Myasthenia gravis is an autoimmune disorder of peripheral nervous system, leading to fluctuating muscle weakness. It is caused by circulating antibodies that block acetylcholine nicotinic postsynaptic receptors at the postsynaptic neuromuscular junction. Myasthenic crisis is a life-threatening complication, which is defined as weakness from acquired myasthenia gravis. In this paper we described a 15-year-old boy who was admitted to the Paediatric Intensive Care Unit due to myasthenic crisis. He had suffered not only from myasthenia gravis but also hypothyroidism, cerebral palsy and epilepsy. The patient required mechanical ventilation and was successfully treated with both plasmapheresis and intravenous immunoglobulins. He recovered from the crisis and then thymectomy was performed. Perioperative period and anaesthesia passed uncomplicated. Discharged home from the hospital after 2.5 month-treatment, for the last 4 years, he has only come on scheduled outpatient medical appointments. This case reveals that myasthenic crisis, albeit rare, may occur in male adolescents. In such cases multidisciplinary care followed by surgery becomes a procedure of choice. Concomitant medical problems, if well controlled, do not affect the results of outcome of the underlying disease.Key words: myasthenia gravis, children; myasthenia gravis, myasthenic crisis; myasthenia gravis, general anaesthesia; myasthenic crisis, management; myasthenic crisis, plasmapheresis Anaesthesiology Intensive Therapy 2013, vol. 45, no 2, 82-84 Myasthenia gravis (MG) is an autoimmune disease in which neuromuscular transmission is impaired due to a partial blocking of acetylcholine receptors in the postsynaptic membrane by circulating antibodies. Clinically, MG manifests as an abnormal fatigability of skeletal muscles that is relieved by rest and anticholinesterase drugs [1]. Its incidence rate is approximately 50-125: 1 000 000. The two major groups of patients include women aged 20-35 years and men aged 60-75 years. Children < 16 years of age constitute approximately 10-16% of all patients. Moreover, the prevalence of MG is three-fold higher in girls than in boys [1].When this disease progresses rapidly or the dosage of anticholinesterase drugs is too low and the susceptibility to them is consequently reduced, MG can progress to a life--threatening condition known as myasthenic crisis.
CASE REPORTA 15-year-old boy (with a weight of 70 kg and a height of 165 cm) was admitted to the ICU of the Children's University Hospital from the neurology ward due to progressive hypodynamic respiratory failure and bulbar symptoms. His history revealed myasthenia of the lower limbs and articulation disorders that had been detected a year earlier. Based on clinical examinations, the patient was diagnosed with MG, and treatment with pyridostigmine at a dose of 60 mg day -1 was initiated. Moreover, a diagnosis of hypothyroidism was made, and levothyroxine was started 1 × 50 μg day -1 (euthyreosis for 3 months). The patient had a history of infantile cerebral ...