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 47-year-old gentleman presented a with 2-year history of bilateral ptosis, diplopia, and bulbar weakness. Neostigmine test and RNS were positive, while CECT of the chest was normal. Anti-AChR antibody was negative. Symptoms responded to steroids, pyridostigmine, and azathioprine. He was well for the next 2 years when he had developed severe bulbar weakness and respiratory difficulty. Anti-MuSK was positive, and five cycles of plasma exchange were administered with no response. IV rituximab was then started with good response after two doses, steroids and immunomodulation were tapered, and rituximab was given weekly for 4 weeks every 6 months. He is symptom free on 2-year follow-up.