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. © 1983 Springer-Verlag.