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January 1992

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

Author Affiliations

Department of Neurology School of Medicine University of North Carolina Chapel Hill, NC 27599-7025
Department of Pathology Duke University Medical Center Durham, NC 27710

Arch Neurol. 1992;49(1):13-14. doi:10.1001/archneur.1992.00530250017003

In Reply.  —Singer correctly points out the similarities between dentato-rubro-pallido-luysian atrophy and Haw River syndrome. However, as pointed out in our comment,1 we still feel that the differences are adequate to merit a separate name. We have recently had the opportunity to examine two additional family members at autopsy. The first was symptomatic but was still functioning in the community until death. The only changes in the brain were mild neuronal loss in the dentate nucleus and calcification of the globus pallidus. The brain stem and spinal cord were not examined. Another family member was institutionalized and died at age 29 years. At autopsy, there was severe neuronal loss in the dentate nucleus, calcification of the globus pallidus, neuroaxonal dystrophy in the nucleus gracilis, and demyelination of the optic nerves. The subthalamic nucleus and red nucleus were histologically normal, although mild gliosis of the red nucleus was revealed by