Abstract

Background Substantia nigra (SN) hyperechogenicity determined by transcranial sonography (TCS) and olfactory dysfunction are common findings in Parkinson disease (PD), which may reveal a prodromal synucleinopathy in idiopathic REM sleep behavior disorder (iRBD). Methods TCS and the Odor Stick Identification Test for Japanese (OSIT-J) were performed in 34 consecutive patients with iRBD (67.9 ± 6.1 years), 17 consecutive patients with PD (66.4 ± 6.7 years), and 21 control group subjects (64.4 ± 5.8 years). Results There was a significantly increased area of echogenicity in the SN in the iRBD group (0.20 ± 0.13 cm 2) and PD group (0.22 ± 0.11 cm 2) compared with the control group (0.06 ± 0.06 cm 2). We found pathological SN hyperechogenicity (⩾0.20 cm 2) in 41.2% of the iRBD group, 52.6% of the PD group, and 9.5% of the control group. Further, there were abnormal findings of both pathological SN hyperechogenicity (⩾0.20 cm 2) and functional anosmia or hyposmia in 4 (11.8%) or 9 (26.5%) of the iRBD group subjects, respectively, and 7 (57.9%) or 2 (11.8%) of the PD group subjects, respectively. Conclusion Pathological SN hyperechogenic abnormality and functional anosmia in iRBD may be a disease state in the transition to a neurodegenerative disease.

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