Inclusion body myositis functional rating scale: A reliable and valid measure of disease severity

  title={Inclusion body myositis functional rating scale: A reliable and valid measure of disease severity},
  author={Carlayne E. Jackson and Richard J. Barohn and Gary S. Gronseth and S. Pandya and Laura L Herbelin},
  journal={Muscle \& Nerve},
We developed a disease‐specific, 10‐point functional rating scale for patients with inclusion body myositis (IBMFRS). The IBMFRS was utilized as a secondary outcome measure in a multicenter pilot trial of the clinical safety and tolerability of high‐dose beta interferon‐1a. In this trial, 28 IBM patients completed the IBMFRS at baseline and monthly for 6 months. The IBMFRS showed statistically significant correlations (P < 0.001) with maximal voluntary isometric contraction, manual muscle… 
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Longitudinal observational study investigating outcome measures for clinical trials in inclusion body myositis
Moves in muscle strength and physical function in patients with sporadic inclusion body myositis accurately predict disease progression in a reliable and useful way to be used in trial design.
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Demographic and clinical features of inclusion body myositis in north America
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Treatment of inclusion-body myositis with IVIg
Although the study did not establish efficacy of IVIg, possibly because of the small sample size, the drug induced functionally important improvement in 6 (28%) of the 19 patients, and whether the modest gains noted in certain muscle groups justify the high cost of trying IVIG in IBM patients at a given stage of the disease remains unclear.
Assessment of strength measurements
  • Eric J Sorensen
  • Medicine
    Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
  • 2004
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The ALSFRSr predicts survival time in an ALS clinic population
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