OBJECTIVES: To determine if myasthenia gravis (MG) with antibodies to MuSK is a distinct subgroup of seronegative MG. METHODS: We assayed antibodies to muscle specific tyrosine kinase (MuSK) in 55 MG patients who had no antibodies to acetylcholine receptors and looked for the specific phenotype, comparing clinical features of anti-MuSK positive and anti-MuSK negative MG patients. RESULTS: MG with anti-MuSK antibodies was characterised by a striking prevalence of female patients (15 women, two men). Age at onset ranged from 22 to 52 years, with 70.6% of patients presenting at < 40 years of age. The majority of patients (82.4%) had prevalent involvement of facial and bulbar muscles. One third of them did not respond well to anticholinesterase drugs. Steroid immunosuppression was effective in eight patients (44.4%). Nine patients underwent thymectomy; six of these had no thymus pathology, while three had a hyperplastic thymus. At the end of the observation period, six (35.3%) patients were in remission, five (29.4%) improved, four (23.6%) did not change, and two (11.7%) had died. CONCLUSIONS: MG patients with antibodies to MuSK have characteristic clinical features that are different from features of the remaining seronegative MG patients. This emphasises the predictive value of anti-MuSK antibody analysis in seronegative MG patients.
OBJECTIVES: To determine if myasthenia gravis (MG) with antibodies to MuSK is a distinct subgroup of seronegative MG. METHODS: We assayed antibodies to muscle specific tyrosine kinase (MuSK) in 55 MGpatients who had no antibodies to acetylcholine receptors and looked for the specific phenotype, comparing clinical features of anti-MuSK positive and anti-MuSK negative MGpatients. RESULTS:MG with anti-MuSK antibodies was characterised by a striking prevalence of female patients (15 women, two men). Age at onset ranged from 22 to 52 years, with 70.6% of patients presenting at < 40 years of age. The majority of patients (82.4%) had prevalent involvement of facial and bulbar muscles. One third of them did not respond well to anticholinesterase drugs. Steroid immunosuppression was effective in eight patients (44.4%). Nine patients underwent thymectomy; six of these had no thymus pathology, while three had a hyperplastic thymus. At the end of the observation period, six (35.3%) patients were in remission, five (29.4%) improved, four (23.6%) did not change, and two (11.7%) had died. CONCLUSIONS:MGpatients with antibodies to MuSK have characteristic clinical features that are different from features of the remaining seronegative MGpatients. This emphasises the predictive value of anti-MuSK antibody analysis in seronegative MGpatients.
Authors: Lan Zhou; John McConville; Vinay Chaudhry; Robert N Adams; Richard L Skolasky; Angela Vincent; Daniel B Drachman Journal: Muscle Nerve Date: 2004-07 Impact factor: 3.217
Authors: Ivana Z Basta; Tatjana D Pekmezović; Stojan Z Perić; Darija B Kisić-Tepavčević; Vidosava M Rakočević-Stojanović; Zorica D Stević; Dragana V Lavrnić Journal: Neurol Sci Date: 2012-08-09 Impact factor: 3.307
Authors: W Pascale ter Beek; Pilar Martínez-Martínez; Mario Losen; Marc H de Baets; Axel R Wintzen; Jan J G M Verschuuren; Erik H Niks; Sjoerd G van Duinen; Angela Vincent; Peter C Molenaar Journal: Am J Pathol Date: 2009-09-10 Impact factor: 4.307