Ethnic Differences in Atypical Parkinsonism—is South Asian PSP Different?

作者：Bettina Balint, Shermyn Neo, Francesca Magrinelli, Eoin Mulroy, Anna Latorre, María Stamelou, Huw R. Morris, Amit Batla, Kailash P. Bhatia · 发表于：Movement Disorders Clinical Practice · 年份：2024 · DOI：10.1002/mdc3.14182 · 被引用次数：12 · 研究领域：Parkinson's Disease Mechanisms and Treatments、Genetic Neurodegenerative Diseases、Amyotrophic Lateral Sclerosis Research

BACKGROUND: Progressive supranuclear palsy (PSP) is a progressive atypical parkinsonian condition that results in severe disability. There are few studies of PSP in patients of non-white European ancestry. OBJECTIVES: We aim to perform deep phenotyping in a South Asian PSP cohort to uncover possible ethnic differences in disease characteristics. METHODS: Consecutive PSP patients had their clinical records reviewed for clinical features operationalized in the Movement Disorder Society (MDS)-PSP diagnostic criteria and relevant investigations, including imaging and genetic tests. Clinical variables were summarized by descriptive statistics and Kaplan-Meier curves were generated for survival analysis. RESULTS: Twenty-seven patients, comprising Indians (78%), Pakistanis (11%) and Sri Lankans (11%) were included. Mean age of symptom onset was 63.8 ± 7.0 years and 22% of patients had an early age of onset (<60 years). The most common presenting symptom was parkinsonism (56%), followed by cognitive dysfunction (37%), falls (33%) and dysarthria (26%). The predominance types at final review were distributed across PSP-RS (67%), PSP-PGF (15%), PSP-P (15%) and PSP-F (4%). Atypical clinical features like cerebellar signs (33%), REM-sleep behavior disorder (RBD) (55%), visual hallucinations (22%), and a family history of parkinsonism (20%) were evident in a proportion of patients. CONCLUSIONS: We present a South Asian cohort of PSP patients with a higher than previously reported percentag...