Rison Richard A
University of Southern California, Keck School of Medicine, Los Angeles County Medical Center, Presbyterian Intercommunity Hospital, Neurology Consultants Medical Group, Whittier, CA, USA.
Cases J. 2009 Aug 7;2:6565. doi: 10.1186/1757-1626-0002-0000006565.
Bulbar weakness and fatigue resulting in dysphagia and dysarthria is common in myasthenia gravis. In chronic MG it is often assumed that these symptoms herald an exacerbation of the patient's disease and doses of cholinergic agents and immunomodulatory therapies may be increased, along with initiation of plasma exchange. A case is presented in which dysphagia was refractory to standard MG therapy, leading to the subsequent discovery of cricopharyngeal sphincter achalasia as the primary cause of the patient's symptoms rather than an assumed myasthenia gravis exacerbation. The patient's dysphagia resolved after esophageal dilatation. Cricopharyngeal sphincter achalasia is a common disorder producing dysphagia in the elderly and needs to be considered in the evaluation of a myasthenic patient with worsening dysphagia when standard myasthenia gravis therapy fails. Discussion of myasthenia gravis, cholinergic therapy and cricopharyngeal sphincter achalasia is undertaken. Clinicians are encouraged to consider non-neurologic causes of worsening dysphagia in the myasthenic patient.
球部肌无力和疲劳导致吞咽困难和构音障碍在重症肌无力中很常见。在慢性重症肌无力中,通常认为这些症状预示着患者病情加重,胆碱能药物剂量和免疫调节治疗可能会增加,同时开始进行血浆置换。本文介绍了一例病例,该患者的吞咽困难对标准重症肌无力治疗无效,随后发现环咽括约肌失弛缓症是患者症状的主要原因,而非假定的重症肌无力病情加重。患者的吞咽困难在食管扩张后得到缓解。环咽括约肌失弛缓症是老年人吞咽困难的常见病因,在评估标准重症肌无力治疗无效且吞咽困难加重的重症肌无力患者时需要考虑。文中对重症肌无力、胆碱能治疗和环咽括约肌失弛缓症进行了讨论。鼓励临床医生考虑重症肌无力患者吞咽困难加重的非神经学原因。