Respiratory insufficiency in neuronopathic and neuropathic disorders |
| |
Authors: | Chalmers RM; Howard RS; Wiles CM; Hirsch NP; Miller DH; Williams A; Spencer GT |
| |
Affiliation: | Lane Fox Unit, St Thomas's Hospital, London, UK; Batten/Harris Unit, The National Hospital for Neurology and Neurosurgery, Queen Square, London, UK; Correspondence to Dr RS Howard, Department of Neurology, St Thomas' Hospital, London SE1 7EH, UK; Present address: University of Wales College of Medicine, Heath Park, Cardiff CF4 4XW, UK |
| |
Abstract: | Twenty-nine patients with a neuronopathic or neuropathic disorder were
referred for assessment of respiratory insufficiency between 1978 and 1994.
Diagnoses included spinal muscular atrophy (6), chronic idiopathic
demyelinating neuropathy (4), Vialetto-van Laere syndrome (3), hereditary
motor and sensory neuropathy (3) and a miscellaneous group (5). We also
describe seven patients with Guillain-Barre syndrome (GBS) who required
long-term ventilatory support for over 6 months to 7 years after the
initial illness. Respiratory insufficiency occurred as a consequence of
respiratory muscle weakness, impaired bulbar function and restrictive lung
defects. In some groups presentation was with progressive nocturnal
hypoventilation culminating in acute respiratory failure. Five patients
with GBS or chronic idiopathic demyelinating neuropathy were weaned from
ventilatory support up to 18 months after the initial illness. The
remaining 24 patients required continuous or nocturnal ventilatory support
using intermittent positive-pressure ventilation (13), negative pressure
ventilation (4), nasal-mask-delivered intermittent positive-pressure
ventilation (4), nasal-mask-delivered continuous positive-pressure
ventilation (3), mouthpiece-assisted ventilation by day (2) and rocking bed
(1). None have been weaned from support after a period of ventilation
ranging from one month to 10 years. Eight patients have subsequently died.
|
| |
Keywords: | |
本文献已被 Oxford 等数据库收录! |
|