Relação entre degeneração do trato córtico-espinhal através de ressonância magnética e escala funcional (ALSFRS) em pacientes com esclerose lateral amiotrófica

Garcia, Larissa Nery; Silva, Alexandre Vallota da; Carrete, Henrique; Fávero, Francis Meire; Fontes, Sissy Veloso; Moneiro, Marcelo Tavares; Oliveira, Acary Souza Bullé

doi:10.1590/s0004-282x2007000500029

Cited by 10 publications

(5 citation statements)

References 31 publications

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“…The findings of a bilateral symmetrical hyper intensity of the pyramidal tract are suggestive of Als denoting degeneration of corticospinal tracts 11,12 . considering the clinical and laboratory findings, the motor neuron diseases have been classified as Als / DNM (sporadic cases, family or genetically determined), Alsplus syndromes (multisystem neurodegenerative disease affecting motor neurons), the Als -related syndromes (represent symptomatic or secondary forms of motor neuron disease, with a known associated condition that may be causing the disease) and the Als -variants (are uncommon unless the patient lives in particular geographic locations) ( Table 9) 13,14 .…”

Section: Diagnosismentioning

confidence: 99%

Amyotrophic lateral sclerosis (ALS): three letters that change the people's life. For ever

Oliveira

Pereira

2009

Arq. Neuro-Psiquiatr.

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-Amyotrophic lateral sclerosis (Als) is a neurodegenerative disease affecting the motor nervous system. it causes progressive and cumulative physical disabilities in patients, and leads to eventual death due to respiratory muscle failure. The disease is diverse in its presentation, course, and progression. We do not yet fully understand the cause or causes of the disease, nor the mechanisms for its progression; thus, we lack effective means for treating this disease. currently, we rely on a multidisciplinary approach to symptomatically manage and care for patients who have Als. Although amyotrophic lateral sclerosis and its variants are readily recognized by neurologists, about 10% of patients are misdiagnosed, and delays in diagnosis are common. prompt diagnosis, sensitive communication of the diagnosis, the involvement of the patient and their family, and a positive care plan are prerequisites for good clinical management. A multidisciplinary, palliative approach can prolong survival and maintain quality of life. Treatment with riluzole improves survival but has a marginal effect on the rate of functional deterioration, whereas non-invasive ventilation prolongs survival and improves or maintains quality of life. in this review, we discuss the diagnosis, management, and how to cope with impaired function and end of life on the basis of our experience, the opinions of experts, existing guidelines, and clinical trials. Multiple problems require a multidisciplinary approach including aggressive symptomatic management, rehabilitation to maintain motor function, nutritional support (enteric feeding, gastrostomy), respiratory support (non invasive home ventilation, invasive ventilation, tracheotomy), augmentative communication devices, palliative care, psychological support for both patients and families (because family members so often play a central role in management and care), communication between the care team, the patient and his or her family, and recognition of the clinical and social effects of cognitive impairment. social, bioethical, and financial issues as well as advance directives should be addressed. A plethora of evidence-based guidelines should be compiled into an internationally agreed guideline of best practice. The multidisciplinary team has changed the history of disease, with still no curative therapy available.KeY WorDs: amyotrophic lateral sclerosis, diagnosis, treatment.Esclerose lateral amiotrófica (ELA): três letras que mudam a vida de uma pessoa. Para sempre.Resumo -A esclerose lateral amiotrófica (elA) é doença neurodegenerativa comprometendo o sistema nervoso motor. ela causa comprometimento físico, progressivo e acumulativo, com óbito freqüentemente decorrente de falência respiratória. A enfermidade apresenta características diversas nas formas de apresentação, curso e progressão. Não entendemos ainda a causa ou causas dessa enfermidade, nem os mecanismos que regem a sua progressão; assim, tratamentos efetivos não são, até o momento, conhecidos. Atualmente, recomenda-se que os...

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Section: Diagnosismentioning

confidence: 99%

Amyotrophic lateral sclerosis (ALS): three letters that change the people's life. For ever

Oliveira

Pereira

2009

Arq. Neuro-Psiquiatr.

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“…The MRI examination with sequences ST1 with MTC and transcranial magnetic stimulation (TMS) has identified abnormalities in the cortical spinal tract, thus becoming a useful tool in ALS diagnosis 26 . In patients where there is no clinically defined involvement of motor tract, image and TMS analysis must be requested.…”

Section: Clinical Forms Of Presentationmentioning

confidence: 99%

Amyotrophic lateral sclerosis: considerations on diagnostic criteria

Chieia

Oliveira

Silva

et al. 2010

Arq. Neuro-Psiquiatr.

