Objective: To characterize the demographic, clinical and diagnostic features of patients diagnosed with corticobasal syndrome (CBS) seen at The Queen’s Medical Center in Honolulu, Hawaii.

Background: CBS is a rare neurodegenerative disorder, estimated to be ten times rarer than progressive supranuclear palsy (PSP), which commonly presents as an array of cortical and extrapyramidal signs and symptoms. Studies show average age of disease onset to be 64 years with a slight female predominance and an estimated mean survival of 6.5 years; however, limited research exists on CBS in diverse non-White populations.¹

Method: We performed a retrospective chart review of all patients meeting the Armstrong criteria² for probable or possible CBS (2006-2025). Data extracted included age of symptom onset, diagnosis, last follow-up and death. Survival rate was analyzed using a Kaplan-Meier estimator; 8 deaths occurred during the study period, with remaining patients censored at last follow-up. Group differences were assessed using Kruskal-Wallis rank sum tests with Bonferroni-corrected post-hoc comparisons.

Results: A total of 24 (White=10, Asian American [AA]=12, Native Hawaiian or Pacific Islander [NHPI]=2) patients were identified; 50% (White=7, AA=3, NHPI=2) were male. Mean age of symptom onset (in years) was 69.2 ± 8.6 and diagnosis 72.4 ± 7.6. Median age of symptom onset was significantly higher in AA patients (76.5; IQR=13.3) compared to White patients (64; IQR=11.3), p=0.03. The majority of patients met criteria for possible CBS (n=15, 63%), while 37% met criteria for probable CBS. Most common symptoms were limb rigidity or akinesia (n=24), limb dystonia (n=23) and orobuccal or limb apraxia (n=19). Two patients had symmetric presentation. Median survival was not reached; estimated 3-year survival was 91.7% (95% CI 81-100%) and 6-year survival was 74.6% (95% CI 57-98%).

Conclusion: The observed age of symptom onset of AA patients was higher than prior reports, highlighting possible population-specific variation. Racial disparities may exist in CBS, underscoring the importance of future research to clarify and address these gaps.

References: 1. Constantinides VC, Paraskevas GP, Paraskevas PG, Stefanis L, Kapaki E. Corticobasal degeneration and corticobasal syndrome: A review. Clin Park Relat Disord. 2019;1:66-71. doi:10.1016/j.prdoa.2019.08.005
2. Armstrong MJ, Litvan I, Lang AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496-503. doi:10.1212/WNL.0b013e31827f0fd1

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MDS Abstracts - https://www.mdsabstracts.org/abstract/corticobasal-syndrome-a-single-center-retrospective-review/