| Autor: |
Pasco PMD; Department of Neurosciences, College of Medicine-Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.; Child Neuroscience Center, Philippine Children's Medical Center, Quezon City, Philippines., Jamora RDG; Department of Neurosciences, College of Medicine-Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.; Movement Disorders Service and Section of Neurology, Institute for Neurosciences, St. Luke's Medical Center, Quezon City and Global City, Philippines., Rosales RL; Movement Disorders Service and Section of Neurology, Institute for Neurosciences, St. Luke's Medical Center, Quezon City and Global City, Philippines.; Department of Neurology and Psychiatry, University of Santo Tomas Hospital, Manila, Philippines.; Center for Neurodiagnostic and Therapeutic Services, Metropolitan Medical Center, Manila, Philippines., Diesta CCE; Department of Neurosciences, Movement Disorders Clinic, Makati Medical Center, Makati City, Philippines., Ng AR; Movement Disorders Service and Section of Neurology, Institute for Neurosciences, St. Luke's Medical Center, Quezon City and Global City, Philippines., Teleg RA; Child Neuroscience Center, Philippine Children's Medical Center, Quezon City, Philippines.; Section of Neurology, Department of Internal Medicine, National Kidney and Transplant Institute, Quezon City, Philippines., Go CL; Departments of Neurology and Behavioral Medicine, Dr. Jose R. Reyes Memorial Medical Center, Manila, Philippines., Lee L; Child Neuroscience Center, Philippine Children's Medical Center, Quezon City, Philippines., Fernandez HH; Center for Neurological Restoration, Cleveland Clinic, Cleveland, OH USA.; Cleveland Clinic Lerner College of Medicine, Cleveland, OH USA. |
| Abstrakt: |
X-linked dystonia-parkinsonism(XDP) is a neurodegenerative disorder endemic to the Philippines. A rating scale was developed by the authors under the guidance of the Movement Disorder Society of the Philippines (MDSP) to assess XDP severity and progression, functional impact, and response to treatment in future clinical trials. Our main objective was to validate our new scale, the XDP-MDSP scale. The initial validation process included pragmatic testing to XDP patients followed by a modified Delphi procedure with an international advisory panel of dystonia, parkinsonism and scale development experts. Pearson correlation was used to assess construct validity of our new scale versus the assess construct validity of our new scale versus standard dystonia, parkinsonism, non-motor and functional scales; and also to assess divergent validity against behavioral and cognitive scales. The 37-item XDP-MDSP scale has five parts: I-dystonia, II-parkinsonism, III-non-motor features, IV-ADL, and V-global impression. After initial validation, the scale was administered to 204 XDP patients. Inter-domain correlation for the first four parts was acceptable. The correlation between these domains and the global rating was slightly lower. Correlations between Parts I, II, III, and IV versus standard dystonia, parkinsonism, non-motor and functional scales were acceptable with values ranging from 0.323 to 0.428. For divergent validity, a significant correlation was seen with behavioral scales. No significant correlation was noted with the cognitive scale. The proposed XDP-MDSP scale is internally valid but the global rating subscale may need to be modified or eliminated. While there is convergent validity, divergent validation was successful only on cognitive and not behavioral scales. The frequent co-occurrence of anxiety and depression, and its effect on the motor and functional state, may explain this finding. |
