Literature DB >> 16894103

The Spastic Paraplegia Rating Scale (SPRS): a reliable and valid measure of disease severity.

R Schüle1, T Holland-Letz, S Klimpe, J Kassubek, T Klopstock, V Mall, S Otto, B Winner, L Schöls.   

Abstract

OBJECTIVE: To develop and evaluate a clinical Spastic Paraplegia Rating Scale (SPRS) to measure disease severity and progression.
METHODS: A 13-item scale was designed to rate functional impairment occurring in pure forms of spastic paraplegia (SP). Additional symptoms constituting a complicated form of SP are recorded in an inventory. Two independent patient cohorts were evaluated in a two-step validation procedure.
RESULTS: Application of SPRS requires less than 15 minutes and does not require any special equipment, so it is suitable for an outpatient setting. Interrater agreement of SPRS was high (intraclass correlation coefficient = 0.99). Reliability was further supported by high internal consistency (Cronbach alpha = 0.91). SPRS values were almost normally distributed without apparent floor or ceiling effect. Construct validity was shown by high correlation of SPRS to Barthel Index and the International Cooperative Ataxia Rating Scale (convergent validity) and low correlation to Mini-Mental Status Examination (discriminant validity).
CONCLUSION: The Spastic Paraplegia Rating Scale is a reliable and valid measure of disease severity.

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Year:  2006        PMID: 16894103     DOI: 10.1212/01.wnl.0000228242.53336.90

Source DB:  PubMed          Journal:  Neurology        ISSN: 0028-3878            Impact factor:   9.910


  81 in total

1.  Characterization of Alu and recombination-associated motifs mediating a large homozygous SPG7 gene rearrangement causing hereditary spastic paraplegia.

Authors:  Eva López; Carlos Casasnovas; Javier Giménez; Antoni Matilla-Dueñas; Ivelisse Sánchez; Víctor Volpini
Journal:  Neurogenetics       Date:  2014-11-16       Impact factor: 2.660

2.  FAHN/SPG35: a narrow phenotypic spectrum across disease classifications.

Authors:  Tim W Rattay; Tobias Lindig; Jonathan Baets; Katrien Smets; Tine Deconinck; Anne S Söhn; Konstanze Hörtnagel; Kathrin N Eckstein; Sarah Wiethoff; Jennifer Reichbauer; Marion Döbler-Neumann; Ingeborg Krägeloh-Mann; Michaela Auer-Grumbach; Barbara Plecko; Alexander Münchau; Bernd Wilken; Marc Janauschek; Anne-Katrin Giese; Jan L De Bleecker; Els Ortibus; Martine Debyser; Adolfo Lopez de Munain; Aurora Pujol; Maria Teresa Bassi; Maria Grazia D'Angelo; Peter De Jonghe; Stephan Züchner; Peter Bauer; Ludger Schöls; Rebecca Schüle
Journal:  Brain       Date:  2019-06-01       Impact factor: 13.501

3.  Pigmentary degenerative maculopathy as prominent phenotype in an Italian SPG56/CYP2U1 family.

Authors:  Luca Leonardi; Lucia Ziccardi; Christian Marcotulli; Anna Rubegni; Antonino Longobardi; Mariano Serrao; Eugenia Storti; Francesco Pierelli; Alessandra Tessa; Vincenzo Parisi; Filippo M Santorelli; Casali Carlo
Journal:  J Neurol       Date:  2016-02-25       Impact factor: 4.849

4.  Late-onset hereditary spastic paraplegia with thin corpus callosum caused by a new SPG3A mutation.

Authors:  Antonio Orlacchio; Pasqua Montieri; Carla Babalini; Fabrizio Gaudiello; Giorgio Bernardi; Toshitaka Kawarai
Journal:  J Neurol       Date:  2011-02-19       Impact factor: 4.849

5.  A new double-trouble phenotype: fascioscapulohumeral muscular dystrophy ameliorates hereditary spastic paraparesis due to spastin mutation.

Authors:  Marina Scarlato; Arturo Nuara; Simonetta Gerevini; Sara Benedetti; Paolo Rossi; Maurizio Ferrari; Stefano Carlo Previtali
Journal:  J Neurol       Date:  2014-12-16       Impact factor: 4.849

6.  Hereditary spastic paraplegia type 5: a potentially treatable disorder of cholesterol metabolism.

Authors:  Andrea Mignarri; Alessandro Malandrini; Marina Del Puppo; Alessandro Magni; Lucia Monti; Federica Ginanneschi; Alessandra Tessa; Filippo Maria Santorelli; Antonio Federico; Maria Teresa Dotti
Journal:  J Neurol       Date:  2014-02-08       Impact factor: 4.849

7.  Early-onset optic neuropathy as initial clinical presentation in SPG7.

Authors:  Christian Marcotulli; Luca Leonardi; Alessandra Tessa; Anna Maria De Negris; Riccardo Cornia; Alberto Pierallini; Shalom Haggiag; Francesco Pierelli; Filippo M Santorelli; Carlo Casali
Journal:  J Neurol       Date:  2014-07-18       Impact factor: 4.849

8.  Analysis of CYP7B1 in non-consanguineous cases of hereditary spastic paraplegia.

Authors:  Rebecca Schüle; Elisabeth Brandt; Kathrin N Karle; Maria Tsaousidou; Stephan Klebe; Sven Klimpe; Michaela Auer-Grumbach; Andrew H Crosby; Christian A Hübner; Ludger Schöls; Thomas Deufel; Christian Beetz
Journal:  Neurogenetics       Date:  2008-10-15       Impact factor: 2.660

9.  Three novel mutations in 20 patients with hereditary spastic paraparesis.

Authors:  Mehmet Bugrahan Duz; Selcuk Dasdemir; Aysel Kalayci Yigin; Mehmet Ali Akalin; Mehmet Seven
Journal:  Neurol Sci       Date:  2018-06-16       Impact factor: 3.307

10.  Identification of a heterozygous genomic deletion in the spatacsin gene in SPG11 patients using high-resolution comparative genomic hybridization.

Authors:  Peter Bauer; Beate Winner; Rebecca Schüle; Claudia Bauer; Veronika Häfele; Ute Hehr; Michael Bonin; Michael Walter; Kathrin Karle; Thomas M Ringer; Olaf Riess; Jürgen Winkler; Ludger Schöls
Journal:  Neurogenetics       Date:  2008-09-12       Impact factor: 2.660

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