Abstract
X-linked dystonia-parkinsonism (XDP) is a debilitating movement disorder endemic to the Panay Island, Philippines. Most studies focus on motor symptoms, hence we reviewed the neurocognitive profile of XDP patients. Neurocognitive testing of XDP patients focused on five domains: general intellectual functioning, episodic memory, language, attention and executive function, and affect. Twenty-nine genetically confirmed patients were included. Twenty-six (89.6%) had impairments in one or more domains, while only three had no impairment in any domain. Attention and executive function was the most commonly affected domain (n = 23, 79.3%). Deficits in general intellect, episodic memory, attention and executive function and affect were seen in our subset of XDP patients. The striatal pathology affecting the frontostriatal circuitry mandating these cognitive processes is mainly implicated in these impairments. The results of our study provided further evidence on the extent of cognitive impairment in XDP using a select battery of neurocognitive tests.
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The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.
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Acknowledgements
We would like to thank Dr. Aloysius Domingo, Ms. Joyce Monding-Yabres, Ms. Estrela Silverio and Ms. Ena Anadelle Honrado for their assistance with this study.
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RDGJ: conceptualization, data curation, formal analysis, interpretation of data, writing–original draft, writing–review, and editing. CTRS: conceptualization, data curation, formal analysis, interpretation of data, writing-original draft, writing-review, and editing. JECB: conceptualization, data curation, formal analysis, interpretation of data, writing–original draft, and writing–review. GMIR: conceptualization, data curation, formal analysis, interpretation of data, writing–original draft, and writing–review. AW: formal analysis, interpretation of data, writing–review, and editing. CK: formal analysis, interpretation of data, writing–review, and editing. LKL: conceptualization, data curation, formal analysis, interpretation of data, writing–review, and editing.
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Jamora, R.D.G., Suratos, C.T.R., Bautista, J.E.C. et al. Neurocognitive profile of patients with X-linked dystonia-parkinsonism. J Neural Transm 128, 671–678 (2021). https://doi.org/10.1007/s00702-021-02317-z
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DOI: https://doi.org/10.1007/s00702-021-02317-z