Muscle & nerve · 2014
Neuromuscular junction disorders mimicking myopathy.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
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Mongiovi Phillip C, Elsheikh Bakri, Lawson Victoria H, Kissel John T, Arnold W David
Аннотация
INTRODUCTION: Small-amplitude, short-duration motor unit action potentials are non-specific findings seen in myopathies and neuromuscular junction (NMJ) disorders. NMJ studies (repetitive nerve stimulation and single-fiber electromyography) can determine if such findings are related to NMJ abnormalities but are not considered routinely in atypical cases.
METHODS: Medical records of 338 patients with confirmed NMJ disorders were reviewed to identify cases with a clinical or electrodiagnostic impression of myopathy during initial evaluation. A history of muscle biopsy with findings that did not support a myopathic process was required for inclusion.
RESULTS: Four patients met the inclusion criteria. NMJ studies were abnormal in all cases. One patient had elevated acetylcholine receptor antibodies. Three patients were antibody negative: 2 demonstrated immunotherapy responsiveness, and 1 had a Rapsyn mutation.
CONCLUSIONS: NMJ disorders may mimic myopathies, and NMJ studies should be performed to clarify so-called "myopathic" electromyographic findings to avoid unnecessary testing and delayed diagnosis.
METHODS: Medical records of 338 patients with confirmed NMJ disorders were reviewed to identify cases with a clinical or electrodiagnostic impression of myopathy during initial evaluation. A history of muscle biopsy with findings that did not support a myopathic process was required for inclusion.
RESULTS: Four patients met the inclusion criteria. NMJ studies were abnormal in all cases. One patient had elevated acetylcholine receptor antibodies. Three patients were antibody negative: 2 demonstrated immunotherapy responsiveness, and 1 had a Rapsyn mutation.
CONCLUSIONS: NMJ disorders may mimic myopathies, and NMJ studies should be performed to clarify so-called "myopathic" electromyographic findings to avoid unnecessary testing and delayed diagnosis.