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Pulmonary Physiology |

The Relevance of Amyotrophic Lateral Sclerosis Clinical Presentation: A Cohort Study

Artur Vale, DO; Ana Ferreira, DO; Elsa Matos, DO; Duarte Rocha, DO; Bebiana Conde, DO
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CHTMAD, Vila Real, Portugal


Chest. 2014;145(3_MeetingAbstracts):461A. doi:10.1378/chest.1824443
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Abstract

SESSION TITLE: Physiology/PFTs/Rehabilitation Posters

SESSION TYPE: Poster Presentations

PRESENTED ON: Saturday, March 22, 2014 at 01:15 PM - 02:15 PM

PURPOSE: Compare symptons, respiratory function evolution and survival of patients with bulbar presentation amyothrophic lateral sclerosis and spinal presentation amyothrophic lateral sclerosis.

METHODS: We 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.

RESULTS: We 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.

CONCLUSIONS: Bulbar 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 IMPLICATIONS: Amyothrophic 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.

DISCLOSURE: The following authors have nothing to disclose: Artur Vale, Ana Ferreira, Elsa Matos, Duarte Rocha, Bebiana Conde

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