RT Journal Article
SR Electronic
T1 Hereditary Ataxia with Sensory Neuronopathy
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 273
OP 275
DO 10.1212/WNL.48.1.273
VO 48
IS 1
A1 Nachmanoff, D. B.
A1 Segal, R. A.
A1 Dawson, D. M.
A1 Brown, R. B.
A1 De Girolami, U.
YR 1997
UL http://n.neurology.org/content/48/1/273.abstract
AB We report the case of a man with late-onset hereditary ataxia and sensory loss. Three of his sisters were affected by a similar disorder; to date no other members of his family have developed symptoms. The clinical features of this family are similar to a rare form of autosomal dominant hereditary ataxia, recently classified as SCA4. Postmortem findings indicate that this syndrome is marked by degeneration of cerebellar Purkinje cells, dorsal root sensory ganglion neurons, and the ascending posterior columns. Similar clinical and pathologic findings were reported by Biemond in 1954. NEUROLOGY 1997;48: 273-275