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder, compromising the motor neuron, characterized by progressive muscle weakness, with reserved prognosis. The diagnosis is based on inclusion and exclusion clinical criteria, since there is no specific confirmation test. The objective of this research is to critically examine the main diagnosis instrument -El Escorial revisited, from the World Federation of Neurology (1998). Of the 540 patients with initial ALS diagnosis, either probable or definite, seen at UNIFESP-EPM, 190 underwent thorough investigation, following regular clinical and therapeutic treatment for over two years. Thirty patients (15.78%) had their diagnosis completely changed. The false-positive diagnoses were related to: early age, clinical presentation of symmetry, weakness greater than atrophy, symptomatic exacerbation. In addition, three patients with myasthenia gravis developed framework for ALS, suggesting the post-synaptic disability as a sign of early disease. Key words: amyotrophic lateral sclerosis, correct diagnosis, El Escorial revisited.Esclerose lateral amiotrófica: considerações sobre critérios diagnósticos RESUMO Esclerose lateral amiotrófica (ELA) é uma doença neurodegenerativa, que compromete o neurônio motor, caracterizada por fraqueza muscular progressiva, com prognóstico reservado. O diagnóstico é baseado na inclusão e exclusão de critérios clínicos, uma vez que não existe um teste de confirmação específica. O objetivo desta pesquisa é analisar criticamente o instrumento de diagnóstico principal -El Escorial revisited, da Federação Mundial de Neurologia (1998). Dos 540 pacientes com diagnóstico inicial de ELA, seja provável ou definitiva, vistos pela UNIFESP-EPM, 190 foram submetidos a investigação aprofundada, após tratamento clínico e terapêutico regular há mais de dois anos. Trinta pacientes (15,78%) tiveram seu diagnóstico mudado completamente. Os diagnósticos falso-positivos foram relacionados à idade precoce, a apresentação clínica da simetria, a fraqueza superior a atrofia, exacerbação sintomática. Além disso, três pacientes com miastenia gravis desenvolveram quadro de ELA, sugerindo a lesão pós-sináptica como um sinal precoce da doença. Palavras-chave: esclerose lateral amiotrófica, diagnóstico correto, El Escorial revisited. CorrespondenceMarco Antonio Troccoli Chieia Rua Mairinque 267 04037-020 -São Paulo SP -Brasil

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“…The disease affects the fibers along the corticospinal tract, which transmit impulses that control voluntary movements. These patients have progressive muscle weakness with a reserved prognosis, absent or diminished deep reflexes, fasciculations and spasticity (1)(2)(3) .…”

Section: Introductionmentioning

confidence: 99%

Quality of life of patients with Amyotrophic Lateral Sclerosis

Siqueira¹,

Vitorino²,

Prudente³

et al. 2017

Rev Rene

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Objective: to analyze the scientific evidence that evaluated the quality of life of patients with Amyotrophic Lateral Sclerosis through ALSAQ-40/ALSAQ-5. Methods: this is an integrative review carried out with eight articles in the database, using controlled keywords. Results: the most impaired domains of quality of life were physical mobility and activities of daily living. Patients with bulbar Amyotrophic Lateral Sclerosis, communication and feeding were more impaired. Problems with health care and lack of social support to the caregiver negatively impacted the patient´s quality of life. Emotional stress was correlated with communication, physical mobility and disease progression in patients with breathing and artificial feeding, but it is not directly related to the advances of physical deterioration, but rather how the patient processes the experiences. Conclusion: patients with Amyotrophic Lateral Sclerosis have a poorer quality of life regarding the motor aspect, and lack of caregiver support worsens the patient´s quality of life. Descriptors: Quality of Life; Amyotrophic Lateral Sclerosis; Nursing. Objetivo: analisar as evidências científicas que avaliaram a qualidade de vida de pacientes com Esclerose Lateral Amiotrófica por meio do ALSAQ-40/ALSAQ-5. Métodos: revisão integrativa realizada com oito artigos em base de dados, utilizando-se descritores controlados. Resultados: os domínios mais prejudicados da qualidade de vida foram mobilidade física e atividades de vida diária. Os pacientes com Esclerose Lateral Amiotrófica bulbar, comunicação e alimentação foram mais prejudicados. Problemas com a assistência à saúde e a falta de apoio social ao cuidador impactaram negativamente a qualidade de vida do paciente. Estresse emocional correlacionou com a comunicação, mobilidade física e progressão da doença em pacientes com respiração e alimentação artificial, mas não está diretamente relacionado aos avanços da deterioração física e sim como o paciente processa as experiências. Conclusão: pacientes com Esclerose Lateral Amiotrófica têm pior qualidade de vida referente ao aspecto motor; e a falta de suporte ao cuidador piora a qualidade de vida do paciente. Descritores: Qualidade de Vida; Esclerose Amiotrófica Lateral; Enfermagem.

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Relação entre degeneração do trato córtico-espinhal através de ressonância magnética e escala funcional (ALSFRS) em pacientes com esclerose lateral amiotrófica

Cited by 10 publications

References 31 publications

Amyotrophic lateral sclerosis (ALS): three letters that change the people's life. For ever

Amyotrophic lateral sclerosis (ALS): three letters that change the people's life. For ever

Amyotrophic lateral sclerosis: considerations on diagnostic criteria

Quality of life of patients with Amyotrophic Lateral Sclerosis

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