The unique phenomenology of sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag").
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.
Child Neuroscience Center, Philippine Children's Medical Center, Quezon City, Philippines. lillianvleemd@yahoo.com
Lillian V
LV
Lee
Corazon
C
Rivera
Rosalia A
RA
Teleg
Marita B
MB
Dantes
Paul Matthew D
PM
Pasco
Roland Dominic G
RD
Jamora
Jose
J
Arancillo
Rodelyn F
RF
Villareal-Jordan
Raymond L
RL
Rosales
Cynthia
C
Demaisip
Elma
E
Maranon
Olivia
O
Peralta
Ruth
R
Borres
Cirnueb
C
Tolentino
Mercy Joyce
MJ
Monding
Sonia
S
Sarcia
D003937:Diagnosis, Differential; D020821:Dystonic Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics*; D005260:Female; D006580:Genetic Carrier Screening; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics*; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D008297:Male; D020734:Parkinsonian Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics*; D010679:Philippines / Q000453:epidemiology