Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease of unknown etiology characterized by death of motor neurons. In the last 20 years, over 25 ALS genes were discovered. 10% of cases are considered to be familial cases (FALS) with the majority inherited as autosomal dominant. In Western countries, ALS is considered an adult onset disease with a mean age of 65, while in developing countries onset is approximately 10 years earlier. Similarly, survival is reportedly on average 9 months less in South America compared to Europe and North America. ALS is more frequent in males than females with an incidence in Europe and North America of 1.5 to 2.7 cases per 100,000 individuals and a prevalence of 2.7 to 7.4 (/100,000 individuals). Preliminary studies in the Caribbean and South America suggest a lower incidence of ALS in these regions. The diagnosis of ALS is based on signs and symptoms of degeneration of upper and lower motor neurons, the absence of alternative explanations, and progression of symptoms. Electrophysiology is essential for early diagnosis, in addition to other neuroimaging and laboratory tests. In this review, we summarize the literature concerning the El Escorial criteria for diagnosis and the electromyography.
Artículos de Revisión
El Estrés Crónico, ¿Factor de Riesgo para la Enfermedad de Alzheimer?
Alzheimer’s disease (AD) is a progressive neurodegenerative disorder, with epidemiological importance due to the high prevalence of people affected worldwide. Over time, various hypotheses have been raised in the pathophysiology and etiology of the disease and now the study of this disorder, is tackled from a multi-causal perspective, taking into account different etiological factors, among which are: genetics, oxidative stress, intracellular calcium dynamics, vascular effects, inflammation and stress, among others. The following literature review, aims to show studies that correlate stress as a risk factor in AD, recognizing the pathophysiological findings of AD, due to augmentation on glucocorticoids by chronic stress and the subsequent alteration of the hypothalamic-pituitary-adrenal axis. Critical view of these findings according to the literature.
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Neurobiología del Estrés Agudo y Crónico: Su Efecto en el Eje Hipotálamo-Hipófisis-Adrenal y la Memoria.
The presently available data about stress and its effects is too broad. That’s why this paper will focus on the effect of stress hormones in some brain areas, like limbic structures and the hypothalamo-pituitary-adrenal axis. In this way we will discuss how repetitive stress can change those areas, mainly by the effect on its two types of nuclear receptors, changing the basal activity of the amygdala, hippocampus and the medial prefrontal cortex, resulting in an increased hypothalamo-pituitaryadrenal axis function and impairing cognitive functions like memory. However, the exact mechanism by which these effects are produced is poorly understood. That is why one of the challenges for future research is to link the cellular changes with its behavioral effects in order to understand how it works in a living organism.
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Consideraciones en la Monitorización Intraoperatoria del Nervio Facial.
Currently, intraoperative facial nerve monitoring practice is considered a complementary part during surgery of cerebellopontine lesions and skull base surgery to decrease the likelihood of functional and aesthetic sequelae. It is therefore essential to understand the main technical and neurophysiological aspects for appropriate performance. Intraoperative monitoring requires joined efforts of neurosurgeon, otologist, neurologist and neurophysiologist to increase the chances of success.
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Disfagia en el Paciente con Enfermedad Cerebrovascular.
Dysphagia in patients with cerebrovascular disease is a common problem that is associated with increased rates of pneumonia, malnutrition, hospital stay and mortality. A literature search was conducted in MEDLINE and Cochrane Library for recommendations for assessment and management of dysphagia in patients with stroke. Because the evidence is poor in quality, hence doubtful, there are no clear guidelines. Emerging data suggest that the implementation of a protocol for early screening and management of dysphagia improves the prognosis of these patients. Further studies are needed to provide reliable evidence to make valid recommendations.
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Esclerosis Lateral Amiotrófica: Revisión de Evidencia Médica para Tratamiento.
Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative adult onset disease. Presently, ALS is considered a complexed multifactorial disorder of unknown etiology. Consequently, optimal treatment of ALS requires an integral multidisciplinary approach. Early care and palliative support in ALS lead to an improvement in survival and quality of life of patients. A multidisciplinary treatment approach is essential to provide adequate management of symptoms, respiratory problems, psychological, and social support. This review is based on literature with evidence-based practice parameters, which substantiate recommendations to guide clinical decisions in the treatment of ALS. The focus is mainly on how information is provided to the patient in regards to diagnosis and prognosis, symptomatic treatment, decisions about nutrition and gastrulation, respiratory assistance, and palliative care.
