Is the Pathology of Corticobasal Syndrome Predictable in Life?

Prince of Wales Medical Research Institute, Barker Street, Randwick, New South Wales, Australia
Movement Disorders (Impact Factor: 5.68). 08/2009; 24(11):1593 - 1599. DOI: 10.1002/mds.22558
Source: PubMed


Corticobasal syndrome (CBS) has been associated with a heterogeneous spectrum of pathologies with an increasing number of reports of Alzheimer's type pathology. There is, however, no means of predicting pathology of CBS in vivo at present. We compared the clinical features of patients presenting with CBS who have either pathologic changes of classic corticobasal degeneration (CBD) or Alzheimer's disease (AD) at post-mortem to identify predictors of the specific pathological processes in life. Twelve patients with CBS were followed prospectively; six had AD and six had classic CBD neuropathology. After review of the presenting clinical features, we identified nine potential predictor variables, compared their frequency in the two groups, and performed a discriminant function analysis. Initial episodic memory complaints and poor performance on the combined orientation-memory subtest of the Addenbrooke's Cognitive Examination (ACE) reliably predicted AD pathology while varying combinations of early frontal-lobe type behavioral symptoms, nonfluent language disturbance, orobuccal apraxia, and utilization behavior predicted CBD pathology ante-mortem. CBS is frequently associated with Alzheimer's disease pathology. Early episodic memory impairment versus early behavioral symptomatology appears to best predict AD or CBD pathology in life. © 2009 Movement Disorder Society

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Available from: Thomas H Bak, Dec 19, 2013
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    • "The bulk of these observations suggest that most phenotypes are in general poorly predictive of the underlying pathology (Josephs et al. 2006b). CBS can include a diverse, non-CBD range of neuropathologies including PSP and most notably AD (Shelley et al. 2009), particularly when episodic memory complaints are prominent (see Bradley Boeve's, " The multiple phenotypes of corticobasal syndrome and corticobasal degeneration, " this issue). "
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    • "So far, normal dopamine transporter density has been reported only sporadically in CBS [19], [45], while none of the few systematic imaging studies in consecutive cohorts ever documented any normal scan [40]–. To our knowledge, there are only few reports of patients with clinical diagnosis of CBD with preserved SNc neuronal density at post-mortem, and most of them displayed either Alzheimer's or Pick's disease pathology [2], [3], [5], [7], [9], [10], [14], [15], [46]–[48]. The first possible explanation would come from a possible pathological involvement of the downstream postsynaptic neurons, even though the pathological involvement of postsynaptic striatal neurons in CBD is variable and unpredictable [17], [49]. "
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    • "It is less common, however, for subjects presenting with CBS or bvFTD to show underlying AD pathology (Johnson et al., 1999; Alladi et al., 2007; Hu et al., 2009; Shelley et al., 2009), and neither syndrome is typically associated with predominant temporoparietal atrophy. Corticobasal syndrome is characterized by insidious onset and a progressive course of asymmetric cortical dysfunction, for example, ideomotor apraxia, cortical sensory loss or myoclonus, and asymmetric extrapyramidal dysfunction reflected by at least levodopa unresponsive appendicular rigidity or dystonia (Boeve et al., 2003; Litvan et al., 2003). "
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