2012
DOI: 10.1590/s0100-879x2012007500072
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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 cover… Show more

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Cited by 22 publications
(19 citation statements)
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References 28 publications
(36 reference statements)
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“…Nissenkorn et al 132 developed a clinical global impression scale for ataxia-telangiectasia, which includes sections on motor function. For progressive myelopathy, including mucopolysaccharidosis types I and VI, Castilhos et al 133 developed and validated the Severity Score System for Progressive Myelopathy. Darling et al 24 designed and pilot-tested the PKAN-Disease Rating Scale, which includes a motor examination protocol for infantile neuroaxonal dystrophy.…”
Section: Resultsmentioning
confidence: 99%
“…Nissenkorn et al 132 developed a clinical global impression scale for ataxia-telangiectasia, which includes sections on motor function. For progressive myelopathy, including mucopolysaccharidosis types I and VI, Castilhos et al 133 developed and validated the Severity Score System for Progressive Myelopathy. Darling et al 24 designed and pilot-tested the PKAN-Disease Rating Scale, which includes a motor examination protocol for infantile neuroaxonal dystrophy.…”
Section: Resultsmentioning
confidence: 99%
“…The EDSS, originally designed to assess disability in multiple sclerosis but also widely used in ALD, measures neurological disability ranging from 0 (no disability) to 10 (death) ( Kurtzke, 1983 ; Moser et al, 2004 ; Zackowski et al, 2006 ; Castellano et al, 2016 ; Huffnagel et al, 2019b ). SSPROM measures severity of myelopathy ranging from 0 to 100, with lower scores indicating a higher degree of impairment ( Castilhos et al, 2012 ; D’Souza et al, 2017 ). The 6MWT measures the maximum walking distance in 6 min and was performed on a 50-m flat indoor trail ( van Hedel et al, 2005 ).…”
Section: Methodsmentioning
confidence: 99%
“…22 SSPROM measures the severity of myelopathy ranging from 0 to 100, with lower scores indicating a higher degree of impairment. 23,24 The timed up-and-go is used to assess walking function by recording the time that the patient needs to get up from an armchair, walk 3 meters, turn around, walk back, and sit again. 25,26 Sample processing and laboratory methods Blood was collected in 4-mL EDTA tubes and processed within 2 hours at the biobank of Amsterdam UMC.…”
Section: Assessment Of Myelopathymentioning
confidence: 99%