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Non-Commercial Use Only Neurology International 2015; volume 7:5885 Amyotrophic lateral sclerosis: Introduction Correspondence: Marco Orsini, Programa de Pós- new perpectives and update Graduação em Ciências da Reabilitação, Praça The technological breakthrough in the field das Nações, 34, Bonsucesso, Rio de Janeiro, CEP: Marco Orsini,1,2 Acary Bulle Oliveira 3 of neurolog /neuroscience, with modern imag- 21041021, Brasil. Osvaldo J.M. Nascimento,1 , ing and genetic studies, may, sometimes, E-mail: [email protected] Carlos Henrique Melo Reis 1 make the neurologist stay away from indispen- Key words: Amyotrophic lateral sclerosis; neu- Marco Antonio Araujo Leite, 1 sable propaedeutic techniques for the correct rodegenerative diseases; diagnosis; treatment; 1 1 Jano Alves de Souza Camila, Pupe diagnosis of amyotrophic lateral sclerosis rehabilitation. Olivia Gameiro de Souza, 1 , (ALS). ALS is without doubt a disease of the Victor Hugo Bastos 4 , central nervous system (CNS), which its natu- Contributions: the authors contributed equally. Marcos R.G. de Freitas, 1 Silmar Teixeira 4 ral history is one of the darkest in neurology. A progressive, devastating and inexorable dis- Carlos Bruno 1 Eduardo, Davidovich 1 , Conflict of interest: the authors declare no poten- ease, commonly leads to death by respiratory tial conflict of interest. Benny Smidt3, , failure a few years after onset of first symp- 1Neurology Department, Universidade toms. Rowland,1 centuries ago, defines its nat- Received for publication: 25 February 2015. Federal Fluminense, Rio de Janeiro; ural history well by stating that any notifica- Accepted for publication: 15 June 2015. 2Programa de Mestrado em Ciências da tion of improvement in patients with this dis- This work is licensed under a Creative Commons ease deserves careful review, because probably Reabiitação – UNISUAM, Rio de Janeiro; Attribution NonCommercial 3.0 License (CC BY- 3Neurology Department, Universidade it is not a case of ALS. Prompt diagnosis, sen- NC 3.0). Federal de São Paulo – UNIFESP, São sitive communication of the diagnosis, the involvement of the patient and their family, Paulo; 4Neuroscience Department, ©Copyright M. Orsini et al., 2015 and a positive care plan are pre requisites for Licensee PAGEPress, Italy Universidade Federal do Piaiu, Parnaiba, good clinical management. While ALS is an Neurology International 2015; 7:5885 Brazil incurable disease, many symptoms are doi:10.4081/ni.2015.5885 amenable to supportive and adjunctive thera- only pies, some of which may even improve the dis- U.S. alone.6 A high prevalence of ALS cases is ease course.2 Nowadays, ALS is considered a reported in certain geographical areas, for Abstract multisystemic disease with broad pathophysio- example the Pacific island of Guam (50 times logical framework and numerous theoriesuse that that of ALS in western countries), leading to Amyotrophic lateral sclerosis (ALS), surround it, hampering a unique therapeutic speculation about environmental and genetic target.3 These pathophysiological mechanisms Charcot’s disease or Lou Gehrig’s disease, is a factors as potential triggers for ALS. People include oxidative stress, mitochondrial impair- term used to cover the spetrum of syndromes over fifty are the most affected. Men are affect- ment, protein aggregation, cytoskeletal disrup- caracterized by progressive degeneration of ed nearly twice as often as women, with no motor neurons, a paralytic disorder caused by tion, glutamate and neuronal cytotoxicity, altered regulation of gene expression, inflam- racial differences. motor neuron degeneration. Currently, there 7 mation, and apoptotic cell death. An under- A study by Orsini et al., that aimed to delin- are approximately 25,000 patients with ALS in eate the clinical and functional profile of the USA, with an average age of onset of 55 standing of how these potential therapeutic patients with ALS in Brazil and compare with years. The incidence and prevalence of ALS are targets interrelate will provide direction both other regions of the world, identified a rapid 1-2 and 4-6 per 100,000 each year, respectively, in the development of a pharmacotherapy and 4 depletion of functional capacity, muscle with a lifetime ALS risk of 1/600 to 1/1000. It in the design of clinical trials. Countless experts in the field, for example, the group strength, swallowing and breathing pattern. In causes progressive and cumulative physical conducted by Oliveira and Pereira,5 consider that study, the onset of ALS is often insidious disabilities, and leads to eventual death due to that a combination of drugs focused on more and can manifest by unexplained trip or motor respiratory muscle failure. ALS is diverse in its than one pathogenic pathway may slow dis- disabilities, usually