Immunosuppressive treatment of rippling muscles in patients with myasthenia gravis
ABSTRACT Rippling muscle disease is a rare autosomal dominant disorder that may occur sporadically. In this report two patients presenting with rippling muscles followed by myasthenia gravis are described. Our first patient developed rippling muscles about 1 month after infection with Yersinia enterocolitica. Two years later myasthenia gravis appeared. Our second patient had a 2-year history of asthma prior to the onset of rippling muscles which preceded the myasthenic symptoms by 4-8 weeks. Acetylcholine receptor and anti-skeletal muscle antibody titers were positive in both patients. In both patients the rippling phenomena worsened with pyridostigmine treatment but markedly improved after immunosuppression with azathioprine.
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ABSTRACT: An unusual association of asthma and myasthenia gravis (MG) complicated by tracheal stenosis is reported. The patient was a 35-year-old black woman with a history of severe asthma and rhinitis over 30 years. A respiratory tract infection triggered a life-threatening asthma attack whose treatment required orotracheal intubation and mechanical ventilatory support. A few weeks later, tracheal stenosis was diagnosed. Clinical manifestations of MG presented 3 years after her near-fatal asthma attack. Spirometry showed severe obstruction with no response after inhalation of 400 microg of albuterol. Baseline lung function parameters were forced vital capacity, 3.29 L (105% predicted); forced expiratory volume in 1 second (FEV1), 1.10 L (41% predicted); maximal midexpiratory flow rate, 0.81 L/min (26% predicted). FEV1 after administration of albuterol was 0.87 L (32% predicted). The patient's flow-volume loops showed flattened inspiratory and expiratory limbs, consistent with fixed extrathoracic airway obstruction. Chest computed tomography scans showed severe concentric reduction of the lumen of the upper thoracic trachea.Journal of investigational allergology & clinical immunology: official organ of the International Association of Asthmology (INTERASMA) and Sociedad Latinoamericana de Alergia e Inmunología 02/2007; 17(4):267-70. · 2.64 Impact Factor
Article: Rippling muscle disease: A review[Show abstract] [Hide abstract]
ABSTRACT: Rippling muscle disease (RMD) is a benign myopathy with symptoms and signs of muscular hyperexcitability. The typical finding is electrically silent muscle contractions provoked by mechanical stimuli and stretch. After the first description in 1975, there have been several publications on this disorder. Although RMD most often is reported with autosomal dominant inheritance, some sporadic cases are found, and an association with other diseases such as myasthenia gravis has also been reported. The pathophysiological mechanism is still not clarified. Abnormalities in calcium homeostasis in the sarcoplasmic reticulum have been proposed as the most probable causes. However, recent genetic studies make a primary channelopathy unlikely. In this article, a review of this curious disease is presented.Muscle & Nerve 01/2002; Suppl 11(S11):S103-7. DOI:10.1002/mus.10156 · 2.31 Impact Factor
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ABSTRACT: Rippling muscle disease (RMD) is a rare disorder that occurs in both familial and sporadic forms. Seven patients have previously been reported with myasthenia gravis and sporadic RMD. There have been conflicting reports of the electrophysiological characteristics of rippling muscles in this acquired form. Another such patient is reported, and the clinical, electrophysiological, and laboratory features of this disorder are described. In addition, this patient had alopecia areata and recurrent metastatic thymoma, years after resection of a benign thymoma. This report emphasizes the clinical manifestations of RMD in association with myasthenia gravis (RMD-MG), and its distinctive features, in this and previously reported patients.Muscle & Nerve 01/2004; 29(1):143-6. DOI:10.1002/mus.10494 · 2.31 Impact Factor