CBS is the most common type of corticobasal degeneration (CBD) although the terms CBD and CBS have been used interchangeably in the past.[2] The other three phenotypes of CBD are:[1]
Symptoms of CBS include apraxia, alien limb phenomenon, frontal deficits, and extrapyramidal motor symptoms such as myoclonus or rigidity.[5] Movement deficits often begin on one side and progress to the other.[1]
Pathophysiologyedit
CBD is the pathology underlying approximately 50% of CBS cases.[6]
Diagnosisedit
The Armstrong criteria were proposed in 2013; the accuracy of these is limited and further research is needed.[7] Symptoms may be symmetric or asymmetric, with one or more of the following:[citation needed]
limb rigidity or akinesia
limb dystonia
limb myoclonus, plus one of:
orobuccal or limb apraxia
cortical sensory deficit
alien limb phenomena (more than simple levitation)
The onset is insidious with gradual progression, lasting one year or more, with no exclusion criteria present. The diagnosis is more likely if onset is after age 50, there is no family history (2 or more relatives),[clarification needed] and there is no genetic mutation affecting T[clarification needed] (e.g., MAPT).[8]
Probably sporadic CBS is more likely if there are two of:
limb rigidity or akinesia
limb dystonia
limb myoclonus
plus two of:
orobuccal or limb apraxia,
cortical sensory deficit
alien limb phenomena (more than simple levitation)[8]
The diagnosis is excluded if there is evidence of:
^ abcConstantinides VC, Paraskevas GP, Paraskevas PG, Stefanis L, Kapaki E (August 2019). "Corticobasal degeneration and corticobasal syndrome: A review". Clinical Parkinsonism & Related Disorders. 1: 66–71. doi:10.1016/j.prdoa.2019.08.005. ISSN 2590-1125. PMC8288513. PMID 34316603.
^Di Stasio F, Suppa A, Marsili L, et al. (May 2019). "Corticobasal syndrome: neuroimaging and neurophysiological advances". Eur. J. Neurol. 26 (5): 701–e52. doi:10.1111/ene.13928. hdl:11573/1225619. PMID 30720235. S2CID 73426149.
^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. PMC3590050. PMID 23359374.
^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. PMC5390937. PMID 27042903.
^ abcShimohata T, Aiba I, Nishizawa M (2016). "[Diagnoses of corticobasal syndrome and corticobasal degeneration]". Rinsho Shinkeigaku (in Japanese). 56 (3): 149–57. doi:10.5692/clinicalneurol.cn-000841. PMID 26876110.
^ abcAlexander SK, Rittman T, Xuereb JH, Bak TH, Hodges JR, Rowe JB (August 2014). "Validation of the new consensus criteria for the diagnosis of corticobasal degeneration". J. Neurol. Neurosurg. Psychiatry. 85 (8): 925–29. doi:10.1136/jnnp-2013-307035. PMC4112495. PMID 24521567.
^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. PMC3232678. PMID 22014136.
^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