2011/06/14 by Jeffrey T. Guptill, Donald B. Sanders, Amelia Evoli · 3 citations
Medicine · #Autoimmune Neurological Disorders and Treatments #Myasthenia Gravis and Thymoma #Peripheral Neuropathies and Disorders
paper · doi:10.1002/mus.22006
openalex publication_date 2011/06/14 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/29
INTRODUCTION: Myasthenia gravis (MG) patients with autoantibodies to muscle-specific tyrosine kinase (MuSK) represent a distinct subset of those with this disease. Treatment and outcomes data in these patients are limited and conflicting. METHODS: We reviewed 110 MuSK-MG patients from two large clinics in Italy and the USA. RESULTS: Thirty-nine to 49% of patients with generalized, acetylcholine receptor antibody (AChR-Ab)-negative MG had MuSK-MG. Eighty-five percent were female, with disease onset typically in the fourth decade. Ocular and/or bulbar symptoms were present at onset in 79% of those studied. Eighty-five percent were MGFA class III or greater, and crisis occurred in 28%. Plasma exchange (PLEX) produced improvement in 93%, whereas only 61% improved after intravenous immunoglobulin. Long-term outcomes were comparable to those of patients with AChR-Ab-positive MG. CONCLUSIONS: MuSK-MG has a marked female predominance with frequent oculobulbar weakness and crises. Many patients deteriorate rapidly early in the disease, and PLEX is usually the preferred treatment. Long-term outcomes are similar to those of patients with AChR-Ab(+) MG.