TY - JOUR
T1 - Electrodiagnostic Characteristics Suggestive of Muscle-Specific Kinase Myasthenia Gravis
AU - Skolka, Michael
AU - Lamb, Christopher J.
AU - Rubin, Devon I.
AU - Klein, Christopher J.
AU - Laughlin, Ruple S.
N1 - Publisher Copyright:
© American Academy of Neurology.
PY - 2022/6/1
Y1 - 2022/6/1
N2 - Background and ObjectivesMuscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MuSK + MG) is a form of MG with bulbar-predominant symptoms often resistant to conventional treatments. Patients with MuSK + MG may have an electrodiagnostic (EDX) profile distinct from other MG. This study compares EDX features of MuSK + MG with acetylcholine receptor (AChR) antibody-positive MG (AChR + MG) to discern whether any unique EDX pattern exists that can aid in clinical diagnosis.MethodsFrom January 1, 2010, through December 31, 2020, all patients with MuSK + MG at our institution were identified and randomly matched to an AChR + MG cohort in a 1:2 ratio based on sex, age at onset, and subsequently Myasthenia Gravis Foundation of America (MGFA) clinical severity for a case-control study. Each patient's clinical profile, treatment, and EDX testing were summarized and analyzed.ResultsTwenty-two patients with MuSK + MG (18 female) and 44 patients with AChR + MG were studied. The average symptom duration at presentation was shorter in the MuSK + MG group (4.7 years) compared with AChR + MG (10.9 years). Myotonic discharges were rare in both groups but more frequently observed in patients with MuSK + MG (10%) identified in 5 muscles in 2 patients compared with AChR + MG (2%) noted in only 1 muscle in 1 patient. Patients with MuSK + MG more often had myopathic appearing motor unit potentials (MUPs) (41% vs 30%) compared with AChR + MG. Myopathic appearing MUPs were found in milder cases of MuSK + MG (MGFA class I-IIB) compared with AChR + MG (MGFA Class IIB-V).DiscussionPatients with MuSK + MG may have a recognizable EDX profile from AchR + MG that includes (1) myotonic discharges, (2) greater occurrence of myopathic appearing MUPs in clinically mild disease, and (3) symptoms leading to earlier testing.
AB - Background and ObjectivesMuscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MuSK + MG) is a form of MG with bulbar-predominant symptoms often resistant to conventional treatments. Patients with MuSK + MG may have an electrodiagnostic (EDX) profile distinct from other MG. This study compares EDX features of MuSK + MG with acetylcholine receptor (AChR) antibody-positive MG (AChR + MG) to discern whether any unique EDX pattern exists that can aid in clinical diagnosis.MethodsFrom January 1, 2010, through December 31, 2020, all patients with MuSK + MG at our institution were identified and randomly matched to an AChR + MG cohort in a 1:2 ratio based on sex, age at onset, and subsequently Myasthenia Gravis Foundation of America (MGFA) clinical severity for a case-control study. Each patient's clinical profile, treatment, and EDX testing were summarized and analyzed.ResultsTwenty-two patients with MuSK + MG (18 female) and 44 patients with AChR + MG were studied. The average symptom duration at presentation was shorter in the MuSK + MG group (4.7 years) compared with AChR + MG (10.9 years). Myotonic discharges were rare in both groups but more frequently observed in patients with MuSK + MG (10%) identified in 5 muscles in 2 patients compared with AChR + MG (2%) noted in only 1 muscle in 1 patient. Patients with MuSK + MG more often had myopathic appearing motor unit potentials (MUPs) (41% vs 30%) compared with AChR + MG. Myopathic appearing MUPs were found in milder cases of MuSK + MG (MGFA class I-IIB) compared with AChR + MG (MGFA Class IIB-V).DiscussionPatients with MuSK + MG may have a recognizable EDX profile from AchR + MG that includes (1) myotonic discharges, (2) greater occurrence of myopathic appearing MUPs in clinically mild disease, and (3) symptoms leading to earlier testing.
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U2 - 10.1212/CPJ.0000000000001166
DO - 10.1212/CPJ.0000000000001166
M3 - Article
AN - SCOPUS:85132211976
SN - 2163-0402
VL - 12
SP - 211
EP - 217
JO - Neurology: Clinical Practice
JF - Neurology: Clinical Practice
IS - 3
ER -