Update on diagnosis and therapy of juvenile myasthenic crisis / 中国小儿急救医学

ABSTRACT

Myasthenia gravis is an autoimmune disorder resulting from the production of antibodies against acetylcholine receptors leading to the destruction of the postsynaptic membrane at the neuromuscular junction.Myasthenic crisis is defined as any myasthenia gravis exacerbation necessitating mechanical ventila-tion or post-operation extubation delayed beyond 24 hours because of respiratory weakness.Most patients presenting with myasthenic crisis have an identifiable risk factor.The diagnosis of myasthenic crisis should be suspected in all patients with respiratory failure,particularly those with unclear etiology.Acute management of myasthenic crisis includes ventilatory therapy,removal of the offending trigger and immunomodulatory treat-ments.The latter includes plasma exchange or intravenous immunoglobulin,and high dose corticosteroids therapy.