Amyotrophic lateral sclerosis: considerations on diagnostic criteria

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. Key words: amyotrophic lateral sclerosis, correct diagnosis, El Escorial revisited.Esclerose lateral amiotrófica: considerações sobre critérios diagnósticos RESUMO 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. Palavras-chave: esclerose lateral amiotrófica, diagnóstico correto, El Escorial revisited. CorrespondenceMarco Antonio Troccoli Chieia Rua Mairinque 267 04037-020 -São Paulo SP -Brasil

Pathophysiological implications of actin-free Gc-globulin concentration changes in blood plasma and cerebrospinal fluid collected from patients with Alzheimer’s disease and other neurological disorders

“…The clinically definite ALS was diagnosed using the Airlie House/El Escorial Revisited World Federation of Neurology criteria. 13,14 All patients with ALS showed features of damage to upper and lower motor neurons as confirmed by electromyography (EMG) examination. None of the patients were treated with riluzol.…”

Benign fasciculations and Corticosteroid use: possible association? An update

“…Although the neurologic exam of our patient was entirely normal, ENMG revealed signs of fasciculation potentials, positive waves as well as fibrillations in the various muscle groups tested. 8–10 …”

Esclerosis lateral amiotrófica: actualización

“…La forma clásica de esta enfermedad se caracteriza por la mezcla de manifestaciones clínicas de lesión de las neuronas motoras superior e inferior y signos de alteración bulbar y respiratoria (tabla 2) (38 (39). Muchos pacientes con ELA presentan síntomas de déficit cognitivo, conductual y comportamental en el espectro de la DFT; estos son principalmente disfunción ejecutiva, irritabilidad, cambios de la personalidad con impulsividad y mal reconocimiento de la enfermedad; su presencia constituye un marcador pronóstico negativo.…”