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NEUROLOGY 2008;71:486-492
© 2008 American Academy of Neurology

SCA Functional Index

A useful compound performance measure for spinocerebellar ataxia

T. Schmitz-Hübsch, MD, P. Giunti, MD, D. A. Stephenson, PhD, C. Globas, MD, L. Baliko, MD, F. Saccà, MD, C. Mariotti, MD, M. Rakowicz, MD, S. Szymanski, MD, J. Infante, MD, B.P.C. van de Warrenburg, MD, D. Timmann, MD, R. Fancellu, MD, R. Rola, MD, C. Depondt, MD, PhD, L. Schöls, MD, E. Zdzienicka, MD, J. -S. Kang, MD, S. Döhlinger, MD, B. Kremer, MD, B. Melegh, MD, PhD, A. Filla, MD and T. Klockgether, MD

From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Germany; Department of Molecular Neuroscience (P.G., D.A.S.), Institute of Neurology, Queen Square, London, UK; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Germany; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (F.S., A.F.), University of Naples, Italy; Department of Biochemistry and Genetics (C.M., R.F.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Clinical Neurophysiology (M.R.), Institute of Psychiatry and Neurology, Warsaw, Poland; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Germany; Department of Neurology (J.I.), University Hospital "Marqués de Valldecilla", CIBERNED, Santander, Spain; Department of Neurology (B.P.C.v.d.W., B.K.), Radboud University Nijmegen Medical Center, The Netherlands; Department of Neurology (D.T.), University of Duisburg-Essen, Essen, Germany; First Neurological Department (R.R.), Institute of Psychiatry and Neurology, Warsaw, Poland; Department of Neurology (C.D.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Genetics (E.Z.), Institute of Psychiatry and Neurology, Warsaw, Poland; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurodegeneration and Restorative Research (S.D.), Centers of Molecular Physiology of the Brain and Neurological Medicine, Medical University, Göttingen, Germany; and Department of Medical Genetics and Child Development (B.M.), University of Pécs, Faculty of Medicine, Hungary.

Address correspondence and reprint requests to Dr. Tanja Schmitz-Hübsch, Department of Neurology, University Hospital of Bonn, Sigmund-Freud-Str. 25, D-53105 Bonn, Germany tanja.schmitz-huebsch{at}ukb.uni-bonn.de.

Objective: To evaluate the usefulness of functional measures in patients with spinocerebellar ataxia (SCA).

Methods: We assessed three functional measures—8 m walking time (8MW), 9-hole peg test (9HPT), and PATA repetition rate—in 412 patients with autosomal dominant SCA (genotypes 1, 2, 3, and 6) in a multicenter trial.

Results: While PATA rate was normally distributed (mean/median 21.7/20.5 per 10 s), the performance times for 8MW (mean/median 10.8/7.5 s) or 9HPT (mean/median 47.2/35.0 s in dominant, 52.2/37.9 s in nondominant hand) were markedly skewed. Possible learning effects were small and likely clinically irrelevant. A composite functional index (SCAFI) was formed after appropriate transformation of subtest results. The Z-scores of each subtest correlated well with the Scale for the Assessment and Rating of Ataxia (SARA), the Unified Huntington's disease Rating Scale functional assessment, and disease duration. Correlations for SCAFI with each of these parameters were stronger (Pearson r = –0.441 to –0.869) than for each subtest alone. Furthermore, SCAFI showed a linear decline over the whole range of disease severity, while 9HPT and 8MW had floor effects with respect to SARA. Analysis of possible confounders showed no effect of genotype or study site and only minor effects of age for 8MW.

Conclusion: The proposed functional measures and their composite SCAFI have favorable properties to assess patients with spinocerebellar ataxia.

Abbreviations: 8MW = 8 m walking time; 9HPT = 9-hole peg test; FC = Functional Composite; FRDA = Friedreich ataxia; MS = multiple sclerosis; MSFC = Multiple Sclerosis Functional Composite; PASAT = Paced Auditory Serial Addition Test; SARA = Scale for the Assessment and Rating of Ataxia; SCA = spinocerebellar ataxia; UHDRS = Unified Huntington's Disease Rating Scale.

Supplemental data at www.neurology.org.

Supported by grants EUROSCA/LSHM-CT-2004-503304 from the European Union, GeneMove/01 GM 0503 from the German Ministry of Education and Research, and grant 3 PO5B 019 24 from the Polish Ministry of Scientific Research and Information Technology.

Disclosure: The authors report no disclosures.

Received October 31, 2007. Accepted in final form May 5, 2008.






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