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G.P.13.06 Sporadic inclusion body myositis (sIBM): A phenotypic spectrum in a cohort of 57 cases
Journal article   Peer reviewed

G.P.13.06 Sporadic inclusion body myositis (sIBM): A phenotypic spectrum in a cohort of 57 cases

M. Needham, A. Corbett, T. Day and F. Mastaglia
Neuromuscular Disorders, Vol.17(9-10)
2007
DOI: https://doi.org/10.1016/j.nmd.2007.06.298
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Abstract

Inclusion body myositis is the most important myopathy associated with aging and usually presents after the age of 50 years. There have been few studies of large cohorts of sIBM patients. We have performed a cross-sectional study on a cohort of 57 biopsy-proven patients who fulfilled the diagnostic criteria for definite (44) or probable (13) sIBM, to determine the degree of variability in the clinical phenotype and mode of presentation of the condition. The mean duration of disease was 9.5 years (range 1–23 years). Males were more often affected than females (1.4:1) and the average age of onset was 60.5 years (range 40–82 years); 10.5% presented before the age of 50 years. In 79% of cases the first symptoms were due to quadriceps weakness, and 35.1% presented only after starting to fall; 12% presented because of hand weakness, 7% because of foot drop and 1.8% because of dysphagia. Although the initial sequence of muscle involvement was variable, most patients eventually developed weakness of all these limb muscle groups, and 58% developed dysphagia. Asymmetric involvement, particularly in the upper limbs, was common (82%), with the non-dominant side being more severely affected in 91% of cases. Contractures of the fingers and toes were present in 73% of cases. Although falls occurred at some point in 93% of cases, only 7% of patients were wheelchair-bound and many remained relatively independent until late in the course of the disease. This was confirmed by relatively low scores on a self-reported functional status score (Stanford Health Assessment Questionnaire). However 63% of patients reported symptoms of depression, indicating a disparity between their functional status and perception of well-being. The findings in this large cohort of cases further emphasises the variable phenotype and patterns of presentation of sIBM and the problem of late presentation in over a third of cases.

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