Objective: To compare clinical features and natural course of corticobasal degeneration (CBD) presenting as corticobasal syndrome (CBD-CBS) and progressive supranuclear palsy presenting as corticobasal syndrome (PSP-CBS).

Background: Background pathologies of corticobasal syndrome (CBS) vary. Its most frequent cause is CBD, followed by Alzheimer’s disease, progressive supranuclear palsy, and other proteinopathies, making correct diagnosis of CBD before death difficult.

Method: Patients with pathologically verified CBD-CBS and PSP-CBS were evaluated clinically, and their data were analyzed retrospectively to compare the frequency of symptoms and signs of CBD-CBS and PSP-CBS. The interval from initial symptoms to key clinical milestones was compared using Kaplan–Meier and log-rank tests. Sensitivity and specificity of Armstrong’s criteria were analyzed.

Results: Twelve CBD-CBS patients and 8 PSP-CBS patients were identified. All patients showed similar age at death (71.9 vs. 75.0 years, p > 0.05) and disease duration (8.3 vs. 9.5 years, p > 0.05). Common core features of CBS included rigidity and dystonia, but intensity of the alien limb sign and myoclonus was low in both groups. Apraxia and cortical sensory loss were observed more frequently in PSP-CBS patients than in CBD-CBS patients. Apart from CBS, common symptoms included executive dysfunction, general cognitive impairment, and postural instability and falls in both groups. Urinary incontinence was more common in CBD-CBS patients, whereas supranuclear gaze palsy and slow vertical saccades were more common in PSP-CBS patients. Sensitivities of probable sporadic and possible CBD as per Armstrong’s criteria were 50% and 70%, respectively, and specificities were 75% and 29%, respectively. The intervals from initial symptoms to key clinical milestones were similar in both groups; however, the interval to gait with assistance was significantly shorter in CBD-CBS patients.

Conclusion: Urinary incontinence was more common in CBD-CBS patients, whereas supranuclear gaze palsy and slow vertical saccades were more common in PSP-CBS patients. Despite similar disease duration, CBD-CBS patients showed a more rapid worsening of mobility than PSP-CBS patients.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-features-and-natural-course-of-corticobasal-degeneration-and-progressive-supranuclear-palsy-presenting-as-corticobasal-syndrome/