Objective: This study aims to provide a quantitative and qualitative description of the neuropsychological profile of patients diagnosed with X-linked dystonia-parkinsonism (XDP) given in a private clinic prior to deep brain stimulation (DBS) surgery.

Background: XDP is endemic to the Philippines. It is characterized by a dystonic and Parkinsonian phase. Few publications describe its non motor symptoms.

Methods: This is a retrospective descriptive study involving adult patients diagnosed with XDP. The anonymized results of the neuropsychologic tests given prior to DBS surgery were obtained from a private clinic. The raw scores, scaled scores, and corresponding descriptions were tabulated. Item and subgroup analysis were done. Other epidemiologic and clinical data, if available, were also obtained.

Results: All 18 patients in this study are males, whose ages range from 30 to 52 years old. Four patients had impairment on Verbal Comprehension and only one on Perceptual Reasoning. Seven patients showed impairment on at least one of the following subtests: Logical Memory, Verbal Learning, and Design Memory. Impairment of storage and a greater impairment of memory retrieval were observed. Nine had impairment of semantic fluency and twelve on cognitive flexibility. Three patients had impairment of planning, monitoring, self-regulation and problem-solving. Six patients had depression on HAM-D test. All patients had affectation of at least one domain of cognition or affect. There was relative sparing of abstract reasoning, confrontation naming and response inhibition. Associations between the severity of impairments and either a poor educational background, unemployment, or age were not observed.

Conclusions: The observed impairment of executive functions, memory storage and retrieval, learning and affect suggest the involvement of the frontal lobes, frontal subcortical areas and limbic lobe, consistent with the postulated corticostriatolimbic dysfunction. There is a need to screen XDP patients for cognitive impairments with a tool that can easily be used bedside or in the clinic.

5th Asian and Oceanian Parkinson’s disease and Movement Disorders Congress, March 2016.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/cognitive-profile-of-filipino-patients-with-x-linked-dystonia-parkinsonism/