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Muscle Nerve. 2011 Jul;44(1):36-40. doi: 10.1002/mus.22006.

Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts.

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1
Department of Medicine, Duke University Medical Center, Durham, North Carolina, USA.

Abstract

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.

PMID:
21674519
DOI:
10.1002/mus.22006
[Indexed for MEDLINE]
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