Corticobasal syndrome

Corticobasal syndrome (CBS) is a progressive neurodegenerative disease and a type of frontotemporal dementia.[1] Corticobasal syndrome is one of four clinical phenotypes of corticobasal degeneration (CBD), the other three being: frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS).[2]

CBD is the pathology underlying approximately 50% of CBS cases.[3] Other degenerative pathologies that can cause corticobasal syndrome include: Alzheimer’s disease, Pick’s disease with Pick bodies, Lewy body dementias, neurofilament inclusion body disease, Creutzfeldt-Jakob disease, frontotemporal degeneration due to progranulin gene mutation and motor neuron disease‐inclusion dementia.[4]

Symptoms

Symptoms of CBS include apraxia, alien limb phenomenon, frontal deficits, and extrapyramidal motor symptoms such as myoclonus or rigidity.[1]

The symptoms of classic CBS differ from CBD in that CBD additionally features cognitive deficits in the executive functions.[5]

References

  1. 1 2 Finger EC (April 2016). "Frontotemporal Dementias". Continuum (Minneap Minn) (Review). 22 (2 Dementia): 464–89. doi:10.1212/CON.0000000000000300. PMC 5390934. PMID 27042904.
  2. ↑ Armstrong MJ, Litvan I, Lang AE, et al. (January 2013). "Criteria for the diagnosis of corticobasal degeneration". Neurology (Multicenter study). 80 (5): 496–503. doi:10.1212/WNL.0b013e31827f0fd1. PMC 3590050. PMID 23359374.
  3. ↑ Gomperts SN (April 2016). "Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia". Continuum (Minneap Minn) (Review). 22 (2 Dementia): 435–63. doi:10.1212/CON.0000000000000309. PMC 5390937. PMID 27042903.
  4. ↑ Hassan A, Whitwell JL, Josephs KA (November 2011). "The corticobasal syndrome-Alzheimer's disease conundrum". Expert Rev Neurother (Review). 11 (11): 1569–78. doi:10.1586/ern.11.153. PMC 3232678. PMID 22014136.
  5. ↑ Fredericks CA, Lee SE (2016). "The cognitive neurology of corticobasal degeneration and progressive supranuclear palsy". In Miller, Bruce L.; Boeve, Bradley F. The Behavioral Neurology of Dementia (Second ed.). Cambridge, United Kingdom: Cambridge University Press. pp. 203–6. ISBN 9781107077201. OCLC 934020279. [CBD is] reminiscent of classic CBS but with executive function deficits
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