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Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease
Author(s) -
Georgiou Nellie,
Bradshaw John L.,
Chiu Edmond,
Tudor Anne,
O'Gorman Louise,
Phillips Jim G.
Publication year - 1999
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/1531-8257(199903)14:2<320::aid-mds1018>3.0.co;2-z
Subject(s) - huntington's disease , monozygotic twin , disease , degenerative disease , psychology , medicine , audiology , pediatrics , genetics , biology
We report a pair of monozygotic Huntington's disease (HD) twins who, although sharing identical CAG repeat lengths, not only present with marked differences in clinical symptoms but also behavioral abilities as measured by our experimental procedures. Both HD twins and two healthy control subjects were tested twice over 2 years. Patient A was generally more impaired at a motor level, whereas Patient B showed greater attentional impairment; Patient B, however, showed more progressive deterioration. The control subjects' performance remained consistent over the 2‐year interval. Patient A clinically had the more hyperkinetic hypotonic variant of the disease, whereas Patient B, who was the more impaired, presented with a more hypokinetic hypertonic (rigid) variant. The influences of epigenetic pre‐ and postnatal environmental factors should not be ignored.