Clinical Scales of Cerebellar Ataxias
The development, evaluation, and validation of new clinical scales for the assessment of disease severity and progression in cerebellar disease have gained increasing impact during the last years. Currently, there are four different scales that are most f
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Clinical Scales of Cerebellar Ataxias
80
€rk Katrin Bu
Abstract
The development, evaluation, and validation of new clinical scales for the assessment of disease severity and progression in cerebellar disease have gained increasing impact during the last years. Currently, there are four different scales that are most frequently applied: The International Cooperative Ataxia Rating Scale (ICARS), the Brief Ataxia Rating Scale (BARS), the Friedreich Ataxia Rating Scale (FARS), and the Scale for the Assessment and Rating of Ataxia (SARA). All scales have been validated and compared with regard to their testing properties. It has been shown that each scale has its strengths and weaknesses. It is therefore important to carefully select the clinical scale when preparing a clinical or therapeutic study. It is also important to note that the quality of a rating scale is not only defined by its interrater reliability and construct validity but also by its user friendliness. A scale’s compactness guarantees attractiveness to the clinician, reduces expenditure of time and money, and will thereby alleviate further clinical trials. Large, elaborated scales are certainly useful for thorough documentation of specific features of certain phenotypes, but this gain of information is not always essential for the purpose of a study. It is therefore no surprise that short and manageable scales like SARA are often preferred to more elaborated scales in observational and therapeutic studies.
K. B€urk Department of Neurology, Philipps University of Marburg, Baldingerstrasse, Marburg, 35043, Germany e-mail: [email protected] M. Manto, D.L. Gruol, J.D. Schmahmann, N. Koibuchi, F. Rossi (eds.), 1785 Handbook of the Cerebellum and Cerebellar Disorders, DOI 10.1007/978-94-007-1333-8_80, # Springer Science+Business Media Dordrecht 2013
1786
K. B€ urk
Introduction The development, evaluation, and validation of new clinical scales for the assessment of disease severity and progression in cerebellar disease have gained increasing impact during the last years. Such scales should also serve as outcome measures in therapeutic trials. It is very likely that future therapeutic interventions rather slow progression than reverse morbidity. Therefore, thorough evaluation of clinical scales is essential to ensure that trials are efficient and their conclusions are accurate. There are various types of degenerative ataxias that share the common feature of cerebellar dysfunction but differ in the underlying neurodegenerative pattern. Friedreich ataxia (FA), e.g., is primarily affecting dorsal root ganglia and spinal tracts while spinocerebellar ataxias mainly involve the cerebellum and brainstem. In addition, the clinical phenotype and the age of onset may vary considerably even in the same disease: In FA, there are childhood onset cases while spinocerebellar ataxia (SCA) may become symptomatic in nonagenarians. Furthermore, extracerebellar involvement has a more pronounced impact on disability than cerebellar dysfunction itself. There are seve
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