Autophagic myopathies / myofibrillar myopathies / distal myopathies / pompe disease

Immune-mediated necrotizing myopathy (IMNM) belongs to the group of idopathic inflammatory myopathies. Affected patients present with severe proximal lower-limb-predominant muscle weakness and strong elevation of the creatine kinase. The presence of anti-HMGCR- or anti-SRP- autoantibodies or the absence of known myositis specific antibodies is diagnostically essential and divides IMNM into three subgroups. Treatment with immune modulators can be effective, but escalation of therapy is often necessary.

Source: Neuromuscular Disorders - September 28, 2020 Category: Neurology Authors: N. Fischer, C. Preusse, Y. Allenbach, O. Benveniste, A. Roos, H. Goebel, W. Stenzel Source Type: research