1995
DOI: 10.1093/brain/118.3.707
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Natural history of amyotrophic lateral sclerosis in a database population Validation of a scoring system and a model for survival prediction

Abstract: Over 1200 patients with motor neuron disease have been carefully diagnosed, followed, and included in a detailed database delineating characteristics of the disease. Of these patients, 831 were identified as exhibiting typical, sporadic amyotrophic lateral sclerosis (ALS). The progression of the disease in these patients has been followed with our scoring system, and the ALS score was verified as a significant covariate of survival. Age at first symptom, delay from first symptom to entering ALS clinic, and rat… Show more

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Cited by 611 publications
(396 citation statements)
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“…Approximately 10% of ALS cases are familial (1) and in some of these the disease is linked to mutations in the CuZn-superoxide dismutase (SOD1) gene (2). Overall, Ϸ6% of all cases with ALS show SOD1 mutations, and more than 100 different such mutations have been identified (3).…”
mentioning
confidence: 99%
“…Approximately 10% of ALS cases are familial (1) and in some of these the disease is linked to mutations in the CuZn-superoxide dismutase (SOD1) gene (2). Overall, Ϸ6% of all cases with ALS show SOD1 mutations, and more than 100 different such mutations have been identified (3).…”
mentioning
confidence: 99%
“…Em nossos casos encontramos apenas alterações compatíveis com discoartropatias degenerativas da coluna, sem sinais de compressão medular. Dosagem aumentada da CK, descrita em 45 a 50% dos casos de DNM 13 , mostrouse presente em 5 dos nossos doentes. Este aumento enzimático, mais freqüentemente visto na forma de DNM com envolvimento puro do NMI 21 , às vezes muito acima do valor normal, pode trazer uma confusão diagnóstica com as miopatias, mas não reflete, necessariamente, um pior prognóstico 13 .…”
Section: Discussionunclassified
“…Dosagem aumentada da CK, descrita em 45 a 50% dos casos de DNM 13 , mostrouse presente em 5 dos nossos doentes. Este aumento enzimático, mais freqüentemente visto na forma de DNM com envolvimento puro do NMI 21 , às vezes muito acima do valor normal, pode trazer uma confusão diagnóstica com as miopatias, mas não reflete, necessariamente, um pior prognóstico 13 . Os achados de biopsia de músculo refletem o processo de desenervação, como era esperado 13 .…”
Section: Discussionunclassified
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“…Patients with ALS die due to respiratory failure, usually 2 -5 years after onset because of irreversible progression of symptoms such as muscle weakness [1]. Noninvasive ventilation (NIV) and tracheostomy and invasive ventilation (TIV) are used to treat respiratory disorders and can improve survival time [2] [3] [4].…”
Section: Introductionmentioning
confidence: 99%