There has been much speculation on the subject of the utility of antibody status (acetylcholine receptor antibody positive (AChR+ve) vs MuSK antibody-positive) in prognostication and arranging therapy.[3] AIM OF THE STUDY This is a single-center, ambispective, comparative study comparing demographic and clinical characteristics, treatment response, and outcome of MuSK+ve MG with AChR+ve MG and patients with double-seronegative myasthenia (DN-MG). Rabbit Polyclonal to GRAK MATERIALS AND METHODS A retrospective chart review of MuSK+ve MG presenting to our institute from January 2010 to January 2016 was performed. Seropositivity for antibodies should not be used in isolation to guide the management or forecast the prognosis. Undue bad prognostication may impact the morale of individual. Clinical features and response to therapy in addition to antibody status must be regarded as before planning therapy. in 2001,[1] there have been multiple descriptions of medical features of these individuals. MuSK+ve MG are considered to have a more turbulent course at the beginning, and more severe symptoms at onset than acetylcholine receptor positive myasthenia (AChR+ve MG).[2] Neurologists tend to treat MuSK+ve MG more aggressively than AChR+ve MG. There has been much speculation about the power of antibody status (acetylcholine receptor antibody positive (AChR+ve) vs MuSK antibody-positive) in prognostication and arranging therapy.[3] AIM OF THE STUDY This is a single-center, ambispective, comparative study comparing demographic and clinical characteristics, treatment response, and outcome of MuSK+ve MG with AChR+ve MG and individuals with GSK583 double-seronegative myasthenia (DN-MG). MATERIALS AND METHODS A retrospective chart review of MuSK+ve MG showing to our institute from January 2010 to January 2016 was performed. All consecutive MuSK+ve MG who offered GSK583 to our institute from February 2016 to July 2017 were also recruited. Demographic data, medical details, and investigations were recorded. The analysis of myasthenia was made based on medical, electrophysiological, and serological findings. All the antibody screening (anti-AChR or anti-MuSK) was carried out by radioimmune assay. The severity of disease and response to therapy were recorded relating to Myasthenia Gravis Basis of America (MGFA) recommendations. Response to treatment and end result analysis were carried out only in those individuals with adequate follow-up. Poor end result was defined as one or more of the following: (1) postintervention status: unchanged, worse, exacerbation, death from MG; (2) failure to achieve low maintenance dose of pyridostigmine or steroids; (3) intravenous immunoglobulin (IVIg) or plasmapheresis (PLEX) on a regular basis. Quality-of-life assessment was carried out by MGQoL15r questionnaire. (4) Severe disease GSK583 was defined as MGFA IV or MGFA V. Low maintenance treatment was defined as pyridostigmine 120 mg and prednisolone having a dose reduction by 50% from the maximum dose. Good response to acetylcholine-esterase inhibitors (AChEIs) was defined as more than 50% improvement. Statistical analysis was carried out using STATA IC/11.1. Assessment of means/medians/proportions among three organizations was done. The association between antibody type and individual end result was analyzed by logistic regression. Significance was arranged at 0.05. RESULTS In this study, 23 MuSK+ve MG, 55AChR+ve MG, and 9 DN individuals were included [Table 1]. All the three organizations were comparable to each other in terms of duration of illness and connected comorbidities. The proportion of females in MuSK+ve MG (69.6%) was significantly higher than that in AChR+ve MG (41.8%) (= 0.02). There was no significant difference between the three organizations in terms of age of onset, bulbar symptoms at onset, median interval between the 1st sign and analysis, diurnal variance, positive neostigmine test, and positive repeated nerve stimulation test. Thymic hyperplasia on contrast-enhanced computed tomography chest was significantly higher in AChR+ve MG (41.3%) than MuSK+ve MG (13.3%) (= 0.04). The average quantity of myasthenic problems per patient-year was not significantly different between the three organizations (= 0.99). Table 1 Assessment of demographic, medical characteristics, and investigations of individuals with MuSK+ve MG with individuals with AChR+ve MG and individuals with double-seronegative MG (%)7 (30.4)32 (58.2)5 (55.6)?Female, (%)16 (69.6)23 (41.8)4 (44.4)Duration of disease (years), median (range)4 (0.5-19)3.5 (0.33-30)3 (1-19)0.900.69Other comorbidities, (%)0.390.84?Hypothyroidism3 (13.0)12 (21.8)1 (11.1)?Hyperthyroidism01 (1.8)0?Vascular risk factors8 (34.7)15 (27.3)1 (11.1)?Autoimmune illnesses3 (13.0)1 (1.8)0?Infectious disease2 (8.7)2 (3.6)0Age at onset, median (range)44 (14-66)35 (8-76)20 (14-65)0.340.52 First sign at onset, (%)0.310.11Ocular8 (34.8)29 (53.7)7 (77.8)Bulbar**11 (47.8)15 (27.8)1 (11.1)Limb4 (17.4)9 (16.7)1 (11.1)Respiratory01 (1.9)0Reported symptoms during illness0.120.10Pure ocular031Oculobulbar542Generalized18486Interval between 1st symptom and analysis (weeks), median (range)4 (0.3-72)4 (0.25-192)3 (0.25-48)0.510.32Patient with diurnal variation, (%)18/23 (78.2)46/53 (86.8)9/9 (100)0.570.65Patients (%) with positive neostigmine GSK583 test10/13 (76.9)28/29 (96.6)6/7 (85.7)0.07***0.12Patients (%) with positive RNST17/18 (94.7)39/43 (90.7)7/9 (77.8)0.350.88Patients (%) with thymic hyperplasia on imaging*2/15 (13.3)19/46 (41.3)2/6 (33.3)0.14****0.07 Open in a separate window AChR+ve MG: acetylcholine receptor antibodyCpositive myasthenia gravis; MuSK+ve MG: MuSK antibodyCpositive myasthenia gravis; RNST: repeated nerve stimulation test. #p1: value on assessment between individuals with MuSK+ve MG with individuals with AChR+ve MG.