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Pathologic findings in prospectively ascertained essential tremor subjects

From the Sun Health Research Institute (H.A.S., M.N.S., D.J.C., T.G.B.), Sun City, AZ; and Parkinson's Disease and Movement Disorders Center (C.H.A., J.N.C.) and Section of Biostatistics (J.G.H.), Mayo Clinic, Scottsdale, AZ.

Address correspondence and reprint requests to Dr. Holly Shill, 10515 W. Santa Fe Dr., Sun City, AZ 85351

Objective: To assess pathologic changes in prospectively characterized subjects with essential tremor (ET).

Methods: Subjects enrolled in the Sun Health Research Institute Brain and Body Donation Program were examined annually by a movement disorders neurologist, and semiannually by a behavioral neurologist and neuropsychologist. Twenty-four subjects without a prior diagnosis of dementia or other major movement disorder met clinical criteria for ET and came to autopsy. Subjects with mild cognitive impairment (n = 3) were included. These subjects were compared with 21 controls. Brains were examined postmortem according to standardized protocols for assessment of age-related changes and specific pathologic conditions (e.g., Parkinson disease, Alzheimer disease).

Results: Subjects had a mean age of 86.2 years and a mean duration of tremor of 11.1 years. Seven subjects had evidence for cerebellar pathology (Purkinje cell loss, cerebellar cortical sclerosis, and proliferation of Bergmann glia). Pigmented neurons were qualitatively depleted in the locus ceruleus in eight subjects and in the substantia nigra in five subjects. Of these, three had Lewy bodies, one subject had brainstem predominant disease, and two had limbic stage. Three subjects had a nonspecific cerebral tauopathy and another met pathologic criteria for progressive supranuclear palsy. However, when compared with controls, only changes in the locus ceruleus and gliosis of the cerebellum remained significant findings.

Conclusions: This study supports previous findings of heterogenous pathology in essential tremor (ET). There is an increased frequency of cerebellar gliosis and locus ceruleus depletion. We did not find an increased incidence of Lewy bodies in subjects with ET.

GLOSSARY: ET = essential tremor; LB = Lewy body; MCI = mild cognitive impairment; NA = not available; PD = Parkinson disease; PSP = progressive supranuclear palsy; SHRI = Sun Health Research Institute; SN = substantia nigra; WMR = white matter rarefaction.

holly.shill{at}sunhealth.org

Supported by the Sun Health Research Institute and the Mayo Foundation. Arizona Biomedical Research Commission provided funding through 04-800, 40001, and 05-901. The Michael J. Fox Foundation provided funding through the Prescott Family Initiative.

Disclosure: None of the authors has a financial relationship with the sponsors of this study outside the activities described in this manuscript.

Received July 20, 2007. Accepted in final form October 25, 2007.