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Enfermedad de Moyamoya: Revisión de la Literatura.
Moyamoya disease is an idiopathic cerebrovascular disorder, uncommon in non-Asian population, which is characterized by bilateral stenosis of the internal carotid arteries and their major branches, as well as by the neoformation of vessels at the base of the skull (rete mirabile) and branches originated from the external carotid artery, as a compensatory mechanism due to a chronic ischemia. Its peak age is biphasic appearing around 5 and 40 years of age. In children the predominant pictures are of ischemic types while, in adults it presents itself as hemorrhagic pictures. The digital angiography is pointed out as the gold standard among all the diagnostic methods, although similar results can be obtained with an angio-MRI and angio-CT. The disease’s treatment is merely surgical characterized by a revascularization achievement, either made in a direct or indirect form. The prognosis of these types of patients is not well defined yet; but it has been observed that its worst in which the disease appeared at an early age compared to those in whom it appeared later on.
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Monitoreo Neurofisiológico Intraoperatorio: Utilidad y Ventajas en cirugía de columna.
Injuries to neural structures such as the spinal cord, peripheral nerves, nerve roots, and important vessels often occur during spinal surgery despite the experience and knowledge of the surgeon. Intraoperative neurophysiologic monitoring is an important tool to protect the integrity of those structures. This monitoring is possible due to the electric properties of the nervous system. By the continuous recording of this electrical data over key anatomical regions and periodic stimulation of neural structures to verify neurologic function, the monitoring staff can relay crucial information about the neurological status of the patient to the operating physician. The use of this information by the operating surgeon can help prevent post-operative neurological deficit and improve patient outcomes. Due to the protective and preventative nature of IONM, in some European countries and in the United States of America this test is considered the Gold Standard of the procedures that prevent and minimize neurologic complications during spinal surgery. The incorporation of the neurophysiologic monitoring duringneurological surgeries has begun in many Latin American countries and is just emerging in Ecuador. We attempt in this article to introduce and explain the importance of this support in Neurosurgery, Traumatology and other surgical specialties.
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Mecanismos Moleculares y Métodos Diagnósticos de la Atrofia Muscular Espinal Infantil
The spinal muscular atrophies are a group of disease characterized by loss or degeneration of the neurons of the horn previous of the spinal marrow. The bad operation of the same ones makes that the nervous impulse cannot be transmitted correctly so that the movements like the muscular tone are affected. The SMA is classified in five groups: SMA proximal, variants of the childhood, non proximal, paralysis bulbar and espinobulbar Kennedy type. In turn, the Infantile Spinal Muscular Atrophy (SMAi) that is a variant of the childhood, it is classified in three groups in dependence of the appearance age and clinical severity. One of the genes responsible for this disease is known as gene of the survival of the neuron (SMN), it is located in the chromosome 5 (5q 11.2 – 13.3), it presents two copies one in the region telomérica (SMN t or SMN1) of the chromosome and another in the centromérica (SMNc or SMN2) and it codes to the protein Smn. Until the moment a good therapy is not known for this illness, so that the molecular diagnosis is of great importance to improve the quality of life of the affected families.
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El Abordaje Subfrontal: Es en realidad Minimamente Invasivo?
Subfrontal key-hole approach has been considered more traumatic to the brain than the traditional pterional approach. We describe some technical tips and make recommendations to choose this approach to some specific target areas. We conclude that the subfrontal key-hole is as minimally invasive as the pterional approach if it is performed in the correct settings.
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Aspectos Históricos del Tratamiento Farmacológico de la Epilepsia
In the present work, we make an historical revision on the different treatments that had been employed for epilepsy since ancient times through the present. This article reviews different periods such as: the believe between the Incas and the Aztecas that the cause of epilepsy was the action of malignant spirits and the basements of the treatments in magical and religious methods; the starting of pharmacological treatment in the XIX century with the use of bromides, the discovery of the antiepileptic drug phenobarbital by Alfred Hauptmann in 1912, phenytoin by H. Houston Merrit and Tracy Jackson Putnam in 1937, through the introduction of new antiepileptic drugs such as lamotrigine, tiagabine, vigabatrine, levetiracetam, etc. in the nineties.
