A clinical epidemiological study of 251 cases of amyotrophic lateral sclerosis in the south of Brazil

Objective: To evaluate the functional status of amyotrophic lateral sclerosis (ALS) patients diagnosed at this institution; to analyze hospital and palliative care; to identify patients' knowledge about home care and supportive resources. Method: Twenty-nine patients were evaluated on the ALSFRS-R scale and two semi-structured questionnaires, at the start of the study and every four months thereafter for 1 year. Results: ALSFRS-R score was 30.1±11.5 initially and 24.4±10.5 at 1 year. There was an increase in use of physiotherapeutic care and adaptive aids. The primary caregivers were spouses (55.2%), parents/children/cousins (20.7%), friends (10.3%) and private nurses (3.5%); 10.3% of patients had no caregivers. Basic ALS patient care was provided by the public health system. Conclusion: ALS patients' multidisciplinary care was provided by UNICAMP hospital and its outpatient clinics and, in some patients, complemented by a private health plan or personal expenditure. Few ALS patients were aware of the possibility of home nursing. It is necessary to implement national and regional public home nursing in addition to multidisciplinary specialized care of ALS patients. Key words: amyotrophic lateral sclerosis, ALSFRS-R scale, home nursing, caregivers, health system.Perfil clínico e assistencial de pacientes com esclerose lateral amiotrófica RESUMO Objetivo: Avaliar o estado funcional de um grupo de pacientes com esclerose lateral amiotrófica (ELA) na instituição; analisar a assistência hospitalar e de cuidados paliativos; identificar o conhecimento dos pacientes sobre potenciais recursos de assistência e home care. Método: Vinte e nove pacientes foram submetidos à escala ALSFRS-R e dois questionários semi-estruturados no início do estudo e a cada 4 meses, por um ano. Resultados: O escore inicial da ALSFRS-R foi 30,1±11,5 e o final 24,4±10,5, tendo sido necessária maior assistência fisioterápica e uso de órteses. O cuidador primário foi: cônjuge (55,2%), pais/filhos/primos (20,7%), amigos (10,3%), particular (3,5%); 10,3% não tinham cuidador. A assistência na ELA baseou-se no sistema público de saúde. Conclusão: O cuidado multidisciplinar ao paciente com ELA fez-se através do hospital e ambulatórios UNICAMP e, para alguns pacientes, houve complementação por plano de saúde e custeio pessoal. A maioria dos pacientes desconhecia a possibilidade de assistência domiciliar na ELA. Faz-se necessária a estruturação nacional e regional da assistência domiciliar pública em ELA, acrescida ao trabalho multidisciplinar especializado. Palavras-chave: esclerose lateral amiotrófica, escala ALSFRS-R, assistência domiciliar, cuidador, sistema de saúde.

Section: Discussionsupporting

confidence: 87%

Clinical attention and assistance profile of patients with amyotrophic lateral sclerosis

Lima

Nucci

2011

“…Regarding patient-related characteristics, we found no differences in mortality by gender. Other studies conducted in Brazil and in other Countries found that prevalence is increased in men relative to women [18][19][20] , although more recent reports suggest a decline in the gender ratio. This apparent discrepancy may be explained by the length of our study, by certain causal factors, by subtle differences in the natural history of the disease, or by the overall lower expectancy of life of men relative to women (69.1 vs. 77.2 in the studied population) 21 .…”

Section: Discussionmentioning

confidence: 80%

Mortality rates due to amyotrophic lateral sclerosis in São Paulo City from 2002 to 2006

Matos

Conde

Fávero

et al. 2011

OBJECTIVE: To describe the mortality rates of amyotrophic lateral sclerosis (ALS) in the city of São Paulo as a function of demographics, year, and region. METHOD: This was a retrospective descriptive study. Information was obtained from death certificates registered at the Program for the Improvement of Mortality Information, Municipal Health Department (PRO-AIM/SMS), coded as G12.2 according to International Classification of Diseases (ICD-10), from 2002 to 2006. RESULTS: Over the studied time, were found 326 deaths (51.6% women, overall mean age of 64.1 years). Highest deaths percentages happened in those from 60 to 69 and 70 to 79 years and in white individuals. ALS mortality rates ranged 0.44/100,000 in 2002 and 0.76/100,000 in 2006. No significant changes overtime in administrative districts were found. CONCLUSION: ALS mortality rates in São Paulo were lower in comparison to other countries, however any risk factor in our environment, lifestyle or genetic characteristics were found.

“…However, some authors did not find this difference. Matos et al 26 and Werneck et al 27 compared the incidence of ALS among men and women. They analyzed death certificates and hospital charts from two different regions of Brazil and found no gender differences.…”

Section: Epidemiological Characteristicsmentioning

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

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.