The unique phenomenology of sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag") .
- Author:
Lee Lillian V.
;
Rivera Corazon
;
Teleg Rosalia A.
;
Dantes Marita B.
;
Pasco Paul Matthew D.
;
Arancillo Jose
;
Jamora Roland Dominic G.
;
Villareal-Jordan Rodelyn F.
;
Demaisip Cynthia
;
Maranon Elma
;
Peralta Olivia
;
Rosales Raymond L.
;
Borres Ruth
;
Tolentino Cirnueb
;
Monding Mercy Joyce
;
Sarcia Sonia
- Publication Type:Journal Article
- Keywords: Dystonia Parkinsonism; Muscle Dystonia
- MeSH: Human; Male; Female; Aged; Adult; Dystonia; Dystonia Musculorum Deformans; Dystonic Disorders; Genetic Diseases, X-linked; Islands; Parkinsonian Disorders; Penetrance
- From: Philippine Journal of Neurology 2012;16(1):63-71
- CountryPhilippines
- Language:English
-
Abstract:
Sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag") is an adult-onset, progressive, debilitating movement disorder first described in Filipino males from Panay Island in 1975. XDP manifests predominantly as torsion dystonia, later combined with or sometimes replaced with parkinsonism. Within the Island of Panay, the preva-lence 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.