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.
|
Authors | A E de Jager, A F Meinesz |
Journal | Journal of neurology
(J Neurol)
Vol. 230
Issue 2
Pg. 105-10
( 1983)
ISSN: 0340-5354 [Print] Germany |
PMID | 6196456
(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
|