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, progressive supranuclear palsy, 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, visuospatial deficits, acalculia, bradykinesia, dystonia, and extrapyramidal motor symptoms such as myoclonus or rigidity.[1] Movement deficits often begin on one side and progress to the other.

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

Also, intoxication with Annonaceae is believed to cause atypical forms of corticobasal syndromes.

References
  1. 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. (eds.). 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
This article is issued from Wikipedia. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.