Immune globulin/pyridostigmine
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Lack of efficacy: 2 case reports A 12-year-old girl and a 7-year-old boy exhibited lack of efficacy to treatment with immune globulin (the girl), and immune globulin with pyridostigmine (the boy), for myasthenia gravis. Patient 1: A 12-year-old girl, who had been diagnosed with myasthenia gravis in October 2010, was hospitalised due to blepharoptosis, dyspnoea and dysphagia in November 2017. When she had been diagnosed with myasthenia gravis, she had presented with blepharoptosis, responsive to pyridostigmine 180 mg/day. However, 2 years later, her neurological condition worsened, and in 2013, her condition deteriorated and she experienced two myasthenic crises. Later, in January 2017, she experienced her third myasthenic crisis in the course of respiratory tract infection, and in November 2017, she was hospitalised for further treatment. On admission, she was found to have blepharoptosis, masticatory muscle fatigue, dysphagia and difficulty with activities of daily living. Her Quantitative Myasthenia Gravis (QMG) score was 24. She was treated with increased doses of unspecified corticosteroids and subsequently discharged. She exhibited slight weakness and ptosis at discharge. However, she soon complained of fatigue, and 2 days later, she experienced another myasthenic crisis. She was re-hospitalised for mechanical ventilation. However, her limb fatigue progressively worsened after removal of ventilator. She received IV immune globulin 0.4 g/kg/day for 5 days; however, her neurological condition did not improve, but progressively worsened, necessitating resumption of ventilation. Thereafter, she received methylprednisolone, following which her ptosis, dysphagia and dyspnoea disappeared; however, her limbs readily fatigued on exercise. She was prescribed pyridostigmine, azathioprine and methylprednisolone at discharge. She was followed regularly in outpatient clinic. In March 2018, evaluation showed blepharoptosis as her major symptom; hence, tacrolimus was introduced, resulting in a favourable outcome. Patient 2: A 7-year-old boy was diagnosed with myasthenia gravis in June 2012. His symptoms subsided after 1 month of treatment with pyridostigmine 60 mg/day [route not stated]. However, in August 2012, it was observed that he experienced difficulty swallowing, and his cry was weak. His QMG score was found to be 11. He was hospitalised, and he was administered pyridostigmine 90 mg/day and IV immune globulin 5 g/day for 5 days. However, no significant improvement was noted over the following 2 weeks. He developed dyspnoea secondary to pneumonia, which necessitated mechanical ventilation. He received prednisone, IV immune globulin and unspecified antibiotics, resulting in symptomatic improvement. He continued receiving prednisone and pyridostigmine 90 mg/day. However, in January 2013, his blepharoptosis worsened and he developed ophthalmoplegia. He started receiving prednisone, after which his symptoms disappeared. Thereafter, he was followed up in an outpatient clinic. In January 2016, pyridostigmine was stopped.
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