A 23-year-old lady presented a with 9-month history of weakness in the neck and limb muscles along with bilateral ptosis. RNS and neostigmine test were suggestive of MG. AChR antibodies were negative. With anticipating an impending crisis, she was given IVIG in a dosage of 2 g/kg. Her symptoms improved and she was discharged on steroids, mycophenolate, and pyridostigmine. Two months later, she started having swallowing difficulty for which she received another course of IVIG (2 g/kg) elsewhere. Mycophenolate and steroids were continued. Five months later, she again had dysphagia and ptosis with progressive shortness of breath. She was given IVIG (2 g/kg) for the third time; mycophenolate was replaced by azathioprine. Anti-MuSK sent now was positive and CECT chest was normal. Again 2 months later, she had ptosis, dysphagia, and hoarseness of voice. This time she was administered five cycles of plasma exchange with only mild improvement. In view of persistent symptoms, rituximab (375 mgm2 weekly for 4 weeks) was given. Her bulbar symptoms improved in 2 weeks, and she started taking oral feeds. She is doing well and is in complete remission at 18-month follow-up after completion of two cycles of rituximab.
A 48-year-old gentleman, diagnosed as anti-MuSK-positive MG at another center, was on treatment with steroids and pyridostigmine. He presented to our emergency services with shortness of breath. Five cycles of plasma exchange followed by a course of IVIG were given without improvement. He was then given IV rituximab (375 mg/m2 weekly for 4 weeks) and he had complete resolution of symptoms. Steroids were tapered and stopped. He is in remission for the past 1½ year and is on intermittent pulses of rituximab.