Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience.
We performed a retrospective chart review on 53 muscle-specific kinase antibody (MuSK-Ab)-positive myasthenia gravis (MG) patients at nine university-based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9-79 years. Twenty-seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long-term (> or =3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG-related death. This survey reinforces several cardinal features of MuSK-Ab-positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long-term outcome is favorable in about 60% of cases.
Pasnoor
Mamatha
M
University of Kansas Medical Center, 3901 Rainbow Boulevard, Mail Stop 2012, Kansas City, Kansas 66160, USA. mpasnoor@kumc.edu
Wolfe
Gil I
GI
Nations
Sharon
S
Trivedi
Jaya
J
Barohn
Richard J
RJ
Herbelin
Laura
L
McVey
April
A
Dimachkie
Mazen
M
Kissel
John
J
Walsh
Ronan
R
Amato
Anthony
A
Mozaffar
Tahseen
T
Hungs
Marcel
M
Chui
Luis
L
Goldstein
Jonathan
J
Novella
Steven
S
Burns
Ted
T
Phillips
Lawrence
L
Claussen
Gwendolyn
G
Young
Angela
A
Bertorini
Tulio
T
Oh
Shin
S
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002648:Child; D018572:Disease-Free Survival; D004576:Electromyography; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007167:Immunotherapy; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000276:immunology* / Q000628:therapy*; D010951:Plasma Exchange; D011241:Prednisone / Q000627:therapeutic use; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology*; D011950:Receptors, Cholinergic / Q000276:immunology*; D012074:Remission Induction; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013934:Thymectomy; D016896:Treatment Outcome; D014481:United States
D016756:Immunoglobulins, Intravenous; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases; D011241:Prednisone