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Severity score system for progressive myelopathy: development and validation of a new clinical scale
Castilhos, R.M.; Blank, D.; Netto, C.B.O.; Souza, C.F.M.; Fernandes, L.N.T.; Schwartz, I.V.D.; Giugliani, R.; Jardim, L.B..
  • Castilhos, R.M.; Programa de Pós-graduação em Ciências Médicas. Porto Alegre. BR
  • Blank, D.; Hospital de Clínicas de Porto Alegre. Serviço de Genética Médica. Porto Alegre. BR
  • Netto, C.B.O.; Hospital de Clínicas de Porto Alegre. Serviço de Genética Médica. Porto Alegre. BR
  • Souza, C.F.M.; Hospital de Clínicas de Porto Alegre. Serviço de Genética Médica. Porto Alegre. BR
  • Fernandes, L.N.T.; Universidade Federal do Rio Grande do Sul. Departamento de Medicina Interna. Porto Alegre. BR
  • Schwartz, I.V.D.; Programa de Pós-graduação em Ciências Médicas. Porto Alegre. BR
  • Giugliani, R.; Programa de Pós-graduação em Ciências Médicas. Porto Alegre. BR
  • Jardim, L.B.; Programa de Pós-graduação em Ciências Médicas. Porto Alegre. BR
Braz. j. med. biol. res ; 45(7): 565-572, July 2012. ilus, tab
Article in English | LILACS | ID: lil-639465
ABSTRACT
Progressive myelopathies can be secondary to inborn errors of metabolism (IEM) such as mucopolysaccharidosis, mucolipidosis, and adrenomyeloneuropathy. The available scale, Japanese Orthopaedic Association (JOA) score, was validated only for degenerative vertebral diseases. Our objective is to propose and validate a new scale addressing progressive myelopathies and to present validating data for JOA in these diseases. A new scale, Severity Score System for Progressive Myelopathy (SSPROM), covering motor disability, sphincter dysfunction, spasticity, and sensory losses. Inter- and intra-rater reliabilities were measured. External validation was tested by applying JOA, the Expanded Disability Status Scale (EDSS), the Barthel index, and the Osame Motor Disability Score. Thirty-eight patients, 17 with adrenomyeloneuropathy, 3 with mucopolysaccharidosis I, 3 with mucopolysaccharidosis IV, 2 with mucopolysaccharidosis VI, 2 with mucolipidosis, and 11 with human T-cell lymphotropic virus type-1 (HTLV-1)-associated myelopathy participated in the study. The mean ± SD SSPROM and JOA scores were 74.6 ± 11.4 and 12.4 ± 2.3, respectively. Construct validity for SSPROM (JOA r = 0.84, P < 0.0001; EDSS r = -0.83, P < 0.0001; Barthel r = 0.56, P < 0.002; Osame r = -0.94, P < 0.0001) and reliability (intra-rater r = 0.83, P < 0.0001; inter-rater r = 0.94, P < 0.0001) were demonstrated. The metric properties of JOA were similar to those found in SSPROM. Several clinimetric requirements were met for both SSPROM and JOA scales. Since SSPROM has a wider range, it should be useful for follow-up studies on IEM myelopathies.
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Full text: Available Index: LILACS (Americas) Main subject: Spinal Cord Diseases / Severity of Illness Index / Disability Evaluation Type of study: Etiology study / Observational study / Prognostic study / Risk factors Limits: Adolescent / Adult / Child / Female / Humans / Male Language: English Journal: Braz. j. med. biol. res Journal subject: Biology / Medicine Year: 2012 Type: Article Affiliation country: Brazil Institution/Affiliation country: Hospital de Clínicas de Porto Alegre/BR / Programa de Pós-graduação em Ciências Médicas/BR / Universidade Federal do Rio Grande do Sul/BR

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Full text: Available Index: LILACS (Americas) Main subject: Spinal Cord Diseases / Severity of Illness Index / Disability Evaluation Type of study: Etiology study / Observational study / Prognostic study / Risk factors Limits: Adolescent / Adult / Child / Female / Humans / Male Language: English Journal: Braz. j. med. biol. res Journal subject: Biology / Medicine Year: 2012 Type: Article Affiliation country: Brazil Institution/Affiliation country: Hospital de Clínicas de Porto Alegre/BR / Programa de Pós-graduação em Ciências Médicas/BR / Universidade Federal do Rio Grande do Sul/BR