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Measuring disease progression in corticobasal syndrome
Nancy Huang1, Michael Hornberger, John R Hodges
1Neuroscience Research Australia, Barker Street, Randwick, Sydney, NSW 2031, Australia.
Journal of Neurology
|June 5, 2014
Summary
Corticobasal syndrome (CBS) progression can be identified by specific clinical signs. Motor speech disorders and dysgraphia suggest slower progression, while surface dyslexia indicates faster CBS progression.
Area of Science:
- Neuroscience
- Neurology
Background:
- Corticobasal syndrome (CBS) is a heterogeneous neurodegenerative disorder.
- Disease progression measurement in CBS is challenging and poorly understood.
Purpose of the Study:
- To identify clinical and neuropsychological indicators of prognosis in Corticobasal syndrome.
- To differentiate between rapid and slow progressing CBS patients.
Main Methods:
- Retrospective analysis of CBS patients from a specialized clinic.
- Detailed clinical and neuropsychological assessments, including the Frontotemporal Dementia Rating Scale (FRS).
- Categorization into rapid and slow progressor groups based on FRS score changes over 12 months.
Main Results:
- No significant baseline differences in demographics, motor/cognitive presentation, or ACE-R scores between progressor groups.
- Slow progressors were more likely to have motor speech disorders and dysgraphia.
- Rapid progressors showed a higher incidence of surface dyslexia.
- No significant differences in overall neuropsychological performance between groups.
Conclusions:
- Motor speech disorder, dysgraphia, and surface dyslexia may help distinguish between rapid and slow CBS progression.
- These clinical features offer potential prognostic value in Corticobasal syndrome management.
