Escala revisada de valoración funcional de esclerosis lateral amiotrófica: adaptación transcultural / Argentine cross-cultural adaptation of the amyotrophic lateral sclerosis: functional rating scale-revised

Resumen Introducción: La Escala Revisada de Valoración Funcional de la Esclerosis Lateral Amiotrófica es una herramienta básica para la evaluación de personas con esta enfermedad. En nuestro país se utilizan versiones sin el proceso metodológico necesario para adaptarlas culturalmente y poder certificar lo vero símil de los datos recogidos. Fue nuestro objetivo generar una apropiada versión adaptada al español argentino. Métodos: Se realizó la adaptación transcultural de la escala produciendo su versión argentina, conforme proce dimientos y medidas de verificación de calidad metodológica internacionalmente aceptadas: proceso secuencial de traducción/síntesis/retrotraducción; resolución de discrepancias y consolidación de la versión obtenida por comité multidisciplinario bajo metodología Delphi; puesta a prueba de dicha versión en una población de perso nas con esclerosis lateral amiotrófica analizando el nivel de dificultad sintáctico-pragmático; constitución de la versión final. El análisis del nivel de dificultad se constituyó en base a los siguientes indicadores: moda, mediana, índice variación cualitativa, razón de variación, frecuencia acumulada de apreciaciones positivas, identificación/ modificación de ítems observados, tomando como base un criterio de concordancia ≥ 80%. Resultados: Tras 3 rondas de consulta se obtuvo la versión consensuada por el comité, obteniéndose niveles de concordancia del 83,33%-100%. Su puesta a prueba involucró a 21 alfabetos funcionales, nativos/residentes, 67% hombres, media edad 51 años, media evolución 1,4 años. Ausencia de dificultad a nivel comprensión y la ausencia de ítems conflictivos. Discusión: La versión obtenida demostró su validez de contenido conceptual respecto de la original, sin presentar conflictos semánticos o pragmáticos que afecten su uso en nuestra población.

Abstract Introduction: Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised is a basic tool for the evaluation of people with this disease. In our country, versions are used without the necessary methodological process to adapt them culturally and to be able to certify the plausibility of the data collected. It was our goal to generate an appropriate version adapted to Argentine Spanish. Methods: Cross-cultural adaptation of the scale was ca rried out, producing its Argentine version. Said production was carried out according to internationally accepted methodological procedures: sequential process of translation / synthesis / back-translation and consolidation of the version obtained by a multidisciplinary committee that solved of disagreements under Delphi methodology. The version obtained was tested on ALS people to determine of the existing syntactic - pragmatic level of difficulty, after which the final version was constituted. Difficulty level analysis was performed according to indicators: mode, median, qualitative variation index, variation ratio, accumulated frequency of positive evaluations, identification/ modification of observed items (determining criterion: concordance criterion ≥ 80%). Results: After 3 rounds of consultation, the version agreed upon by the committee was obtained, obtaining agreement levels of 83.33%-100%. Its testing involved 21 functional alphabets, natives/residents, 67% men, mean age 51 years old, mean evolution 1.4 years. The general indicators supported the understanding of the version and the absence of conflicting items. Discussion: The version obtained demonstrated its conceptual content validity regarding the English version, presenting not semantic nor pragmatic conflicts affecting their use in an Argentine population of ALS patients.

Clinical attention and assistance profile of patients with amyotrophic lateral sclerosis / Perfil clínico e assistencial de pacientes com esclerose lateral amiotrófica

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 percent), parents/children/cousins (20.7 percent), friends (10.3 percent) and private nurses (3.5 percent); 10.3 percent 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.

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 por cento), pais/filhos/primos (20,7 por cento), amigos (10,3 por cento), particular (3,5 por cento); 10,3 por cento 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.

Amyotrophic lateral sclerosis: combined nutritional, respiratory and functional assessment / Esclerose lateral amiotrófica: correlações dos indicadores da avaliação nutricional, funcional e respiratória

OBJECTIVE: To establish correlations between nutritional, functional and respiratory indices of patients with amyotrophic lateral sclerosis (ALS). METHOD: Twenty patients (13 appendicular - GA and 7 bulbar - GB) were included in the multidisciplinary study at the Neurological Clinic Ambulatory of the University of Campinas Hospital. RESULTS: Among the GA type significant correlation was observed between maximal inspiratory (MIP) and expiratory (MEP) pressure (r= -0.76), MEP and pulse oxymetry (r=0.58), MIP and percent weight loss ( percentWL; r=0.59), and between MIP, total and subscale respiratory scores (ALSFRS-R) with percentWL. With regard to the GB, correlation was found between MEP and body mass index (BMI) (r=0.97). In both GA and GB correlations were noticed between the BMI and the variables mass (kg), fat ( percent), arm and wrist circumference (cm), and tricipital, subscapular and supra-iliac skinfolds (mm), as well as the arm muscle circumference (cm) and fatty arm muscular area (mm²). CONCLUSION: It is suggested that the application of simple anthropometric measurements could be useful in routine monitoring of patients with ALS.