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Neuroprotección en isquemia cerebral aguda. Estado actual e importancia clínica de la cascada isquémica.
Knowledge of physiophatological mechanisms of cerebral ischemia permits understanding the mechanisms of action on which many aspects of treatment are based and the clinical and neuro-radiological changes. The only treatment with clinically demonstrated success is the use of thrombolytic to restore the cerebral circulation. A cellular and molecular mechanisms such as free radical production, lipid peroxidation, excitotoxicity and calcium ion overload constitute the important therapeutic targets of neuroprotection and it is now known that interventions such a delivering neuroprotective agents can participate to salvage a portentially reversible ischaemic region known as the ischaemic penumbra. We review the vascular and biochemistry that produce necrosis of neurons after a cerebrovascular occlusion and actual evidence of neuroprotective drugs.
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Enfermedad Vascular Cerebral en los Trópicos
Objectives. To review the clinical manifestations, diagnosis, and therapy of diseases causing cerebrovascular disease (CVD) in the tropics. Development. Most prevalent conditions causing CVD in the tropics include: sickle cell disease, Takayasu’s arteritis, cysticercosis, infective endocarditis, Chagas’ disease, viral hemorrhagic fevers, gnathostomiasis, leptospirosis, snake bites, cerebral malaria, puerperal venous thrombosis, and tuberculosis. These conditions may cause cerebral infarcts or hemorrhages, and in most instances are related to either vascular damage secondary to angiitis or hemorrhagic diathesis with bleeding in other organs. In some patients, the severity of the neurological picture makes impossible to identify an specific stroke syndrome and cerebrovascular complications are only recognized on neuroimaging studies or autopsy. Conclusions. There is a group of tropical infectious and non-infectious diseases that may cause cerebral infarcts or hemorrhages. Prompt diagnosis and therapy are needed to reduce the severity or brain damage and to avoid recurrent strokes.
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Narcolepsia, Emoción, Conciencia y su Hipotética Relación
In this work a hipotetical relationship has settled down among emotion, Narcolepsy and Conscience. For it, two of the symptoms of this syndrome have been selected, day excessive drowsiness and cataplexy. This way, and after a detailed analysis of both symptoms, we coincide in pointing toward the importance that have the emotion, the anomalies of the dream REM found in subjects with narcolepsy and the conscious in the dysfunction that occupies us. For it, and to relate the narcolepsy with the conscience and the emotion, we have worked on the theory of the Emotion of Mandler, the theory of Activity of Search on the functions of the dream REM of Rottenberg and that of William James. The final result of this work has been a speculation of the processes that happen in the subjects with narcolepsy abiding to the previously noted theories.
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Perfil de la Epilepsia en el Ecuador
The point prevalence of active epilepsy in Ecuador is between 7/1000 and 12/1000, which is similar to that reported in developed countries; however, this finding does not necessarily indicate that the risk to develop epilepsy is the same in these two populations. The incidence of epilepsy in Ecuador is between 120/100,000 and 172/100,000, which is two to threefold higher than that reported in developed countries. These results should be confirmed by further studies. In Ecuador, the frequency of epilepsy is highest during adolescence and adulthood, probably due to the high prevalence of infectious and parasitic diseases in these age groups. Regarding the prognosis, seizures recur in 43% of patients with epilepsy. The risk of recurrence by the Kaplan-Meier actuarial analysis is 30% at 12 months, 51% at 24 months, and 79% at 45 months of follow-up. Statistically significant risk factors for recurrence are the etiology (52% recurrence risk in patients with symptomatic epilepsy) and the presence of an abnormal CT scan (51% recurrence risk in patients with abnormal CT scans). Mortality in patients with epilepsy is six-fold higher than in the general population of Ecuador (standardized mortality ratio SMR of 6.3). The SMR for sudden death in patients with epilepsy (3.9) is also higher than that reported in developed countries. These greater mortality ratios probably explain the relatively low prevalence rates in our country. In 1995, Ecuador spent 4.6% of the gross national product on the health system. The Ministry of Health, which funds the public health system, contributed only 1.5% of this amount. This government contribution is one of the lowest in South America. There are no government programs in Ecuador working to manage and prevent chronic diseases, such as epilepsy. There are also no laws to protect patients with epilepsy. This complex background obligates the health authorities, scientific community, and the society in general, to join together in the fight against epilepsy, as a bio-psycho- social problem.
