Kirzinger, Lukas and Khomenko, Andrei and Schulte-Mattler, Wilhelm and Backhaus, Roland and Platen, Sabine and Schalke, Berthold (2016) Myopathy in Childhood Muscle-Specific Kinase Myasthenia Gravis. PEDIATRIC NEUROLOGY, 65. pp. 90-92. ISSN 0887-8994, 1873-5150
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BACKGROUND: Adult and pediatric patients suffering from MuSK (muscle-specific kinase) -antibody positive myasthenia gravis exhibit similar features to individuals with acetylcholine receptor (AChR) antibodies, but they differ in several characteristics such as a predominant bulbar, respiratory and neck weakness, a generally worse disease severity and a tendency to develop muscle atrophy. Muscle atrophy is a rare phenomenon that is usually restricted to the facial muscles. RESULTS: We describe a girl with MuSK-antibody positive myasthenia gravis who developed a myopathy with severe generalized muscular weakness, muscle atrophy, and myopathic changes on electromyography. CONCLUSION: This is the first published example of a generalized myopathic syndrome in myasthenia gravis. We review the relevant literature and discuss the hypothesis of a mitochondrial myopathy as a pathogenic mechanism in MuSK-antibody positive myasthenia gravis.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | MUSK; ANTIBODIES; AUTOANTIBODIES; childhood myasthenia; autoimmunity; MuSK; myasthenia gravis; myopathy |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 10 Apr 2019 08:16 |
| Last Modified: | 10 Apr 2019 08:16 |
| URI: | https://pred.uni-regensburg.de/id/eprint/3853 |
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