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Acid maltase deficiency: treatment of respiratory insufficiency with cuirass respirator.

Abstract
Three patients with the late-onset form of acid maltase deficiency showed a gradual weakening of proximal limb and trunk muscles leading to severe respiratory insufficiency. Considerable deterioration of pulmonary function occurred owing to a vicious cycle of hypoventilation and exhaustion. Treatment by nocturnal cuirass ventilation with tailor-made shells was successful. The muscle weakness still progressed very slowly but there were no more respiratory problems.
AuthorsA E de Jager, A F Meinesz
JournalJournal of neurology (J Neurol) Vol. 230 Issue 2 Pg. 105-10 ( 1983) ISSN: 0340-5354 [Print] Germany
PMID6196456 (Publication Type: Case Reports, Journal Article)
Topics
  • Adult
  • Glycogen Storage Disease (complications)
  • Glycogen Storage Disease Type II (complications)
  • Humans
  • Male
  • Middle Aged
  • Respiratory Insufficiency (etiology, therapy)
  • Ventilators, Mechanical

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