Objective: To evaluate the Disability-Adjusted Life Years in X-linked Dystonia Parkinsonism patients at the Health Centrum, Movement Disorders Clinic, Roxas City Capiz, Philippines.

Background:  The disability-adjusted life year (DALY) is a measure of overall disease burden, expressed as the number of years lost due to ill-health, disability or early death. At present, there has been no report to quantify the disease burden among patients with X-linked dystonia parkinsonism (XDP, DYT3, “Lubag”) which is an adult-onset, progressive, debilitating movement disorder presenting with varying degrees of dystonia and parkinsonism. 

Methods:  All subjects fulfilling the clinical criteria of XDP seen at a movement disorder clinic in Capiz, Roxas City from August 2015 to mid-December 2016 and were positive for the gene were included in this study. Baseline demographics and the following information were obtained: sex, age of onset, duration of disease and the age of death of the subjects.  The Disability-Adjusted Life Years for each subject was computed using the formula DALY = YLL + YLD, where YLL (years of life lost) = (standard life expectancy of a person who lives in the Province of Capiz – age at death)  and  YLD (years lived with disability) = DW  disability weight of specific condition, and L = duration of the disease. Since there was no Disability weight associated with Dystonia and since XDP has a wide range of presentation from focal dystonia, to slight parkinsonism all the way to debilitating generalized dystonia throughout a person’s life, the use of Parkinson’s score was determined to be a good marker for disability weight at 0.351.  After tallying the DALY for all the subjects, the mean DALY value of both the recently deceased and the living were ascertained.   Descriptive statistical analysis was used.

Results:  Of the 10 subjects who have died since August 2015, the average DALY was 19.68, the average of YLL due to dying earlier than the average life expectancy is 66.82 for men and 72.85 for women in the Province of Capiz is 17.82 with an average duration of illness of 5.3 years until their death.  For the remaining 99 living subjects, their average YLD and thus their DALY is 2.96 years with a range between 0.702 to 9.477 years.

Conclusions: This is the first report to describe the mean baseline Disability-Adjusted Life Years (DALY) in X-linked Dystonia Parkinsonism subjects.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/disability-adjusted-life-years-daly-in-x-linked-dystonia-parkinsonism/