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Pathology in brainstem regions of individuals with primary dystonia

From the Neuropathology Laboratory (Drs. Zweig, Hedreen, Casanova, Whitehouse, and Price), and the Departments of Pathology (Drs. Zweig, Hedreen, Casanova, and Price), Neuroscience (Drs. Hedreen, Whitehouse, and Price), and Neurology (Drs. Jankel, Whitehouse, and Price), The Johns Hopkins University School of Medicine, Baltimore, MD; the Neuropathology Laboratory (Dr. Casanova), National Institute of Mental Health, St. Elizabeth's Hospital, Washington, DC; and the Alzheimer Center (Dr. Whitehouse), University Hospitals of Cleveland, Cleveland, OH. Dr. Zweig's present address is the Department of Medicine, Veteran's Administration Medical Center, Reno, NV.

Examination of brains from four individuals with the clinical diagnosis of primary dystonia revealed histopathologic abnormalities in two cases. A 29-year-old man with a 15-year history of dystonia musculorum deformans (DMD) had numerous neurofibrillary tangles (NFT) and mild neuronal loss within the locus ceruleus; occasional NFT were also recognized in the substantia nigra pars compacta, pedunculopontine nucleus, and dorsal raphe nucleus. A 68-year-old man with a 35-year history of Meige syndrome had moderate-to-severe neuronal loss in several brainstem nuclei, including the substantia nigra pars compacta, locus ceruleus, raphe nuclei, and pedunculopontine nucleus. Infrequent NFT were also noted in substantia nigra. An examination of these and other brain regions in a 10-year-old boy with a 6-year history of DMD and a 50-year-old woman with a 3-year history of spasmodic torticollis did not disclose similar abnormalities.

Address correspondence and reprint requests to Dr. Hedreen, Neuropathology Laboratory, 509 Pathology Building, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Baltimore, MD 21205–2182.

Supported by grants from the US Public Health Service (NIH NS 07179, NS 06735, and AG 05146), as well as funds from the Dystonia Medical Research Foundation.

Received May 4, 1987. Accepted for publication in final form September 15, 1987.

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