Chest
Pulmonary Function at Diagnosis of Amyotrophic Lateral Sclerosis: Rate of Deterioration
Section snippets
METHODS
Records were reviewed of all ALS patients under our care over a 10-year period ending December 1991. Criteria used for the diagnosis of ALS were described previously.3 In brief, a combination of upper and lower motor neuron dysfunction was required. Those patients in whom pulmonary function studies, including vital capacity (VC), maximum negative inspiratory pressure (NIF), and maximum positive expiratory pressure (PEP), were available from the time of diagnosis are included in this report.
RESULTS
Records of 49 patients with ALS were reviewed. Four were in respiratory failure at the time of presentation and required mechanical ventilation before any pulmonary function tests were performed. Nine did not have pulmonary function studies at the time of diagnosis. Thirty-six (stages at presentation ranging from I to IV) had pulmonary function studies available at the time of diagnosis. Of the latter group, 24 had sequential pulmonary function studies available for analysis over a period of
DISCUSSION
The natural history of ALS is a relentless progression of increasing weakness of all skeletal muscles, including the muscles of respiration, with eventual respiratory failure and death.1, 2 Amyotrophic lateral sclerosis will occasionally go undiagnosed until after the onset of respiratory insufficiency,3, 6 as occurred in 4 of the 49 patients (8.2 percent) reviewed herein. It is much more common, however, for patients to present with limb or bulbar symptoms without any complaints of respiratory
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Manuscript received February 14; revision accepted June30.