Clinical aspects of MuSK antibody positive seronegative MG.
Serum antibodies to muscle-specific receptor tyrosine kinase were detected in 12 of 32 patients with generalized seronegative MG. All were women, with onset between ages 21 and 59 years. Seven had prominent neck, shoulder, or respiratory muscle weakness and little or delayed ocular muscle involvement. The response to cholinesterase inhibitors was variable, and electromyographic findings suggested myopathy in several. None improved after thymectomy. All patients improved after plasma exchange, and most had a good response to selected immunotherapy. MuSK antibody status should help diagnose MG with atypical presentations and ensure appropriate patient treatment.
Sanders
D B
DB
Division of Neurology, Duke University Medical Center, Durham, NC 27710, USA. Donald.Sanders@Duke.edu
El-Salem
K
K
Massey
J M
JM
McConville
J
J
Vincent
A
A
D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D003131:Combined Modality Therapy; D004576:Electromyography; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000145:classification / Q000276:immunology* / Q000628:therapy; D009431:Neural Conduction; D009928:Organ Specificity; D010951:Plasma Exchange; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology*; D011950:Receptors, Cholinergic / Q000276:immunology*; D016037:Single-Blind Method; D013934:Thymectomy; D016896:Treatment Outcome
D001323:Autoantibodies; D001324:Autoantigens; D007166:Immunosuppressive Agents; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases