Objective: To compare the clinicopathologic features of corticobasal syndrome (CBS) associated with corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), Alzheimer’s disease (AD), and frontotemporal lobar degeneration (FTLD) with TDP-43 inclusions (FTLD-TDP).
 pathology may, in part, contribute to CBS in PSP.

Background: Corticobasal syndrome (CBS) is characterized by asymmetric rigidity, apraxia, dystonia, myoclonus, alien limb phenomena, and cortical sensory deficits. This syndrome is associated with various neurodegenerative disorders, such as CBD, PSP, AD, and FTLD-TDP. It is challenging to predict the underlying pathology of patients with corticobasal syndrome on the basis of clinical features.

Method: We reviewed medical records of patients who had a clinical diagnosis of CBS regardless of the pathologic diagnosis in the Mayo Clinic brain bank collected between 1998 and 2021. Armstrong’s criteria for CBD were retrospectively applied to identify patients with possible or probable CBS. We determined the frequency of underlying neuropathology of CBS and compared the clinicopathologic features among the underlying pathology. Alzheimer-type pathology (Braak neurofibrillary tangle stage and Thal amyloid phase) was assessed in all patients. As comparison groups of PSP and CBD, the MDS criteria for PSP was applied to identify patients who had probable Richardson syndrome (RS).

Results: In total, 345 patients were clinically diagnosed with CBS who met criteria for possible or probable CBS. Of those, 122 patients with CBD, 88 patients with PSP, 51 patients with AD, and 5 patients with FTLD-TDP fulfilled the criteria for possible corticobasal syndrome. Only 26 CBD, 7 PSP, 12 AD, and 1 FTLD-TDP met criteria for probable CBS. The age at symptomatic onset was youngest in AD, followed by CBD, FTLD-TDP, and PSP (59 vs. 64 vs. 68 vs. 70 years; p<0.001). In CBD, the age at death did not differ between CBD presenting as CBS (CBD-CBS) and those presenting as RS (CBD-RS). In contrast, PSP presenting as CBD (PSP-CBS) was significantly older (77 vs. 74 years old; p=0.002) and had a higher Braak neurofibrillary tangle stage (2.6 vs. 2.2; p = 0.014) than those presenting as RS (PSP-RS).

Conclusion: The young age at symptomatic onset suggests AD as the underlying pathology of CBS. Concomitant Alzheimer-type pathology may, in part, contribute to CBS in PSP.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinicopathologic-features-of-corticobasal-syndrome-associated-with-corticobasal-degeneration-progressive-supranuclear-palsy-alzheimers-disease-and-ftld-tdp/