Amyotrophic lateral sclerosis in Brazil: 1998 national survey

Dietrich-neto, Flávia; Callegaro, Dagoberto; Dias‐Tosta, Elza; Silva, Helga Cristina Almeida da; Ferraz, Maria Elizabeth; Lima, J.A. De; Oliveira, Acary Souza Bullé

doi:10.1590/s0004-282x2000000400002

Cited by 52 publications

(25 citation statements)

References 23 publications

(11 reference statements)

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“…Additionally, along with other studies, the present study confirms that the disease exhibits a later onset in women and for the bulbar onset variant [35]. It also exhibits an earlier onset in Latin American countries [36] and a predominance in the Caucasian phenotype [2,37], although these features do not impact survival rates.…”

Section: Discussionsupporting

confidence: 89%

Multidisciplinary Care Improves Survival of Patients with Amyotrophic Lateral Sclerosis in the Unique Health System (SUS) in Brazil

Moura¹,

Casulari²,

Novaes³

2017

J Neurol Disord

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Objective: To determine the effectiveness of multi-professional palliative care in patients with amyotrophic lateral sclerosis in Brazilian public health system. Methods:Historic-control study before and after the Reference Center, with records and mortality data of 302 patients treated with riluzole between 2005 and 2015, were retrospectively evaluated and survival curves were analyzed.Results: There were 181 men (59.9%) and 121 women (40.1%) with a diagnostic delay time of 28.1 months for spinal-onset and 20.1 months for bulbar-onset (p=0.003) and a mean survival of 61.7 ± 5.4 months for spinal-onset and 38 ± 3.5 months for bulbar-onset (p=0.01). Electromyography did not confirm the diagnosis in 55.6% of the suspected spinal-onset cases and 36.1% of the bulbar-onset. From 2005 to 2011, mean survival was 33.6 ± 2.4 months, and after multidisciplinary care, 72.2 ± 7.2 months (log-rank: 0.0001). Conclusion:Multidisciplinary care improves the survival of amyotrophic lateral sclerosis patients in the Unique Health System.

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Section: Discussionsupporting

confidence: 89%

Multidisciplinary Care Improves Survival of Patients with Amyotrophic Lateral Sclerosis in the Unique Health System (SUS) in Brazil

Moura¹,

Casulari²,

Novaes³

2017

J Neurol Disord

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“…regional published data concerning ALS mortality in Brazil, where it is fairly reliable (São Paulo and rio de Janeiro cities), infers an incidence rate of 0.3-0.5/100,000 inhabitants. considering the mean duration of the disease to be 3 years, this yields a disease prevalence of about 1,440 to 2,400 cases in Brazil (0.9 to 1.5/100,000 inhabitants), lower than that described in europe and USA 4 . Most cases of ALS are sporadic and more frequent in men than women (3:2).…”

Section: Resultsmentioning

confidence: 80%

Conjugal amyotrophic lateral sclerosis in Brazil

Godeiro-Júnior

Oliveira

Felı́cio

et al. 2009

Arq. Neuro-Psiquiatr.

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-The origin of amyotrophic lateral sclerosis (ALS) remains unknown, although it seems to be multifactorial. The role of environmental factors has been frequently investigated and suspicion of its influence can be obtained when clusters of a rare disease are described. Objective: To describe conjugal cases of ALS in Brazil. Method: We describe 2 couples in which both spouses were affected by ALS. Both couples had lived in southeast Brazil and were married for at least 20 years. Results: There was a great variability in clinical presentation of ALS in our patients. In both couples the interval between disease onsets was short. No precise environmental factors could be identified at the origin of these conjugal cases. Conclusion: The occurrence of ALS in couples living in the same area may be epidemiologically important, but we cannot exclude that cases may be due to a chance association.Key WordS: amyotrophic lateral sclerosis, conjugal, environmental factors, etiology.esclerose lateral amiotrófica em casais no Brasil resumo -A origem da esclerose lateral amiotrófica (eLA) permanece desconhecida. o papel de fatores ambientais tem sido freqüentemente investigado e a suspeição de sua influência pode ser obtida quando são descritas salvas de casos de uma doença rara. Objetivo: descrever casos de eLA em casais no Brasil. Método: Apresentamos dois casais em que ambos os cônjuges forma acometidos pela eLA. Ambos os casais residiram na região sudeste do Brasil e estiveram casados por pelo menos 20 anos. Resultados: Houve grande variabilidade na apresentação clínica da eLA em nossos pacientes. em ambos os casais, o intervalo de início da doença foi curto. Nenhum fator ambiental foi identificado na etiologia destes casos conjugais. Conclusão: A ocorrência de eLA em casais que habitam a mesma região pode ser epidemiologicamente importante, mas não podemos excluir que estes casos tenham sido mera associação.PALAvrAS-cHAve: esclerose lateral amiotrófica, casais, fatores ambientais, etiologia.

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“…The patient may experience fatigue, gait instability, changes in speed or rhythm during speech, and a weak hand grip. Survival time after diagnosis is variable in the literature ( from eight to 66 months) 5,6,7,9,10,11,12,13,14,15,16,17,18,19,20,21,22 . In general, shorter intervals from symptom onset to diagnosis predict lower survival rates.…”

mentioning

confidence: 99%

Epidemiological and clinical factors impact on the benefit of riluzole in the survival rates of patients with ALS

Fávero

Voos

Castro

et al. 2017

Arq. Neuro-Psiquiatr.

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Objective To investigate the impact of epidemiological and clinical factors on the benefit of riluzole in patients with amyotrophic lateral sclerosis (ALS). Methods The survival rate of 578 patients with ALS (1999-2011) was analyzed by descriptive statistics and Kaplan-Meier curves. Considering the median of the sample survival time (19 months), patients were divided in two groups: below (B19) and above the median (A19). Kaplan-Meier curves compared the survival rates of patients treated with riluzole and with patients who did not take the medication. Results Riluzole increased the survival rates of patients with lower limb onset who were diagnosed after the first appointment in B19. Patients with bulbar onset and diagnosed on the first, or after the first appointment showed higher survival rates in A19. Males lived longer than females in both groups. Conclusion Epidemiological and clinical factors influenced the benefit of riluzole in the survival rates of patients with ALS.

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Amyotrophic lateral sclerosis in Brazil: 1998 national survey

Cited by 52 publications

References 23 publications

Multidisciplinary Care Improves Survival of Patients with Amyotrophic Lateral Sclerosis in the Unique Health System (SUS) in Brazil

Multidisciplinary Care Improves Survival of Patients with Amyotrophic Lateral Sclerosis in the Unique Health System (SUS) in Brazil

Conjugal amyotrophic lateral sclerosis in Brazil

Epidemiological and clinical factors impact on the benefit of riluzole in the survival rates of patients with ALS

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