Disease Directory Corticobasal Degeneration
Neurological

Corticobasal Degeneration

Also known as: CBD, corticobasal syndrome, CBS, corticobasal ganglionic degeneration, 4R tauopathy

Prevalence

1-9 per 100,000 (Orphanet)

Onset

Adult

Type

Sporadic (MAPT risk haplotype)

Gene

MAPT (risk haplotype, GRN rarely)

About Corticobasal Degeneration

Corticobasal degeneration is a rare 4-repeat tauopathy causing neuronal loss and tau accumulation in the cortex and basal ganglia. The corticobasal syndrome (CBS) clinical presentation includes asymmetric limb rigidity and apraxia, the alien limb phenomenon, cortical sensory loss, myoclonus, and dystonia. CBD is pathologically defined at autopsy; clinically it overlaps with PSP, frontotemporal dementia, and Parkinson's disease. Disease progression is relentless with no approved therapies.

Common Clinical Features

Asymmetric limb rigidity Apraxia Alien limb phenomenon Cortical sensory loss Myoclonus Dystonia Cognitive decline

Clinical Trial Eligibility Tips

What to know before applying to Corticobasal Degeneration trials.

Probable CBS diagnosis per Armstrong criteria is required — symptom asymmetry documentation is key

FDG-PET or MRI showing asymmetric cortical and basal ganglia changes supports eligibility confirmation

CSF and blood biomarkers (NfL, 4R-tau, phospho-tau) are used in trial stratification

Anti-tau therapeutic trials (same pipeline as PSP) may accept CBS as a phenotypic variant — confirm if the trial is pathology-defined or syndrome-defined

Patient Resources

Patient Organization

CurePSP

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Natural History Registry

CurePSP Patient Registry

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Orphanet

European reference resource for rare diseases (ORPHA:37596)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Corticobasal Degeneration trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Corticobasal Degeneration. Updated daily.

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