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January 1992

'Haw River Syndrome' or Dentato-Rubro-Pallido-Luysian Atrophy?

Author Affiliations

Movement Disorders Center University of Miami School of Medicine 1501 NW Ninth Ave Miami, FL 33136

Arch Neurol. 1992;49(1):13. doi:10.1001/archneur.1992.00530250017002

To the Editor.  —Farmer et al1 report on five generations of a family exhibiting an autosomal dominant disorder characterized by the development (usually between 15 and 30 years of age) of ataxia, seizures, choreiform movements, progressive dementia, and death after 15 to 25 years of illness. They name the disorder "Haw River syndrome" because the first recorded member of this family lived at Haw River, North Carolina.The authors provide neuropathologic data on two of the 18 affected members who had been examined. The findings included marked neuronal loss of the dentate nucleus, microcalcification of the globus pallidus, neuroaxonal dystrophy of the nucleus gracilis, and demyelination of the centrum semiovale.Farmer and colleagues argue that the lack of gross abnormalities in the pallidum, the presence of neuroaxonal dystrophy of the nucleus gracilis, and the demyelination of the centrum semiovale distinguish their cases from dentato-rubro-pallido-luysian atrophy.I would like to

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