TY - JOUR
TI - MuSK-Ab positive myasthenia: Not always grave
AU - Zouvelou, V.
AU - Stamboulis, E.
AU - Skriapa, L.
AU - Tzartos, S.J.
JO - Journal of the Neurological Sciences
PY - 2013
VL - 331
TODO - 1-2
SP - 150-151
PB - Elsevier B.V.
SN - 0022-510X
TODO - 10.1016/j.jns.2013.05.001
TODO - corticosteroid;  immunoglobulin G antibody;  immunoglobulin g4 antibody;  muscle specific tyrosine kinase antibody;  prednisolone;  protein antibody;  protein tyrosine kinase;  pyridostigmine;  unclassified drug, adult;  anamnesis;  brain electrophysiology;  case report;  cesarean section;  clinical observation;  computer assisted tomography;  corticosteroid therapy;  diplopia;  disease course;  disease exacerbation;  drug dose increase;  drug withdrawal;  dysarthria;  fasciculation;  female;  follow up;  histopathology;  human;  immunotherapy;  laboratory test;  low drug dose;  muscle cramp;  myasthenia gravis;  neurologic examination;  phenotype;  physical examination;  pregnant woman;  priority journal;  ptosis;  respiratory distress;  short survey;  symptom;  thymectomy;  thymus
TODO - Antibodies (Abs) to muscle-specific tyrosine kinase (MuSK) are detected in approximately 40% of generalized acetylcholine receptor antibody-negative myasthenia gravis (MG). Anti-MuSK Abs are nearly always associated with generalized symptoms, with prevalent involvement of craniobulbar, cervical and respiratory muscles and with a striking preponderance in women. The typical course of MuSK-MG is acute onset, rapid progression, brittle course in the first years, early respiratory crises and unprovoked relapses in spite of high-dose immunosuppression. Patients often require long-term management with multiple immunosuppressive (IS) agents and many of them remain dependent on IS treatment. The majority of anti-MuSK Abs are of the non-complement-binding IgG4 subclass. We report the case of a Greek female MuSK-MG patient with typical phenotype but clearly atypical clinical course during 12 years of follow-up. The patient received only corticosteroid treatment for one year and showed mild and stable MG symptoms under no treatment for the rest of the observation period, except for postpartum mild and short-lived exacerbation. In mildly symptomatic stable state, anti-MuSK Abs were of the IgG4 subclass and no other immunological peculiarity was detected. © 2013 Published by Elsevier B.V.
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