The unique phenomenology of sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag")

Int J Neurosci. 2011:121 Suppl 1:3-11. doi: 10.3109/00207454.2010.526728. Epub 2010 Nov 3.

Abstract

Sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag") is an adult-onset, progressive, debilitating movement disorder first described in Filipino males from Panay Islands in 1975. XDP manifests predominantly as torsion dystonia, later combined with or sometimes replaced with parkinsonism. Within the Island of Panay, the prevalence rate is highest in the province of Capiz, where 1:4000 men suffer from the disorder. There is a high degree of penetrance and generalization. While women often serve as carriers, XDP is not limited to men. An updated XDP Philippine registry (as of January 2010) has identified 505 cases, with 500 males and 5 females. While some report that females may carry a milder form of the disorder, in our experience, both sexes generally follow a similar progressive clinical course.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Diagnosis, Differential
  • Dystonic Disorders / diagnosis
  • Dystonic Disorders / epidemiology*
  • Dystonic Disorders / genetics*
  • Female
  • Genetic Carrier Screening
  • Genetic Diseases, X-Linked / diagnosis
  • Genetic Diseases, X-Linked / epidemiology*
  • Genetic Diseases, X-Linked / genetics*
  • Genetic Predisposition to Disease / genetics*
  • Humans
  • Male
  • Parkinsonian Disorders / diagnosis
  • Parkinsonian Disorders / epidemiology*
  • Parkinsonian Disorders / genetics*
  • Philippines / epidemiology