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Case of maternally transmitted juvenile Huntington's disease with a very large trinucleotide repeat

✍ Scribed by Spiridon Papapetropoulos; Roberto Lopez-Alberola; Lisa Baumbach; Angela Russell; Manuel A. Gonzalez; Brian C. Bowen; Carlos Singer


Publisher
John Wiley and Sons
Year
2005
Tongue
English
Weight
62 KB
Volume
20
Category
Article
ISSN
0885-3185

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✦ Synopsis


Abstract

We describe and present a video of a patient with maternally inherited juvenile Huntington's disease (HD) caused by a very large (108‐repeat) expansion. Maternally transmitted very large trinucleotide repeats (>100) are extremely rare in juvenile HD and may represent instability during female gametogenesis. © 2005 Movement Disorder Society