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The cerebellar cognitive affective/Schmahmann syndrome scale
Franziska Hoche1, Xavier Guell1,2, Mark G Vangel3
1Ataxia Unit, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Brain : a Journal of Neurology
|December 6, 2017
Summary
A new 10-item scale effectively screens for Cerebellar Cognitive Affective Syndrome (CCAS) in patients with cerebellar disorders. This tool aids in rapid clinical assessment, identifying possible, probable, or definite CCAS with high accuracy.
Area of Science:
- Neuroscience
- Neurology
- Cognitive Science
Background:
- Cerebellar Cognitive Affective Syndrome (CCAS), also known as Schmahmann's syndrome, presents with complex cognitive and emotional deficits.
- Current diagnosis relies on extensive neuropsychological testing, limiting its bedside application.
- A need exists for a brief screening tool to identify CCAS in clinical settings.
Purpose of the Study:
- To develop and validate a brief, office-based cognitive screening instrument for Cerebellar Cognitive Affective Syndrome (CCAS).
- To assess the utility of existing brief cognitive tests in detecting CCAS.
- To explore cognitive differences between isolated cerebellar and cerebrocerebellar pathologies and identify red flags for extra-cerebellar involvement.
Main Methods:
- Administered comprehensive neuropsychological tests, experimental measures, and rating scales to 77 cerebellar patients and healthy controls.
- Developed a 10-item screening scale based on tests differentiating patients from controls.
- Validated the scale in a separate cohort of 39 cerebellar patients and 55 healthy controls.
Main Results:
- The developed 10-item CCAS/Schmahmann scale demonstrated high sensitivity and selectivity in both exploratory and validation cohorts.
- Executive functions (working memory, flexibility, reasoning), language (verbal fluency), and visuospatial skills were significantly impaired in CCAS patients.
- Patients with complex cerebrocerebellar disease showed greater impairment on similarities tests; word recall deficits suggested extra-cerebellar pathology.
Conclusions:
- The CCAS/Schmahmann scale is a valuable tool for the expedited clinical assessment of Cerebellar Cognitive Affective Syndrome.
- The scale effectively identifies patients with possible, probable, and definite CCAS.
- Findings support the distinct cognitive profile associated with cerebellar dysfunction and highlight potential indicators of broader neurological involvement.
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