Corticobasal degeneration
From IDWiki
Background
- One of the Parkinson-plus syndromes
Clinical Manifestations
- Motor features: rigidity, bradykinesia or clumsy limb, postural instability, falls, abnormal gait, axial rigidity, tremor (including myoclonus that is mistaken tremor), limb dystonia, and myoclonus
- Higher cortical features: cognitive impairment, behavioural changes, limb apraxia, aphasia, depression, cortical sensory loss, and alien limb
- Other features: abnormal eye movements, hyperreflexia, speech changes
Diagnostic Criteria
- From 1
Proposed Phenotypes
| Syndrome | Features |
|---|---|
| Probable corticobasal syndrome | Asymmetric presentation of 2 of: limb rigidity or akinesia, limb dystonia, limb myoclonus; plus 2 of: orobuccal or limb apraxia, cortical sensory deficit, alien limb phenomena |
| Possible corticobasal syndrome | May be symmetric with 1 of: limb rigidity or akinesia, limb dystonia, limb myoclonus; plus 1 of: orobuccal or limb apraxia, cortical sensory deficit, alien limb phenomena |
| Frontal behavioural-spatial syndrome | 2 of: executive dysfunction, behavioural or personality changes, visuospatial defects |
| Nonfluent/agrammatic variant of primary progressive aphasia | Effortful, agrammatic speech plus at least 1 of: impaired grammar/sentence comprehension with relatively preserved single worse comprehension, or speech apraxia (groping, distorted speech production) |
| Progressive supranuclear palsy syndrome | 3 of: axial or symmetric limb rigidity or akinesia; postural instability or falls; urinary incontinence; behavioural changes; supranuclear vertical gaze palsy or decreased velocity of vertical saccades |
Diagnostic Criteria
| Feature | Clinica research criteria for probable sporadic CBD | Clinical criteria for possible CBD |
|---|---|---|
| Presentation | Insidious onset and gradual progression | Insidious onset and gradual progression |
| Age at onset (years) | ≥50 | No minimum |
| Family history of 2 or more relatives | Exclusion | Permitted |
| Permitted phenotypes |
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| Genetic mutation affecting tau | Exclusion | Permitted |
References
- ^ Melissa J. Armstrong, Irene Litvan, Anthony E. Lang, Thomas H. Bak, Kailash P. Bhatia, Barbara Borroni, Adam L. Boxer, Dennis W. Dickson, Murray Grossman, Mark Hallett, Keith A. Josephs, Andrew Kertesz, Suzee E. Lee, Bruce L. Miller, Stephen G. Reich, David E. Riley, Eduardo Tolosa, Alexander I. Tröster, Marie Vidailhet, William J. Weiner. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496-503. doi:10.1212/wnl.0b013e31827f0fd1.
