Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease

Citation
N. Georgiou et al., Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease, MOVEMENT D, 14(2), 1999, pp. 320-325
Citations number
27
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
MOVEMENT DISORDERS
ISSN journal
0885-3185 → ACNP
Volume
14
Issue
2
Year of publication
1999
Pages
320 - 325
Database
ISI
SICI code
0885-3185(199903)14:2<320:DCAMCF>2.0.ZU;2-2
Abstract
We report a pair of monozygotic Huntington's disease (HD) twins who, althou gh sharing identical CAG repeat lengths, not only present with marked diffe rences in clinical symptoms but also behavioral abilities as measured by ou r experimental procedures. Both HD twins and two healthy control subjects w ere tested twice over 2 years. Patient A was generally more impaired at a m otor level, whereas Patient B showed greater attentional impairment; Patien t B, however, showed more progressive deterioration. The control subjects' performance remained consistent over the 2-year interval. Patient A clinica lly had the more hyperkinetic hypotonic variant of the disease, whereas Pat ient B, who was the more impaired, presented with a more hypokinetic hypert onic (rigid) variant. The influences of epigenetic pre- and postnatal envir onmental factors should not be ignored.