in the distal arm. Some presentation, course, and progression. We do ease progression in an additive or synergistic not yet fully understand the causes of the dis- patients with bulbar onset have difficulty in Non-commercialfashion. It is noteworthy that such combina- ease, nor the mechanisms for its progression; swallowing and changes in voice tonality. The tion therapy has been successful in oncology, thus, we lack effective means for treating this time between the onset of first symptoms and though multiple drug interactions and seeking care services was 11.6±12.4 months. disease. In this chapter, we will discuss the increased incidence of drug side effects should diagnosis, treatment, and how to cope with The time between the first symptoms and the be considered. The risk for benefit ratio should diagnosis was 20.5±8.4 months. The author impaired function and end of life based on of also be considered. demonstrates that the findings related to ALS our experience, guidelines, and clinical trials. Histopathological findings reveal an impair- presentation in the subgroup of European Nowadays ALS seems to be a more complex ment of the motor neurons of the pyramidal countries (Italy, Germany, Spain) have some disease than it did two decades – or even one beam, the brainstem and spinal cord in varying decade – ago, but new insights have been plen- degrees. Although ALS are readily recognized similarity with the characteristics of other tiful. Clinical trials should be seen more as by neurologists, about 10% of patients are mis- industrialized countries as, for example, in experiments on pathogenic mechanisms. A diagnosed, and delays in diagnosis are com- Brazil. To sum up, ALS is part of the so-called medication or combination of medications that mon.5 motor neuron disease (MND) disease, along targets more than one pathogenic pathway The disease has several features in the dif- with progressive spinal amyotrophy (PSA) and may slow disease progression in an additive or ferent presentation forms, course and progres- primary lateral sclerosis. The progressive bul- synergistic fashion. sion. The incidence is approximately two cases bar palsy (PBP), does not fail to take part in per 100,000 inhabitants, which represents the spectrum of presentation of classical ALS, approximately 5000 patients per year in the so, it should be studied together.8 [Neurology International 2015; 7:5885] [page 39] Review anterograde and retrograde axonal transport, onset is slow and progressive, alveolar Classification and microglial activation, inflammation, and hypoventilation typically goes undiagnosed physiopathogenesis growth factor deficiency. and untreated until an episode of acute respi- Several factors are proposed to instigate ratory failure occurs. This episode of decom- The ALS cases can be classified into spo- these phenomena, including latent infections pensation is frequently seen during common radic, familial, and from the western Pacific by viral and non-viral agents, toxins (for exam- upper airway infections, and it results from (ALS and Parkinsonism-Dementia Complex), ple, insecticides and pesticides) and autoim- patient incapacity to eliminate secretions. At 9 the latter very common in Chamorro people of mune reactions. Genetic factors, changes in the moment that the ventilatory muscles are Guam and Marianas island, the Kii peninsula intracellular calcium levels in motor neurons, compromised, individuals present pulmonary of Honshu Island, and the Auyu and Jakai peo- and programmed cell death (apoptosis) have restrictions, characterized by reduced vital 12,13 ple of south west New Guinea. In around 5-10% also been linked to the development of ALS. capacity (VC) and tidal volume (TV) with con- there is evidence of family history (familial sequently chronic respiratory failure. ALS), and, approximately 20% of these variants Atelectasis, pneumonia and respiratory failure, are linked to the gene encoding the enzyme initially during sleep and later on even during copper-zinc superoxide dismutase (Cu-Zn Clinical profile wakefulness, are the complications expected 21 SOD1), and 2-5% have mutations of TARDBP in this situation. (TDP-43) gene. The clinical presentations of patients do not ALS can produce sleep alterations, since Commonly these cases show Mendelian follow a pattern, although some initial mani- alveolar hypoventilation is more intense dur- autosomal dominant inheritance. However, festations are present in most cases. ing sleep. The worsening of alveolar air autosomal recessive patterns have also been Unexplained tripping (slight foot drop) with or exchange presents subtle symptoms that can identified.8 Investigations of mutant SOD1 without episodes of falls in addition to impair- pass unnoticed if not directly analyzed.
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