diffusion\u2010weighted brain magnetic resonance imaging. We report a case of adult NIID in a 78\u2010year\u2010old Japanese male, who suffered from mild, non\u2010progressive tremor during life but showed no radiographic abnormalities suggestive of adult NIID. Pathologically, ubiquitin\u2010, p62\u2010 and uN2CpolyG\u2010positive neuronal intranuclear inclusions were particularly frequent in the hippocampal formation, but were also seen in the enteric plexuses, kidney and cardiac muscles. By contrast, glial intranuclear inclusions were barely evident in the affected regions. The present case also had an immunohistochemical profile differing from that of typical adult NIID. 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