RESPIRATORY INSUFFICIENCY IN NEURONOPATHIC AND NEUROPATHIC DISORDERS

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 idiopat hic demyelinating neuropathy (4), Vialetto-van Laere syndrome (3), her editary motor and sensory neuropathy (3) and a miscellaneous group (5) . We also describe seven patients with Guillain-Barre syndrome (CBS) w ho required longterm ventilatory support for over 6 months to 7 years after the initial illness. Respiratory insufficiency occurred as a con sequence of respiratory muscle weakness, impaired bulbar function and restrictive lung defects. In some groups presentation was with progres sive nocturnal hypoventilation culminating in acute respiratory failur e. Five patients with CBS or chronic idiopathic demyelinating neuropat hy were weaned from ventilatory support up to 18 months after the init ial illness. The remaining 24 patients required continuous or nocturna l ventilatory support using intermittent positive-pressure ventilation (13), negative pressure ventilation (4), nasal-mask-delivered intermi ttent positive-pressure ventilation (4), nasal-mask-delivered continuo us positive-pressure ventilation (3), mouthpiece-assisted ventilation by day (2) and rocking bed (1). None have been weaned from support aft er a period of ventilation ranging from one month to 10 years. Eight p atients have subsequently died.