Is the pathology of corticobasal syndrome predictable in life?

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 presentin...

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Bibliographic Details
Published in:Movement disorders 2009-08, Vol.24 (11), p.1593-1599
Main Authors: Shelley, Bhaskara P., Hodges, John R., Kipps, Christopher M., Xuereb, John H., Bak, Thomas H.
Format: Article
Language:English
Subjects:
Aged
Alzheimer Disease - complications
Alzheimer Disease - diagnosis
Alzheimer Disease - pathology
Alzheimer's disease
Aphasia - etiology
Aphasia - pathology
Apraxias - etiology
Apraxias - pathology
Autopsy
behavior
Biological and medical sciences
Brain - pathology
Cognition Disorders - etiology
Cognition Disorders - pathology
corticobasal degeneration
corticobasal syndrome
Diagnosis, Differential
Disease Progression
Female
Follow-Up Studies
Humans
Male
Malformations of the nervous system
Medical sciences
Memory Disorders - etiology
Memory Disorders - pathology
Middle Aged
Neurology
Neuropsychological Tests
nonfluent aphasia
pathology
Predictive Value of Tests
Syndrome
Tauopathies - complications
Tauopathies - diagnosis
Tauopathies - pathology
utilization behavior
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Summary: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
ISSN:0885-3185
1531-8257
DOI:10.1002/mds.22558