Navegando por Palavras-chave "Amyotrophic Lateral Sclerosis"
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- ItemAcesso aberto (Open Access)Ação da terapia hormonal na Esclerose Lateral Amiotrófica: revisão sistemática(Universidade Federal de São Paulo (UNIFESP), 2019-12-18) Vasconcelos, Katia De [UNIFESP]; Simoes, Manuel De Jesus [UNIFESP]; Oliveira, Acary Souza Bulle [UNIFESP]; Fuchs, Luiz Fernando Portugal [UNIFESP]; Girão, Manoel João Batista Castello [UNIFESP]; http://lattes.cnpq.br/3911841387107665; http://lattes.cnpq.br/0822381310382371; http://lattes.cnpq.br/0973903299568770; http://lattes.cnpq.br/5987164343458678; Universidade Federal de São Paulo (UNIFESP)Amyotrophic Lateral Sclerosis (ALS), a fatal disease characterized by muscle and fasciculations weakness and atrophy and decreased reflexes due to upper and lower motor neurons death. It can be present in both sexes (55-65 years), but with predominance in males. However, in female patients, ALS presents its first symptoms when they are already postmenopausal, when then the incidence ratio of the disease is practically equal between the sexes. Which leads to a probable involvement of sex hormones in the development and protection against ALS. The aim of this systematic review, which used the PRISMA consensus and NOS (New Casttle-Ottawa Scale) score, was to evaluate the evidence of the action of hormone therapy in women with ALS. The Medline and Cochrane databases were accessed from March 2019 to June 2019, and only full text articles in Spanish, English and Portuguese languages were included. Only four articles matched our inclusion criteria. Postmenopausal women who used exogenous estrogen did not have the same protective factor as women still under the action of endogenous estrogen in the same age group. There was also no increase in survival of these women.
- ItemSomente MetadadadosAnálise Da Interface Entre A Disfagia E As Implicações Nutricionais Nos Pacientes Com Doença Do Neurônio Motor(Universidade Federal de São Paulo (UNIFESP), 2017-01-31) Alves, Percilia Cardoso Lopes [UNIFESP]; Oliveira, Acary Souza Bulle [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Introduction: Amyotriphic Lateral Sclerosis (ALS)is a disease in which there is impairment of the lower and upper motor neurons and the Bulbar Progressive Palsy (BPP) of the lower motor neuron of the brainstem; Both are within the Motor Neuron Disease (MND) group. Muscle weakness is a common sign that can trigger dysphagia. Purpose: To analyze the interaction between dysphagia and nutritional implications in the patient with MND. Method: Through observational, transversal, analytical and prospectiveresearch, 59 patients were evaluated, being 42 (71.18%) with ALS (47.62% male and 52.38%, female) and 17 (28.82%) with BPP (7 (41.17%) male and 17 (58.83%) female. The patients underwent speech therapy and nutritional evaluation; moreover, have been applied: functional range of consistencies (FOIS), scales of functionality (ALSFRS-R and EGELA) and respiratory assessment (peak cough flow).Results: 100% of patients with BPP presented oral and pharyngeal phase changes, while patients with ALS had less changes in oral (66.67%) and pharyngeal phase (73.80%). In nutritional evaluation, malnutrition was observed in 35.71% of patients with ALS and 23.52% of the patients with BPP. 28% (ALS) and 41.17% (BPP) of the patients made use of enteral route.Conclusion: Dysphagia was present in all patients with diagnosis of BPP, relating to increased frequency of malnutrition. Body mass index and Protein Energy Malnutrition score showed correlation with ALSFRS-R functionality. There was correlation between body mass indexes, the scale of food consistency (FOIS) and the peak cough flow.
- ItemSomente MetadadadosMunix Nas Miopatias(Universidade Federal de São Paulo (UNIFESP), 2018-08-30) Sousa, Manoel Wilkley Gomes De [UNIFESP]; Manzano, Gilberto Mastrocola [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Introduction: Munix Is A New Technique That Provides An Index That Is Related To The Number Of Motor Units, Which Has Been Developed In Recent Years And Has Been Evaluated In Studies That Seek To Establish Its Clinical Applicability. It Has Been Shown That Such A Method Is Capable Of Detecting The Loss Of Motor Units In Disorders Leading To Denervation. However, It Is Not Yet Known How The Technique Behaves In Disorders That Lead To Muscle Weakness Not Necessarily Associated With Loss Of Motor Units, As Is The Case Of Myopathies. Objectives: To Evaluate How Munix Behaves In Myopathies Something Not Yet Described, Which Can Bring A Better Understanding Of The Technique. In Addition, As A Secondary Objective, To Differentiate The Relative Variations In The Number Of Motor Units (Through Munix) In Different Types Of Myopathies. Methods: This Is A Case-Control Study Involving Patients With Confirmed Diagnosis Of Myopathy And Healthy Individuals. We Selected Patients With Limb-Girdle Muscular Dystrophy (Lgmd) Or W
- ItemAcesso aberto (Open Access)Suplementação nutricional em pacientes com doença do neurônio motor/esclerose lateral amiotrófica(Universidade Federal de São Paulo (UNIFESP), 2003) Stanich, Patricia [UNIFESP]; Oliveira, Acary Souza Bulle [UNIFESP]A Doenca do Neuronio Motor (DNM) e uma doenca degenerativa, progressiva, irreversivel e incuravel, com envolvimento primario do motoneuronio. Dentre as formas de manifestacao, predominam a Esclerose Lateral Amiotrofica (ELA) e a Paralisia Bulbar Progressiva (PBP). Na evolucao clinica, a disfagia apresenta-se como um dos comprometimentos principais, levando a diminuicao da massa corporea, desidratacao e desnutricao. Associado a diminuicao da inGestão alimentar, observa-se o aumento da taxa de metabolismo basal e das necessidades nutricionais, determinando assim perda de peso. O reconhecimento da deplecao nutricional e intervencao precoce podem ser um diferencial no tratamento. Nesse trabalho foram estudados 20 pacientes com Doenca do Neuronio Motor (DNM)/Esclerose Lateral Amiotrofica (ELA), provenientes do Setor de Investigacao de Doencas Neuromusculares da Disciplina de Neurologia Clinica da Universidade Federal de São Paulo - Escola Paulista de Medicina (UNIFESP-EPM). O objetivo desse estudo foi descrever o estado nutricional dos pacientes com Doenca do Neuronio Motor (DNM) /Esclerose Lateral Amiotrofica (ELA), submetidos a suplementacao nutricional. Os pacientes receberam suplementacao nutricional durante seis meses consecutivos. Eles foram submetidos a avaliacao nutricional bimestral, com medidas antropometricas - peso, estatura, circunferencia do braco e dobra cutanea do triceps - e analise da composicao corporal atraves da bioimpedancia eletrica. O diagnostico de ELA foi estabelecido de acordo com -os criterios propostos pelo El Escorial, modificado era 1998, atraves do quadro clinico e do auxilio de exames como eletroneuromiografia, ressonancia magnetica e tomografia computadorizada. As idade variou entre 36 e 70 anos, com media de 55,0, mediana de 59,5 e desvio padrao de 12,4 anos. O tempo referido de doenca pelo paciente variou de 8 a 218 meses, com media de 31,5 meses. Quinze pacientes (75 por cento) apresentavam ELA como forma de manifestacao, sendo onze (73,3 por cento) do sexo masculino e 4 (26,6 por cento) do sexo feminino...(au)