An Empirical Comparison of Commonly Used Universal Rating Scales for Dystonia

Author:

Boz Deniz1,Kilic‐Berkmen Gamze2,Perlmutter Joel S.3,Norris Scott A.4,Wright Laura J.5,Klein Christine6,Bäumer Tobias6,Löns Sebastian6,Feuerstein Jeanne S.7,Mahajan Abhimanyu8,Wagle‐Shukla Aparna9,Malaty Irene9,LeDoux Mark S.10,Ondo William11,Pantelyat Alexander12,Frank Samuel13,Saunders‐Pullman Rachel14,Jinnah H.A.21516

Affiliation:

1. College of Arts & Sciences Georgia State University Atlanta Georgia USA

2. Department of Neurology Emory University School of Medicine Atlanta Georgia USA

3. Department of Neurology, Radiology, Neuroscience, Physical Therapy and Occupational Therapy Washington University School of Medicine St Louis Missouri USA

4. Department of Neurology and Radiology Washington University St Louis Missouri USA

5. Department of Neurology at Washington University at St Louis St Louis Missouri USA

6. Institute of Neurogenetics University of Lübeck Lübeck Germany

7. Department of Neurology University of Colorado Aurora Colorado USA

8. Rush Parkinson's disease and Movement disorders program, Department of Neurological Sciences Chicago Illinois USA

9. Fixel Institute for Neurological Disease, University of Florida Department of Neurology University of Florida Gainesville Florida USA

10. Department of Psychology University of Memphis, Memphis, TN, USA; Veracity Neuroscience LLC Memphis Tennessee USA

11. Department of Neurology, Methodist Neurological Institute Houston TX and Weill Cornell Medical School New York New York USA

12. Department of Neurology Johns Hopkins University School of Medicine Baltimore Maryland USA

13. Beth Israel Deaconess Medical Center Harvard Medical School Boston Massachusetts USA

14. Department of Neurology Icahn School of Medicine at Mount Sinai, and Mount Sinai Beth Israel New York New York USA

15. Department of Human Genetics Emory University School of Medicine Atlanta Georgia USA

16. Department of Pediatrics Emory University School of Medicine Atlanta Georgia USA

Abstract

ABSTRACTBackgroundThere are several widely used clinical rating scales for documenting the severity and distribution of various types of dystonia.ObjectivesThe goal of this study was to evaluate the performance of the most commonly used scales in a large group of adults with the most common types of isolated dystonia.MethodsGlobal Dystonia Rating Scale (GDRS) and the Burke‐Fahn‐Marsden Dystonia Rating Scale (BFM) scores were obtained for 3067 participants. Most had focal or segmental dystonia, with smaller numbers of multifocal or generalized dystonia. These scales were also compared for 209 adults with cervical dystonia that had Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores and 210 adults with blepharospasm that had Blepharospasm Severity Scale (BSRS) scores.ResultsThere were strong correlations between the GDRS and BFM total scores (r = 0.79) and moderate correlations for their sub scores (r > 0.5). Scores for both scales showed positive skew, with an overabundance of low scores. BFM sub‐scores were not normally distributed, due to artifacts caused by the provoking factor. Relevant sub‐scores of the GDRS and BFM also showed moderate correlations with the TWSTRS (r > 0.5) for cervical dystonia and the BSRS (r > 0.5) for blepharospasm.ConclusionsThe BFM is more widely used than the GDRS, but these results suggest the GDRS may be preferable for focal and segmental dystonias. The overabundance of very low scores for both scales highlights challenges associated with discriminating very mild dystonia from other abnormal movements or variants of normal behavior.

Funder

National Center for Advancing Translational Sciences

National Institute of Neurological Disorders and Stroke

Publisher

Wiley

Subject

Neurology (clinical),Neurology

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