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Article

Sporadic cases of dentatorubral-pallidoluysian atrophy associated with maternal transmission.

Neurology - 1 Jan 1998

Shimohata T, Ishiguro H, Makino K, Takano H, Tanaka H, Tsuji S, Hirota K

Abstract excerpt

We report siblings, a 21-year-old woman (proband) and her 26-year-old brother, with dentatorubral-pallidoluysian atrophy (DRPLA). There was no family history of DRPLA and no clinical abnormalities in their parents, who were both above the age of 50. Analysis of the DRPLA gene of leukocytes showed CAG repeat sizes to be 64/17 in the proband, 58/20 in her brother, and 56/8 in their mother. Sporadic cases of DRPLA...

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PMID: 9443494
DOI: 10.1212/wnl.50.1.282

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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