Disease progression in sporadic inclusion body myositis: Observations in 78 patients

Peng, A; Koffman, Boyd M.; Malley, James D.; Dalakas, Marinos C.

doi:10.1212/wnl.55.2.296

Cited by 103 publications

(75 citation statements)

References 9 publications

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“…This is in contrast to the common conception in neuromuscular disorders that tend to be more disabling with earlier onset such as in dystrophinopathies and spinal muscular atrophy. A previous study also suggested faster progression to disability in patients with onset beyond 60 years of age [17]. As age of onset is an independent predictor of progression this finding needs to be taken into account in the design of future treatment trials.…”

Section: Discussionmentioning

confidence: 90%

Inclusion body myositis

et al. 2005

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The clinical features of inclusion body myositis (IBM) were of minor importance in the design of consensus diagnostic criteria, mainly because of controversial views on the specificity of signs and symptoms, although some authors reported "typical" signs. To re-assess the clinical spectrum of IBM, a single investigator using a standard protocol studied a cohort of 64 patients cross-sectionally. Symptom onset was before the age of 50 years in 20% of cases. Only a few patients (14 %) started with weakness other than that of quadriceps, finger flexor or pharyngeal muscles. The sequence of power loss was erratic, but onset of symptoms with quadriceps weakness predicted an earlier onset of dysphagia in older patients (> or = 56 years) compared with younger ones (< 56 years) (p = 0.02). Despite widespread weakness patients had favourable scores on three commonly used function scales and they kept their employment. Complete wheel-chair dependency was rare (3 %). A dominant characteristic was the anatomical distribution of afflicted muscles: ventral extremity muscle groups were more affected than dorsal muscle groups and girdle muscles were least affected, the latter preserving postural stability. Ankylosis, especially in extension of the fingers,was frequently present. Together with the sparing of intrinsic hand muscles it was helpful in the preservation of many skillful movements. IBM has a unique distribution of muscle weakness. Ankylotic contractures are common. We feel that their joint impact on daily functioning is characteristic for the disease.

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Section: Discussionmentioning

confidence: 90%

Inclusion body myositis

et al. 2005

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“…Once diagnosed, treatment is usually of the supportive nature rather than curative because IBM does not respond to typical immunosuppressive treatments [3,8,9]. Current treatments aim to slow the rate of progression of the disease rather than cure it [5,10]. True treatment efficacy is difficult to gauge, however, because of the relatively slow progression, rate of progression differing between patients, and the fact that older patients tend to decline more rapidly, although the reason for this is not entirely clear [10,11].…”

Section: Introductionmentioning

confidence: 99%

“…Strength usually decreases 5-10% per year, although rates of progression can vary [3,10,12]. Yet most patients remain ambulatory for many years after diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Gait patterns of patients with inclusion body myositis

Bernhardt

Kaufman

2011

Gait & Posture

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“…The amyotrophy can be asymmetric, and in typical cases muscle weakness and wasting are most profound in knee extensors, hip flexors and long finger flexors 8 . Most patients require an assistive device within several years of onset 9 . Neck flexors and extensors and facial muscle are frequently affected 8 .…”

Section: Discussionmentioning

confidence: 99%