Like many other neurodegenerative disorders, symptoms usually begin insidiously in the sixth to eighth decade and gradually progress over 3-15 yr until death. Males and females are equally affected. A defining characteristic of the CBS is asymmetric limb findings, but there does not appear to be any predilection to the right or left side. A relatively high frequency of coexisting autoimmune diseases was noted in one series (12). This is a sporadic disorder, although there are rare reports of a similarly affected relative (13).
The incidence and prevalence of the CBS and CBD are not known. Bower et al. did not identify any case of CBS or CBD in their analysis of parkinsonism in Olmsted County, Minnesota (14). However, in a review of the autopsy records of the Mayo Clinic from 1970 to 2000, two residents of Olmsted County, Minnesota, were found to have CBD pathology, with one exhibiting the CBS and the other exhibiting dementia of the Alzheimer type (B. Boeve, unpublished data). As of 2003, we are following 11 patients with the CBS in the Mayo Alzheimer's Disease Research Center who reside in the state of Minnesota. Considering there are approx 5 million residents in the state, a minimum prevalence estimate is at least 2 per million (B. Boeve, unpublished data). The rarity of the CBS and CBD will make more definitive incidence and prevalence estimates difficult.
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