Frequent manifestations that differed from those of PSP included CSF abnormalities, such as elevated protein or cell counts and positive CSF-specific oligoclonal bands (OCBs) (8/12), no atrophy of midbrain tegmentum on MRI (6/24), weight loss (3/24: associated with anti-DPPX, anti-CV2/CRMP5, and anti-KLHL11 antibody in one case each), and sleep disturbance and limb ataxia (3/24: all associated with the anti-IgLON5 antibody). The gene discussed is KLHL11; the disease is supranuclear palsy, progressive, 1.