Table 1

Demographics and clinical features of patients with X linked dystonia parkinsonism (XDP)

These data are derived from the XDP Philippine Registry, 2000.
Ages and durations are in years.
No. of XDP cases registered 1975–2000373
No. of survivors November 2000268
    Philippine population as of 200074.7 M
    Prevalence of surviving cases/100 0000.36
    Prevalence of cases in Capiz/100 00021.94
    Prevalence of cases in Aklan/100 0007.72
    Prevalence of cases in Iloilo/100 0001.43
    Prevalence of cases in Antique/100 0000.86
    Prevalence of cases in Guimaras/100 0000.73
No. of families187
No. of cases with positive family history343
No. of deaths105
No. of cases that underwent necropsy6
Sex, male/female (%)370 (99)/3 (1)
Mean age at onset (range)39.5 (12–64)
Mean age at initial examination (range)40.8 (20–70)
Mean duration of illness from onset to present mean (range)13.04 (1–41)
Mean duration of illness from onset to generalisation of dystonia (range)3.8 (1–23)
Mean duration of illness from onset to parkinsonism (range)13.39 (7–25)
Mean age of survivors (range)52.73 (28–86)
Mean age at death (range)52.06 (31–81)
No. initially presenting with parkinsonism (%)24 (6)
No. initially presenting with dystonia (%)349 (94)
    Lower extremities122 (33)
    Craniofacial102 (27)
    Cervical and shoulder92 (25)
    Upper extremities52 (14)
    Trunk5 (1)
No. of cases, spread (%)363 (97)
No. of cases, generalised (%)313 (84)
No. of cases, parkinsonian (%)53 (14)
Degree of disability of survivors
    No. still working (%)13 (5)
    No. ambulant, not working (%)192 (72)
    No. non-ambulant; able to care for self (%)3 (1)
    No. wheelchair and bed bound (%)60 (22)