Sir, The recent Brain publication (Dalakas et al., 2009) describing a clinical study of alemtuzumab in patients with inclusion body myositis nicely demonstrates the feasibility of enrolling and following a cohort of these patients over a long period of time as participants in an intervention study. The authors state that ‘in [inclusion body myositis] one series of alemtuzumab infusions can slow...

There is now compelling evidence that sporadic inclusion body myositis (sIBM) is a muscle-specific autoimmune disease in which both T and B-cells play a part and in which both cytotoxic muscle fibre necrosis and degeneration occur. However the factors responsible for breakdown of immune tolerance and the nature of the target antigens expressed by muscle fibres remain unknown. Genetic factors ar...

Inclusion body myositis (IBM) is a slowly progressive skeletal muscle disease for which no effective pharmacological therapy is available. Since its initial pathological description 50 years ago, substantial progress has been made in our clinical understanding of IBM. We now have extensive understanding of its demographics, pattern of muscle involvement, and diagnostic criteria, and large amoun...

Inclusion body myositis is a rare myopathy that clinically resembles a chronic polymyositis and histopathologically is characterized by the presence of rimmed vacuoles containing ultrastructural cytoplasmic degradation products with filamentous intranuclear and cytoplasmic inclusions. Since clinical features are not uniform, histopathologic and ultrastructural studies are necessary to confirm t...

Inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy occurring in patients over the age of 50 years and probably accounts for about 30% of all inflammatory myopathies. Muscle biopsy characteristically reveals endomysial inflammation, small groups of atrophic fibres, eosinophilic cytoplasmic inclusions and muscle fibres with one or more rimmed vacuoles. However, any ...

myopathy affecting proximal and distal musculature with typical onset of symptoms after age 50 years. Inclusion body myositis more often affects males and results in a classical pattern of early asymmetric weakness and wasting of forearm flexors (wrist and long finger flexors), quadriceps and ankle dorsiflexors. Inclusion body myositis is more frequently associated with a monoclonal gammopathy ...

PURPOSE OF REVIEW The pathogenesis of sporadic inclusion body myositis is complex and the disease has a relentless course. Recent observations regarding possible mechanisms of disease may provide targets for therapy. RECENT FINDINGS Evidence is strengthening that specific T-cell and B-cell responses are ongoing in skeletal muscle in sporadic inclusion body myositis and that cytokines and chem...

OBJECTIVE To describe the clinical and electrophysiologic features of patients with inclusion body myositis that was misinterpreted as motor neuron disease. PATIENTS AND METHODS We retrospectively retrieved the medical records of 70 patients with a pathologic diagnosis of inclusion body myositis. From this group, we selected those who had been first diagnosed as having motor neuron disease or...

Sporadic inclusion body myositis (IBM) is an acquired muscle disorder associated with ageing, for which there is no effective treatment. Ongoing developments include: genetic studies that may provide insights regarding the pathogenesis of IBM, improved histopathological markers, the description of a new IBM autoantibody, scrutiny of the diagnostic utility of clinical features and biomarkers, th...