Gastrointestinal Radiologic Manifestations of Proximal Spinal Muscular Atrophy (Kugelberg-Welander Syndrome)

Proximal spinal muscular atrophy (Kugelberg-Welander syndrome) is a degenerating disease of the anterior horn cells of the spinal cord with atrophy of the proximal muscles resembling muscular dystrophy. The patient in this report exhibits radiographic features in the gastrointestinal tract similar to those seen in the muscular dystrophies, including myotonic dystrophy.