Esclerosis lateral amiotrófica esporádica de inicio juvenil

Orsini, Marco; Mr, Freitas; As, Oliveira; Mp, Mello; Ma, Chieia; Reis, Melo; Am, Catharino; Ma, Leite; Oj, Nascimento

doi:10.33588/rn.5007.2009268

RevNeurol

2010

DOI: 10.33588/rn.5007.2009268

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Esclerosis lateral amiotrófica esporádica de inicio juvenil

Marco Orsini¹,

Freitas Mr²,

Oliveira As³

et al.

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Clinical and Functional Profile of Amyotrophic Lateral Sclerosis Patients: A One Year Follow Up

Orsini¹,

Freitas²,

Nascimento³

et al. 2011

American Journal of Neuroscience

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Problem statement: Amyotrophic Lateral Sclerosis (ALS) is a progressive and degenerative disease that affects the anterior horn motor neurons of the spinal cord and pyramidal tracts. In Brazil, there are few epidemiological data on this disease. Recently, some important findings have been reported, allowing a better understanding on the underlying processes of neuronal death, as well as the characteristics of this population. To discuss the clinical and functional profile of a convenience sample of patients with ALS in Rio de Janeiro and Neurology Department-Federal Fluminense University to compare the data with studies of other regions and countries. Approach: We used the Severity and Functional Ability Scale (SFAS) as a clinical and functional indicator for ALS. The modified El Escorial criteria were used to establish the diagnosis. The participants underwent five quarterly assessments during the study period. The research took place at two University Hospitals (Hospital Universitario Antonio Pedro-Universidade Federal Fluminense and Instituto de Neurologia Deolindo Couto-Universidade Federal do Rio de Janeiro) from March 2007 to December 2009. Results: Of the 98 recruited subjects, only 24 have completed all phases of the study. The average age was 52.7±4.1 years. The time between the onset of first symptoms and seeking care services was 11.6±12.:4 months. The time between the first symptoms and the diagnosis was 20.5±8.4 months. Muscle weakness was identified as the initial symptom in most cases. Patients had impaired muscle strength, speech, swallowing, respiratory function and severity stages of SFAS. The disease had different forms of initial presentation (impaired speech, limbs strength, respiratory function or swallowing), time to progression and clinical characteristics in our population. Conclusion: The knowledge on the individual clinical evolution in ALS is of paramount importance for the healthcare team to provide a correct treatment during the decline of Am. J. Neuroscience 2 (1): [28][29][30][31][32][33][34] 2011 29 the disease and formulate theoretical and conceptual issues, aiming at solving problems in clinical practice.

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Clinical and Functional Profile of Amyotrophic Lateral Sclerosis Patients: A One Year Follow Up

Orsini¹,

Freitas²,

Nascimento³

et al. 2011

American Journal of Neuroscience

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Amyotrophic lateral sclerosis: one or multiple causes?

Bastos¹,

Orsini

Machado³

et al. 2011

Neurol Int

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The Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in the adulthood, and it is characterized by rapid and progressive compromise of the upper and lower motor neurons. The majority of the cases of ALS are classified as sporadic and, until now, a specific cause for these cases still is unknown. To present the different hypotheses on the etiology of ALS. It was carried out a search in the databases: Bireme, Scielo and Pubmed, in the period of 1987 to 2011, using the following keywords: Amyotrophic lateral sclerosis, motor neuron disease, etiology, causes and epidemiology and its similar in Portuguese and Spanish. It did not have consensus as regards the etiology of ALS. Researches demonstrates evidences as regards intoxication by heavy metals, environmental and occupational causes, genetic mutations (superoxide dismutase 1), certain viral infections and the accomplishment of vigorous physical activity for the development of the disease. There is still no consensus regarding the involved factors in the etiology of ALS. In this way, new research about these etiologies are necessary, for a better approach of the patients, promoting preventive programs for the disease and improving the quality of life of the patients.

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Amyotrophic lateral sclerosis: new perpectives and update

et al. 2015

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Amyotrophic lateral sclerosis (ALS), Charcot’s disease or Lou Gehrig’s disease, is a term used to cover the spetrum of syndromes caracterized by progressive degeneration of motor neurons, a paralytic disorder caused by motor neuron degeneration. Currently, there are approximately 25,000 patients with ALS in the USA, with an average age of onset of 55 years. The incidence and prevalence of ALS are 1-2 and 4-6 per 100,000 each year, respectively, with a lifetime ALS risk of 1/600 to 1/1000. It causes progressive and cumulative physical disabilities, and leads to eventual death due to respiratory muscle failure. ALS is diverse in its presentation, course, and progression. We do not yet fully understand the causes of the disease, nor the mechanisms for its progression; thus, we lack effective means for treating this disease. In this chapter, we will discuss the diagnosis, treatment, and how to cope with impaired function and end of life based on of our experience, guidelines, and clinical trials. Nowadays ALS seems to be a more complex disease than it did two decades – or even one decade – ago, but new insights have been plentiful. Clinical trials should be seen more as experiments on pathogenic mechanisms. A medication or combination of medications that targets more than one pathogenic pathway may slow disease progression in an additive or synergistic fashion.

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Esclerosis lateral amiotrófica esporádica de inicio juvenil

Cited by 3 publications

References 0 publications

Clinical and Functional Profile of Amyotrophic Lateral Sclerosis Patients: A One Year Follow Up

Clinical and Functional Profile of Amyotrophic Lateral Sclerosis Patients: A One Year Follow Up

Amyotrophic lateral sclerosis: one or multiple causes?

Amyotrophic lateral sclerosis: new perpectives and update

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