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Severity score system for progressive myelopathy : development and validation of a new clinical scale

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Severity score system for progressive myelopathy : development and validation of a new clinical scale

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Título Severity score system for progressive myelopathy : development and validation of a new clinical scale
Autor Castilhos, Raphael Machado de
Blank, Deborah
Netto, Cristina Brinckmann Oliveira
Souza, Carolina Fischinger Moura de
Fernandes, Luiz Nelson Teixeira
Schwartz, Ida Vanessa Doederlein
Giugliani, Roberto
Jardim, Laura Bannach
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), was constructed 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.
Contido em Revista brasileira de pesquisas médicas e biológicas = Brazilian journal of medical and biological research. Ribeirão Preto, SP. Vol. 45, n. 7 (July 2012), p. 565-572
Assunto Mucolipidoses
Mucopolissacaridose
[en] Adrenomyeloneuropathy
[en] Japanese Orthopaedic Association
[en] Mucolipidosis
[en] Mucopolysaccharidosis
[en] Progressive myelopathies
[en] Severity Score System for Progressive Myelopathy
Origem Nacional
Tipo Artigo de periódico
URI http://hdl.handle.net/10183/87727
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