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Journal of the National Medical Association logoLink to Journal of the National Medical Association
. 1982 May;74(5):475–478.

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

David Karasick, Stephen Karasick, Esmond Mapp
PMCID: PMC2552769  PMID: 7120481

Abstract

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Armstrong R. M., Fogelson M. H., Silberberg D. H. Familial proximal spinal muscular atrophy. Arch Neurol. 1966 Feb;14(2):208–212. doi: 10.1001/archneur.1966.00470080092014. [DOI] [PubMed] [Google Scholar]
  2. Goldberg H. I., Sheft D. J. Esophageal and colon changes in myotonia dystrophica. Gastroenterology. 1972 Jul;63(1):134–139. [PubMed] [Google Scholar]
  3. KUGELBERG E., WELANDER L. Heredofamilial juvenile muscular atrophy simulating muscular dystrophy. AMA Arch Neurol Psychiatry. 1956 May;75(5):500–509. doi: 10.1001/archneurpsyc.1956.02330230050005. [DOI] [PubMed] [Google Scholar]
  4. Krain S., Rabinowitz J. G. The radiologic features of myotonic dystrophy with presentation of a new finding. Clin Radiol. 1971 Oct;22(4):462–465. doi: 10.1016/s0009-9260(71)80116-2. [DOI] [PubMed] [Google Scholar]
  5. LEWITAN A., NATHANSON L. The roentgen features of muscular dystrophy. Am J Roentgenol Radium Ther Nucl Med. 1955 Feb;73(2):226–234. [PubMed] [Google Scholar]
  6. Peters H. A., Opitz J. M., Goto I., Reese H. H. The benign proximal spinal progressive muscular atrophies. Acta Neurol Scand. 1968;44(5):542–560. doi: 10.1111/j.1600-0404.1968.tb05591.x. [DOI] [PubMed] [Google Scholar]
  7. Simpson A. J., Khilnani M. T. Gastrointestinal manifestations of the muscular dystrophies. Am J Roentgenol Radium Ther Nucl Med. 1975 Dec;125(4):948–955. doi: 10.2214/ajr.125.4.948. [DOI] [PubMed] [Google Scholar]
  8. Weiner M. J. Myotonic megacolon in myotonic dystrophy. AJR Am J Roentgenol. 1978 Jan;130(1):177–179. doi: 10.2214/ajr.130.1.177. [DOI] [PubMed] [Google Scholar]

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