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Published on: December 18, 2016
Symmetric corticobasal degeneration (S-CBD)
Anhar Hassan1, Jennifer L Whitwell, Bradley F Boeve
1Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Parkinsonism & Related Disorders
|December 19, 2009
Summary
Corticobasal degeneration (CBD) can present symmetrically, differing from typical corticobasal syndrome (CBS). Symmetric CBD cases show less atrophy and may lack classic CBS symptoms like apraxia.
Area of Science:
- Neuroscience
- Neuropathology
- Radiology
Background:
- Corticobasal degeneration (CBD) is a neurodegenerative disease characterized by neuronal loss, gliosis, and tau deposition.
- Typical corticobasal syndrome (CBS) presents with asymmetric rigidity and apraxia, with asymmetry also noted on neuroimaging.
Purpose of the Study:
- To identify and describe cases of CBD with symmetric clinical and radiological features.
- To compare the clinical and imaging characteristics of symmetric CBD (S-CBD) with typical CBS cases.
Main Methods:
- Reviewed pathologically confirmed CBD cases from the Mayo Clinic Rochester database.
- Performed quantitative volumetric analysis of head MRI for symmetric atrophy using atlas-based parcellation.
- Classified cases as S-CBD if no right- vs. left-sided differences in cortical or extrapyramidal signs were observed.
Main Results:
- Identified five cases meeting S-CBD criteria; these cases lacked limb dystonia, myoclonus, apraxia, or alien limb phenomena.
- S-CBD cases exhibited significantly less asymmetric atrophy compared to typical CBS cases (p=0.009).
- S-CBD cases were younger at onset and death and had a family history in 40%.
Conclusions:
- Corticobasal degeneration can manifest with symmetric clinical and radiological features.
- Symmetric CBD presentations may lack hallmark CBS symptoms such as limb apraxia, myoclonus, dystonia, and alien limb phenomenon.
