Limb girdle muscular dystrophy type 2A presenting with cardiac arrest

The occurence of respiratory failure in progressive neuromuscular disorders is well recognized, This failure is observed most commonly in Duchenne dys trophy but sometimes occurs in Becker's, limb-girdle, and facioscapulohumer al dystrophies. Patients usually present acutely or subacutely with cyanosi s and cor pulmonale, with severe decompensation often being precipitated by an acute intercurrent infection, However, cardiopulmonary arrest is an unc ommon presentation. A male diagnosed with limb-girdle muscular dystrophy ty pe 2A who presented with cardiopulmonary arrest that was precipitated by an upper respiratory tract infection is presented. The nocturnal application of noninvasive intermittent positive pressure ventilation with a bilevel po sitive airway pressure (Bi-PAP) device improved his symptoms and quality of life without resorting to more-invasive and more-restrictive forms of supp ort. This report demonstrates an unusual presentation of limb-girdle muscul ar dystrophy and documents that nocturnal nasal administration of continuou s airway pressure using the Bi-PAP device may be sufficient to maintain ade quate ventilation in such patients. (C) 2001 by Elsevier Science Inc. All r ights reserved.