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

Carlos Singer, MD
Arch Neurol. 1992;49(1):13-13. doi:10.1001/archneur.1992.00530250017002
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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

REFERENCES

Farmer TW, Wingfield MS, Lynch SA, et al.  Ataxia, chorea, seizures, and dementia: pathologic features of a newly defined familial disorder . Arch Neurol . 1989;;46:774-779.
Izuka R, Hirayama K.  Dentato-rubro-pallido-luysian atrophy . In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of Clinical Neurology . Amsterdam, the Netherlands: Elsevier Science Publishers BV; 1986;;49:437-443.
Seitelberger F.  Neuroaxonal dystrophy: its relation to aging and neurological diseases . In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of Clinical Neurology . Amsterdam, the Netherlands: Elsevier Science Publishers BV; 1986;;49:391-415.
Pfeiffer RF, McComb RD.  Dentatorubro-pallidoluysian atrophy of the myoclonus epilepsy type with posterior column degeneration . Mov Disord . 1990;;5:134-138.

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Farmer TW, Wingfield MS, Lynch SA, et al.  Ataxia, chorea, seizures, and dementia: pathologic features of a newly defined familial disorder . Arch Neurol . 1989;;46:774-779.
Izuka R, Hirayama K.  Dentato-rubro-pallido-luysian atrophy . In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of Clinical Neurology . Amsterdam, the Netherlands: Elsevier Science Publishers BV; 1986;;49:437-443.
Seitelberger F.  Neuroaxonal dystrophy: its relation to aging and neurological diseases . In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of Clinical Neurology . Amsterdam, the Netherlands: Elsevier Science Publishers BV; 1986;;49:391-415.
Pfeiffer RF, McComb RD.  Dentatorubro-pallidoluysian atrophy of the myoclonus epilepsy type with posterior column degeneration . Mov Disord . 1990;;5:134-138.

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