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 50-year-old gentleman, with no previous comorbidities, presented with difficulty in neck holding, double vision, and ptosis of 2½ year duration. Repetitive nerve stimulation (RNS) and neostigmine test were suggestive of MG. Anti-AChR was negative. The patient was started on steroids, pyridostigmine, and azathioprine. Contrast-enhanced computed tomography (CECT) scan of the chest was normal. He improved symptomatically but never had complete remission. After 1½ years, he had worsening of symptoms associated with difficulty in chewing and swallowing, slurring of speech, and breathing difficulty. A diagnosis of myasthenic crisis was made, and the patient was given five cycles of plasma exchange with no improvement in symptoms. Anti-MuSK antibody was positive. He was started on intravenous (IV) rituximab (375 mg/m2) weekly for 4 weeks, and the symptoms gradually improved. After 2 weeks of initiation of treatment, the breathing difficulty resolved, and ptosis, diplopia, and neck holding improved after the next 2 months. Steroids and azathioprine were tapered and stopped. He is doing well currently at 24 months of follow-up after three cycles of rituximab and is planned for another cycle.