Migrating atelectasis in Werdnig-Hoffmann disease: Pulmonary manifestations in two cases of spinal muscular atrophy type 1

Spinal muscular atrophy (SMA) or Werdnig-Hoffmann disease is the second mos t common neuromuscular disease, with 25% of cases presenting in infancy. De letions in the survival motor neuron gene are believed responsible for auto somal-recessive SMA. SMA affects about 1 in 10,000 births. Symptomatic newb orns have severe hypotonia, may have respiratory distress, may be unable to feed, and rapidly progress to death early in infancy. This paper describes another early pulmonary manifestation of SMA, i.e., migrating or rotating atelectasis, in 2 patients with infantile SMA. Migrating or rotating atelec tasis may suggest the diagnosis of SMA. Pediatr Pulmonol. 1999;28:149-153. (C) 1999 Wiley-Liss, Inc.