Objective:

The purpose of this study is to know the prevalence of juvenile cases in a sample of mexican subjects with confirmed Huntington Disease (HD).

Methods:

Patients with clinical debut before 21 years of age were included who attended at movement disorders clinic of the National Institute of Neurology and Neurosurgery. The demographic and clinical information was obtained from the review of files.

Results:

A total of 198 cases of patients diagnosed with HD were reviewed, of which 6.5% (n = .13) corresponded to juvenile forms. The mean age for the onset of symptoms was 17.8 ± 3.9 years. The mean score of the UHDRS-motor was 46.2 ± 17.4 points. The predominant motor symptom was chorea in (53.8%) of the cases. 84.6% of those affected presented at least one neuropsychiatric disorder.

Conclusion:

It was detected that the dominant motor phenotype of these patients was chorea compared to the world reports until now, accordingly to that, our group of juvenile HD shows atypical motor clinical.