Ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder.

@article{Farmer1989AtaxiaCS,
  title={Ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder.},
  author={Thomas W. Farmer and Martha S. Wingfield and Sally Ann Lynch and F. Stephen Vogel and Christine Hulette and B Katchinoff and Pedro Jacobson},
  journal={Archives of neurology},
  year={1989},
  volume={46 7},
  pages={774-9}
}
Five generations of a family exhibit a unique autosomal dominant neurologic 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. Neuropathologic findings in two deceased family members revealed remarkably similar findings, including marked neuronal loss of the dentate nucleus, microcalcification of the globus pallidus, neuroaxonal dystrophy of the nucleus… CONTINUE READING
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