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Vol. 10. Issue 6.
Pages 499-504 (November - December 2004)
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Vol. 10. Issue 6.
Pages 499-504 (November - December 2004)
CASO CLÍNICO/CLINICAL CASE
DOI: 10.1016/S0873-2159(15)30619-X
Open Access
Insuficiência respiratória aguda como primeira manifestação de esclerose lateral amiotrófica: dois casos clínicos
Amyotrophic lateral sclerosis revealed by acute respiratory failure: two case studies
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Susana Moreira1, Tátá Manuela2, Lurdes Carvalho3, Joaquim Pontes da mata4
1 Interna do 5º ano do Internato Complementar de Pneumologia, Hospital de Pulido Valente, Alameda das Linhas de Torres, 117, 1769-001 Lisboa, Portugal.
2 Interna do 3º ano do Internato Complementar de Pneumologia, Hospital de Pulido Valente, Alameda das Linhas de Torres, 117, 1769-001 Lisboa, Portugal.
3 Assistente graduada de Pneumologia, Hospital de Pulido Valente, Alameda das Linhas de Torres, 117, 1769-001 Lisboa, Portugal.
4 Director de Serviço de Pneumologia, Hospital de Pulido Valente, Alameda das Linhas de Torres, 117, 1769-001 Lisboa, Portugal.
Article information
RESUMO

A esclerose lateral amiotrófica (ELA) é uma doença neuromuscular rapidamente progressiva que envolve todos os grupos musculares com excepção dos músculos extra-oculares e dos esfíncteres. A maior causa de morbilidade e mortalidade são as complicações respiratórias que surgem habitualmente numa fase tardia da história natural da doença. Num número reduzido de casos, a ELA tem como manifestação inicial a insuficiência respiratória aguda.

Os autores apresentam 2 casos clínicos de doentes internados num Serviço de pneumologia, sem doença neuromuscular prévia conhecida, com o diagnóstico de insuficiência respiratória aguda de etiologia desconhecida e que se revelaram ser ELA.

A análise dos casos discutidos, e dos que estão já publicados na literatura, sugere que o aparecimento de insuficiência respiratória aguda em doentes sem história prévia de patologia pulmonar e/ou cardíaca, especialmente na 5.ª e 6.ª décadas de vida, deve alertar para a ELA como hipótese diagnóstica.

REV PORT PNEUMOL 2004; X (6): 499-504

Palavras-chave:
Esclerose lateral amiotrófica
insuficiência respiratória aguda
ventilação não invasiva
ABSTRACT

Amyotrophic lateral sclerosis (ALS) is a fast progressing neuromuscular disease that affects all but the extrinsic muscles of the eye and sphincters. The main cause of morbidity and mortality are the respiratory complications that usually start in a late stage of the disease’s natural history. In a small number of cases acute respiratory failure is the initial manifestation of the disease.

The authors present 2 case studies admitted to a pulmonary department, without previously known neuromuscular disease, with the diagnosis of acute respiratory failure of unknown ethiology and that were later shown to be cases of ALS.

Analysis of the cases presented here, and those already published in the literature, suggests that the occurrence of acute respiratory failure in patients without a previous history of pulmonary and/or heart disease, especially in the 5th and 6th decade of life, should alert to ALS as a possible diagnosis.

REV PORT PNEUMOL 2004; X (6): 499-504

Key-words:
Amyotrophic lateral sclerosis
acute respiratory failure
non invasive ventilation
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Copyright © 2004. Sociedade Portuguesa de Pneumologia/SPP
Pulmonology

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