New insights from a multi-ethnic Asian progressive supranuclear palsy cohort

Lim SY 1 , Dy Closas AMF 2 , Tan AH 2 , Lim JL 3 , Tan YJ 4 , Vijayanathan Y 2 Show all authors , Tay YW 3 , Abdul Khalid RB 5 , Ng WK 6 , Kanesalingam R 7 , Martinez-Martin P 8 , Ahmad Annuar A 3 , Lit LC 9 , Foo JN 10 , Lim WK 11 , Ng ASL 4 , Tan EK 4

Affiliations 

  • 1 Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia; The Mah Pooi Soo & Tan Chin Nam Centre for Parkinson's & Related Disorders, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia. Electronic address: limshenyang@gmail.com
  • 2 Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia; The Mah Pooi Soo & Tan Chin Nam Centre for Parkinson's & Related Disorders, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia
  • 3 Department of Biomedical Science, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia
  • 4 Department of Neurology, National Neuroscience Institute, Singapore
  • 5 Assunta Hospital, Petaling Jaya, Selangor, Malaysia
  • 6 Subang Jaya Medical Centre, Subang Jaya, Selangor, Malaysia
  • 7 KPJ Puteri Specialist Hospital, Johor Bahru, Malaysia
  • 8 Center for Networked Biomedical Research in Neurodegenerative Diseases (CIBERNED), Carlos III Institute of Health, Madrid, Spain
  • 9 Department of Physiology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia
  • 10 Lee Kong Chian School of Medicine, Nanyang Technological University Singapore, Singapore
  • 11 SingHealth Duke-NUS Institute of Precision Medicine, Singapore; Cancer and Stem Cell Biology Program, Duke-NUS Medical School, Singapore
Parkinsonism Relat Disord, 2023 Mar;108:105296.
PMID: 36682278 DOI: 10.1016/j.parkreldis.2023.105296

Abstract

BACKGROUND: Progressive supranuclear palsy (PSP) is a rare, disabling, neurodegenerative disease, with few studies done in Asian populations.

METHODS: We prospectively characterized the clinical features and disease burden in a consecutively-recruited multi-ethnic Asian PSP cohort. Patients were extensively phenotyped using the Movement Disorder Society (MDS-PSP) clinical diagnostic criteria and the PSP-Clinical Deficits Scale (PSP-CDS). Caregiver burden was measured using the modified Zarit Burden Interview (ZBI). Investigations (neuroimaging and genetic tests) were reviewed.

RESULTS: There were 104 patients (64.4% male; 67.3% Chinese, 21.2% Indians, 9.6% Malays), consisting of 48.1% Richardson syndrome (PSP-RS), 37.5% parkinsonian phenotype (PSP-P), and 10.6% progressive gait freezing phenotype (PSP-PGF). Mean age at motor onset was 66.3 ± 7.7 years, with no significant differences between the PSP phenotypes. Interestingly, REM-sleep behaviour disorder (RBD) symptoms and visual hallucinations (considered rare in PSP) were reported in 23.5% and 22.8% of patients, respectively, and a family history of possible neurodegenerative or movement disorder in 20.4%. PSP-CDS scores were highest (worst) in PSP-RS; and correlated moderately with disease duration (rs = 0.45, P 

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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