RESULTS We found that none of the 19 instruments have sufficiently comprehensive reporting of measurement or feasibility performance as would be required by regulatory authorities .
INTRODUCTION In an accompanying study we reported on the content of several activity rating scales that have been used for muscle disease .
To further aid in achieving consensus we conducted a systematic review to assess the quality of the 19 activity rating scales  design ed specifically for muscle disease .
High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study
Abstract Sporadic inclusion body myositis ( s-IBM ) is an acquired inflammatory muscle disease of unknown cause . In general , s-IBM presents with slowly progressive , asymmetric weakness , and atrophy of skeletal muscle . There is a mild transitory or nil responsiveness to st and ard immunosuppressive treatment . A controlled cross-over study of ¶22 s-IBM patients over 3 months showed a partial improvement in those treated with high-dose intravenous immunoglobulin therapy ( IVIG ) versus placebo . The present study included 22 patients aged 32–75 years and with a mean duration of disease of 5.2 ± 3.6 years . They were r and omized by a double-blind , placebo-controlled , cross-over design to monthly infusions of ¶2 g/kg bodyweight IVIG or to placebo for 6 months each , followed by the alternative treatment . After 6 and 12 months the response to treatment was evaluated , using a modified Medical Research Council scale , Neuromuscular Symptom Score ( NSS ) , the patient ’s own assessment of improvement , arm outstretched time , and electromyography . No serious side effects were seen , in particular no viral infection and no major cardiac or neurological complications . Overall there was no progression of the disease in 90 % of patients , unlike that which might have been expected in untreated patients . A mild and significant improvement ( 11 % ) in clinical symptoms was found using NSS , but not with other test procedures . There was a trend to mild improvement in treated patients when using other tests . Individual responses to treatment was heterogeneous . The validity of this study may be reduced by mismatch of groups with regard to age at onset and variability in disease expression . The findings of this study largely confirm those of a previous IVIG trial . Treatment with IVIG may be mildly effective in s-IBM by preventing disease progression or inducing mild improvement . Long-term studies are needed to evaluate further the benefit of IVIG therapy in s-IBM
RESULTS We found that none of the 19 instruments have sufficiently comprehensive reporting of measurement or feasibility performance as would be required by regulatory authorities .
INTRODUCTION In an accompanying study we reported on the content of several activity rating scales that have been used for muscle disease .
To further aid in achieving consensus we conducted a systematic review to assess the quality of the 19 activity rating scales  design ed specifically for muscle disease .