OBJETIVO: Correlacionar os indicadores utilizados na avaliação nutricional, funcional e respiratória de indivíduos com esclerose lateral amiotrófica (ELA). MÉTODO: Vinte pacientes (13 apendiculares - GA e 7 bulbares - GB) foram incluídos no estudo usando parâmetros nutricionais, respiratórios e escala funcional (ALSFRS-R). RESULTADOS: Entre os pacientes do GA, as correlações observadas foram: pressão inspiratória máxima (PImax) e expiratória máxima (PEmax) (r= -0,76); PEmax e oximetria de pulso (r=0,58); PImax e porcentagem de perda de peso ( por centoPP) (r=0,59); e entre PImax, escore ALSFRS-R com por centoPP. No GB, houve correlação entre MEP e índice de massa corporal (IMC) (r=0,97). Em GA e GB, observaram-se correlação entre IMC e as variáveis: massa, gordura ( por cento), circunferência braquial e punho, pregas cutâneas tricipital, subescapular e supra-ilíaca, circunferência muscular do braço (cm), área muscular gordurosa do braço (mm²). CONCLUSÃO: Sugere-se a aplicação deste conjunto de medidas durante a evolução clínica de indivíduos com ELA.

Korean version of Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised: A Pilot Study on the Reliability and Validity

BACKGROUND: The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) is a quantitative measure of ALS-related physical deficit with established reliability and validity in prospective clinical research. This study aimed to test the reliability and validity of the Korean version of ALSFRS-R (K-ALSFRS-R). METHODS: The subjects of the study were 51 patients with ALS diagnosed on the basis of El Escorial criteria. The patients were evaluated by one rater using K-ALSFRS-R, Norris scale, and Appel ALS rating scale on the same day. Subsequently, K-ALSFRS-R was measured by two other raters using a videotape design. Test-retest data were obtained within the time interval of 7 days. RESULTS: Internal consistency and test-retest reliability were high. Inter-rater comparisons showed significant reliability with Kappa or Kendall's tau-b value. Moreover, K-ALSFRS-R scores correlated significantly with clinical status as measured by Norris and Appel ALS rating scale. CONCLUSIONS: Our results showed that K-ALSFRS-R would be a reliable and useful instrument for the evaluation of functional status in patients with ALS.

Availability of Neurophysiological Index in Amyotrophic Lateral Sclerosis: Application of Neurophysiological Index to Median Nerve /Abductor Digiti Minimi and Posterior Tibial Nerve/Abductor Hallucis

BACKGROUND: Neurophysiological Index (NI) is derived from compound muscle action potentials, distal motor latency and F-wave frequency in the ulnar nerve/abductor digiti minimi. Recent studies suggested that NI could be used as a sensitive measure of change during the course of ALS. However, the NI has several limitations which include being derived only from the ulnar nerve territory and is less valuable when atrophic change of the abductor digiti minimi is severe. Thus, this study aimed to evaluate the correlation of NI with the functional status of ALS patients and the availability of NI obtained from median and posterior tibial nerve territory. METHODS: The subjects of the study were 25 patients with ALS. The NI, Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) and grip power were obtained during the same period and NI values were compared with ALSFRS-R and grip power. RESULTS: A significant correlation was observed between ALSFRS-R and various NI values. Moreover, NI values derived from the ulnar nerve/abductor digiti minimi and the median nerve/abductor pollicis brevis on the dominant hand were statistically correlated with fine motor function scores in ALSFRS-R, and NI values from posterior tibial nerve/abductor hallucis were significantly correlated with lower limb function scores. CONCLUSIONS: Our results support the previous findings that NI is a useful neurophysiological measurement in ALS patients. Moreover, we might suggest NI values from the median nerve/abductor pollicis brevis and the posterior tibial nerve/abductor hallucis also could be used in measuring the functional status of ALS patients.