Huntington's disease in Venezuela

• DOI: 10.1212/wnl.36.2.244
• Publication Date: 2012-05-13T12:38:39Z
• Authors: Anne B. Young; Ira Shoulson; John B. Penney; Simon Starosta-Ru...; Fidela Gomez; Helen Travers; Maria A. Ramos-Ar...; S. Robert Snodgrass; Ernesto Bonilla; Humberto Moreno; Nancy S. Wexler
• Keywords: Adult; Male; Heterozygote; Time Factors; Homozygote; Venezuela; Muscle Rigidity; Pedigree; 3. Good health; Dystonia; 03 medical and health sciences; Huntington Disease; 0302 clinical medicine; Chorea; Child, Preschool; Tremor; Saccades; Humans; Female; Prospective Studies; Child

We studied 65 Huntington's disease patients and 225 at-risk individuals over the past 4 years. The rate of decline of these untreated patients from Venezuela was similar to that seen in US patients who had received neuroleptic drugs. Chorea, oculomotor dysfunction, and dysdiadochokinesis were early symptoms; parkinsonian features and dystonia came later. Juvenile patients declined nearly twice as fast as adult-onset patients. No distinctive neurologic phenotypes were seen in children of two affected parents.