• Bebiana Conde, Ana Ferreira, Elsa Matos, and Duarte Rocha.
• Chest. 2014 Mar 1;145(3 Suppl):461A.

Session TitlePhysiology/PFTs/Rehabilitation PostersSESSION TYPE: Poster PresentationsPRESENTED ON: Saturday, March 22, 2014 at 01:15 PM - 02:15 PMPURPOSE: Compare symptons, respiratory function evolution and survival of patients with bulbar presentation amyothrophic lateral sclerosis and spinal presentation amyothrophic lateral sclerosis.MethodsWe perform a retrospective and descriptive study, based on clinical process review. We included bulbar and spinal presentation amyothrophic lateral sclerosis patients followed in a Pulmonology consultation, specialized in Neuromuscular Diseases. We assessed demography, clinical and functional evolution and survival.ResultsWe included 27 patients, 15 with spinal presentation amyothrophic lateral sclerosis and 12 with bulbar presentation; a male gender predominance (n=18) was observed. Both groups had a median age of 70 ± 13 years. At diagnosis, hypoventilation symptoms were very common, affecting 77% (n=10) of bulbar presentation amyothrophic lateral sclerosis patients and 43% (n=6) of spinal presentation amyothrophic lateral sclerosis patients. Respiratory failure was present in 3 patients, all of them with bulbar presentation. At diagnosis, both groups had compromised respiratory function, primarily the bulbar presentation patients, but the differences were not statistically significant. In bulbar presentation patients we document a precocious use of non invasive ventilation, percutaneous endoscopic gastrostomy use and access to assisted cough techniques, the later, statistically significant. Serial evaluation shows progressive functional decay in both groups, more obvious in bulbar presentation patients. No intervention revealed impact in both groups respiratory function decay. Kaplan-Meier curve analysis reveal worst survival in bulbar presentation amyothrophic lateral sclerosis patients and statistical tests validate this difference.ConclusionsBulbar presentation amyothrophic lateral sclerosis patients had worst survival, major respiratory function compromise at diagnosis and a faster functional deterioration. The evaluated therapeutic attitudes did not show impact in respiratory function evolution.Clinical ImplicationsAmyothrophic lateral sclerosis clinical presentation is crucial in definition of therapeutic strategy and prognosis. Respiratory functional evolution cannot be changed by the use of non invasive ventilation or assisted cough techniques.DisclosureThe following authors have nothing to disclose: Artur Vale, Ana Ferreira, Elsa Matos, Duarte Rocha, Bebiana CondeNo Product/Research Disclosure Information.

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