Amyotrophic lateral sclerosis: considerations on diagnostic criteria

Amyotrophic lateral sclerosis: considerations on diagnostic criteria

Alternative title Esclerose lateral amiotrófica: considerações sobre critérios diagnósticos
Author Chieia, Marco A. Autor UNIFESP Google Scholar
Oliveira, Acary Souza Bulle Autor UNIFESP Google Scholar
Silva, Helga Cristina Almeida da Autor UNIFESP Google Scholar
Gabbai, Alberto Alain Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder, compromising the motor neuron, characterized by progressive muscle weakness, with reserved prognosis. The diagnosis is based on inclusion and exclusion clinical criteria, since there is no specific confirmation test. The objective of this research is to critically examine the main diagnosis instrument - El Escorial revisited, from the World Federation of Neurology (1998). Of the 540 patients with initial ALS diagnosis, either probable or definite, seen at UNIFESP-EPM, 190 underwent thorough investigation, following regular clinical and therapeutic treatment for over two years. Thirty patients (15.78%) had their diagnosis completely changed. The false-positive diagnoses were related to: early age, clinical presentation of symmetry, weakness greater than atrophy, symptomatic exacerbation. In addition, three patients with myasthenia gravis developed framework for ALS, suggesting the post-synaptic disability as a sign of early disease.

Esclerose lateral amiotrófica (ELA) é uma doença neurodegenerativa, que compromete o neurônio motor, caracterizada por fraqueza muscular progressiva, com prognóstico reservado. O diagnóstico é baseado na inclusão e exclusão de critérios clínicos, uma vez que não existe um teste de confirmação específica. O objetivo desta pesquisa é analisar criticamente o instrumento de diagnóstico principal - El Escorial revisited, da Federação Mundial de Neurologia (1998). Dos 540 pacientes com diagnóstico inicial de ELA, seja provável ou definitiva, vistos pela UNIFESP-EPM, 190 foram submetidos a investigação aprofundada, após tratamento clínico e terapêutico regular há mais de dois anos. Trinta pacientes (15,78%) tiveram seu diagnóstico mudado completamente. Os diagnósticos falso-positivos foram relacionados à idade precoce, a apresentação clínica da simetria, a fraqueza superior a atrofia, exacerbação sintomática. Além disso, três pacientes com miastenia gravis desenvolveram quadro de ELA, sugerindo a lesão pós-sináptica como um sinal precoce da doença.
Keywords amyotrophic lateral sclerosis
correct diagnosis
El Escorial revisited
esclerose lateral amiotrófica
diagnóstico correto
El Escorial revisited
Language English
Date 2010-12-01
Published in Arquivos de Neuro-Psiquiatria. Academia Brasileira de Neurologia - ABNEURO, v. 68, n. 6, p. 837-842, 2010.
ISSN 0004-282X (Sherpa/Romeo)
Publisher Academia Brasileira de Neurologia - ABNEURO
Extent 837-842
Origin http://dx.doi.org/10.1590/S0004-282X2010000600002
Access rights Open access Open Access
Type Article
SciELO ID S0004-282X2010000600002 (statistics in SciELO)
URI http://repositorio.unifesp.br/handle/11600/6031

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