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Indicaciones y manejo de la toxina botulínica
Botulinus toxin (BTX) is the most potent biological toxin yet known. It is produced by Clostridium botulinium, a Gram positive bacteria. Type A Botulinus toxin is the most widely used in human drug trials. It has become the treatment of choice for blepharospasm, hemifacial spasm, cervical dystonia and laryngeal dystonia. It may also be used in the treatment of patients with oromandibular dystonia and limb dystonia, specially writer’s cramp, and has been used successfully in the treatment of spasticity and cerebral paralysis. There are many benefits from this treatment, including improved walking, improved posture of wheelchair patients, improvement of patients with spasms and easier extension of their arms and knees. The toxin also alleviates pain and may be used in therapeutic trials for prediction of the response to surgical elongation.
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Miastenia gravis: Diagnóstico y Tratamiento
Myasthenia gravis is an autoimmune disorder characterized by fluctuating muscle weakness and fatigue of different muscle groups. Myasthenia gravis may affect persons of all ages, but especially women aged 20 to 40 years. The ocular, facial and bulbar muscles are most often involved in this disease. The muscle weakness of patients with myasthenia gravis becomes worse with intercurrent episodes of infection, fever and physical or emotional exhaustion. Respiratory infection (bacterial or viral) is the most frequent trigger factor. The presence of antibodies to acetycholine receptors in a patient with the clinical features of myasthenia gravis, confirms the diagnosis. Treatment is controversial. Each patient therefore has to be treated individually, as no single treatment is suitable for all patients. Treatment may include anticholinesterase drugs, corticosteroids, plasmapheresis, immunoglobin, immunosuppressive drugs and thymectomy.
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Respiratory Dysfunction Associated with Acute Cerebrovascular Events
Cerebrovascular events (CDVE) are a major cause of morbidity and mortality worldwide. Most patients with CVE do not develop significant respiratory problems but when present, they may be a marker of severe neurologic derangement. In one study (1), only 8% of patients presenting with acute carotid territory stroke were electively intubated and mechanically ventilated due to neurologic deterioration. “Good” outcome in terms of survival and neurological status of patients with hemispheric strokes who required mechanical ventilation have been reported in approximately 20% of cases (2). Respiratory disturbances associates with strokes can result from discrete or difuse lesions to key componets of the respiratory controller. The clinical spectrum of respiratory disordes in stroke include abnormal breathing patterns, hypoxemic and hypercapnic respiratory failure, aspiration pneumonia due to an inability to protect the airways and clear the airway by coughing, and acute pulmonary emblism due to prolonged inmobilization. There is a veriety of altered respiratory patterns associated with strokes. There is a variety of altered respiratory patterns associated with strokes (3-5). These changes are not only important in determining the location of the neuroanatomic lesion, but they havealso been regarded as outcome predictors in CVE. This paper reviews the evaluation, management, and effect of respiratory care interventions, management, and effect of respiratory care interventions on a variety of respiratory system problems in patients with CVE.
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Transtorno de la Atención con Hiperactividad
Attention deficit hyperactivity disorder (ADHD), is one of the four syndromes of minimal brain dysfunction, recongnized by the last classification of the Diagnostic and Statistical Manual of Mental Disorder (DSM IV). It is mainly characterized by attention deficit, hyperactivity, a low-level frustration, impulsivity, and emotional labilit. Although there are no doubts about its organic naure, the presence of this syndrome does not mean a demonstrable cerebral or irreversible lesion, but an alteration in high cerebral function, particulary in the area of behaviour. ADHD can be associated with language, learning, and motor disabilities. Diagnosis should be made on the basis of behaviour. ADHD can be associated with language, learning, and motor disabilities. Neuroimaging and neurophysiological studies are required only to exclude another phatoogies. Stimulants are the first choice in the treatment of ADHD, mainly methylphenidate, because it is well tolerated and improves not only the attention level and hyperactivity, but also learning and writing abilites. Prognosis varies acording to the severity of the clinical picture, early diagnosis and the familial interaction on the social context of child. Approximately 5% of infantile schoool pupulation have ADHD; therefore, this diagnostic possiblity should be considered by neuropediatricians and pediatricians for early diagnosis and treatment.
