Clinical findings in MuSK-antibody positive myasthenia gravis

a U.S. experience

Mamatha Pasnoor, Gil I. Wolfe, Sharon Nations, Jaya Trivedi, Richard J. Barohn, Laura Herbelin, April McVey, Mazen Dimachkie, John Kissel, Ronan Walsh, Anthony Amato, Tahseen Mozaffar, Marcel Hungs, Luis Chui, Jonathan Goldstein, Steven Novella, Ted Burns, Lawrence Phillips, Gwendolyn Claussen, Angela Young & 2 others Tulio Bertorini, O. H. Shin

Research output: Contribution to journalArticle

113 Citations (Scopus)

Abstract

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 (≥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.

Original languageEnglish (US)
Pages (from-to)370-374
Number of pages5
JournalMuscle and Nerve
Volume41
Issue number3
DOIs
StatePublished - Mar 1 2010

Fingerprint

Myasthenia Gravis
Antibodies
Plasma Exchange
Intravenous Immunoglobulins
Cholinesterase Inhibitors
Adrenal Cortex Hormones
Phosphotransferases
Muscles
Thymectomy
Age of Onset
Tongue
Immunotherapy
Atrophy

All Science Journal Classification (ASJC) codes

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

Cite this

Pasnoor, M., Wolfe, G. I., Nations, S., Trivedi, J., Barohn, R. J., Herbelin, L., ... Shin, O. H. (2010). Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience. Muscle and Nerve, 41(3), 370-374. https://doi.org/10.1002/mus.21533

Clinical findings in MuSK-antibody positive myasthenia gravis : a U.S. experience. / Pasnoor, Mamatha; Wolfe, Gil I.; Nations, Sharon; Trivedi, Jaya; Barohn, Richard J.; Herbelin, Laura; McVey, April; Dimachkie, Mazen; Kissel, John; Walsh, Ronan; Amato, Anthony; Mozaffar, Tahseen; Hungs, Marcel; Chui, Luis; Goldstein, Jonathan; Novella, Steven; Burns, Ted; Phillips, Lawrence; Claussen, Gwendolyn; Young, Angela; Bertorini, Tulio; Shin, O. H.

In: Muscle and Nerve, Vol. 41, No. 3, 01.03.2010, p. 370-374.

Research output: Contribution to journalArticle

Pasnoor, M, Wolfe, GI, Nations, S, Trivedi, J, Barohn, RJ, Herbelin, L, McVey, A, Dimachkie, M, Kissel, J, Walsh, R, Amato, A, Mozaffar, T, Hungs, M, Chui, L, Goldstein, J, Novella, S, Burns, T, Phillips, L, Claussen, G, Young, A, Bertorini, T & Shin, OH 2010, 'Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience', Muscle and Nerve, vol. 41, no. 3, pp. 370-374. https://doi.org/10.1002/mus.21533
Pasnoor M, Wolfe GI, Nations S, Trivedi J, Barohn RJ, Herbelin L et al. Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience. Muscle and Nerve. 2010 Mar 1;41(3):370-374. https://doi.org/10.1002/mus.21533
Pasnoor, Mamatha ; Wolfe, Gil I. ; Nations, Sharon ; Trivedi, Jaya ; Barohn, Richard J. ; Herbelin, Laura ; McVey, April ; Dimachkie, Mazen ; Kissel, John ; Walsh, Ronan ; Amato, Anthony ; Mozaffar, Tahseen ; Hungs, Marcel ; Chui, Luis ; Goldstein, Jonathan ; Novella, Steven ; Burns, Ted ; Phillips, Lawrence ; Claussen, Gwendolyn ; Young, Angela ; Bertorini, Tulio ; Shin, O. H. / Clinical findings in MuSK-antibody positive myasthenia gravis : a U.S. experience. In: Muscle and Nerve. 2010 ; Vol. 41, No. 3. pp. 370-374.
@article{d8e211bac8dd4c34a779ae01e6304b67,
title = "Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience",
abstract = "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 (≥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.",
author = "Mamatha Pasnoor and Wolfe, {Gil I.} and Sharon Nations and Jaya Trivedi and Barohn, {Richard J.} and Laura Herbelin and April McVey and Mazen Dimachkie and John Kissel and Ronan Walsh and Anthony Amato and Tahseen Mozaffar and Marcel Hungs and Luis Chui and Jonathan Goldstein and Steven Novella and Ted Burns and Lawrence Phillips and Gwendolyn Claussen and Angela Young and Tulio Bertorini and Shin, {O. H.}",
year = "2010",
month = "3",
day = "1",
doi = "10.1002/mus.21533",
language = "English (US)",
volume = "41",
pages = "370--374",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "3",

}

TY - JOUR

T1 - Clinical findings in MuSK-antibody positive myasthenia gravis

T2 - a U.S. experience

AU - Pasnoor, Mamatha

AU - Wolfe, Gil I.

AU - Nations, Sharon

AU - Trivedi, Jaya

AU - Barohn, Richard J.

AU - Herbelin, Laura

AU - McVey, April

AU - Dimachkie, Mazen

AU - Kissel, John

AU - Walsh, Ronan

AU - Amato, Anthony

AU - Mozaffar, Tahseen

AU - Hungs, Marcel

AU - Chui, Luis

AU - Goldstein, Jonathan

AU - Novella, Steven

AU - Burns, Ted

AU - Phillips, Lawrence

AU - Claussen, Gwendolyn

AU - Young, Angela

AU - Bertorini, Tulio

AU - Shin, O. H.

PY - 2010/3/1

Y1 - 2010/3/1

N2 - 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 (≥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.

AB - 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 (≥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.

UR - http://www.scopus.com/inward/record.url?scp=77649169688&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77649169688&partnerID=8YFLogxK

U2 - 10.1002/mus.21533

DO - 10.1002/mus.21533

M3 - Article

VL - 41

SP - 370

EP - 374

JO - Muscle and Nerve

JF - Muscle and Nerve

SN - 0148-639X

IS - 3

ER -