cbd tauopathy

Extended Data Figure 8.. Seeded tau aggregation induced by CBD filaments in SH-SY5Y cells.

Extended Data Figure 6.. CBD tau filament…

Extended Data Figure 3.. Assembled TDP-43 in…

Extended Data Figure 7.. Protofilament interface in CBD Type II tau filaments.

Conflict of interest statement

Figure 1.. Filamentous tau pathology of CBD.

Extended Data Figure 7.. Protofilament interface in…

Extended Data Figure 2.. Immunolabelling of tau…

Figures

(a-f), Staining of neuronal inclusions, neuropil threads and…

Extended Data Figure 2.. Immunolabelling of tau filaments extracted from additional brain regions of CBD…

Corticobasal degeneration (CBD) is a rare, progressive neurodegenerative disorder with onset in the 5 th to 7 th decade of life. It is associated with heterogeneous motor, sensory, behavioral and cognitive symptoms, which make its diagnosis difficult in a living patient. The etiology of CBD is unknown; however, neuropathological and genetic evidence supports a pathogenetic role for microtubule-associated protein tau. CBD pathology is characterized by circumscribed cortical atrophy with spongiosis and ballooned neurons; the distribution of these changes dictates the patient’s clinical presentation. Neuronal and glial tau pathology is extensive in gray and white matter of the cortex, basal ganglia, diencephalon and rostral brainstem. Abnormal tau accumulation within astrocytes forms pathognomonic astrocytic plaques. The classic clinical presentation, termed corticobasal syndrome (CBS), comprises asymmetric progressive rigidity and apraxia with limb dystonia and myoclonus. CBS also occurs in conjunction with other diseases, including Alzheimer disease and progressive supranuclear palsy. Moreover, the pathology of CBD is associated with clinical presentations other than CBS, including Richardson syndrome, behavioral variant frontotemporal dementia, primary progressive aphasia and posterior cortical syndrome. Progress in biomarker development to differentiate CBD from other disorders has been slow, but is essential in improving diagnosis and in development of disease-modifying therapies.

Key Points

The most common clinical presentation of CBD is asymmetric progressive rigidity and apraxia with limb dystonia and myoclonus, termed corticobasal syndrome

CBD pathology can be found in patients with Richardson syndrome (the most common clinical presentation of progressive supranuclear palsy), behavioral variant frontotemporal dementia and primary progressive aphasia