Publicación Oficial de la Sociedad Ecuatoriana de Neurología, de la Liga Ecuatoriana Contra la Epilepsia y de la Sociedad Iberoamericana de Enfermedad Cerebrovascular

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The Impact of Rheumatoid Arthritis in Neuropsychology, Depression and Anxiety. A Case-Control Study, with 90 Portuguese Female Subjects.

Objectives: The study of the impact of Rheumatoid Arthritis in cognitive functions has not been well documented in Portugal. So, with this original article we tried to clarify this reality in this particular country.

Methods: We assessed the results of 45 Rheumatoid Arthritis female patients (intervention group), comparing each patient in a case control paired strategy (years in school and age), with control subjects (n = 45, in a total of 90 subjects). All subjects were evaluated with Paced Auditory Selective Attention Test, Word List Generation Test, Luria Nebraska Neuropsychological Battery, a Portuguese depression screening test (IACLIDE), STAI (anxiety trace and trait test) and the Mini Mental state examination.

Results: The results show, for the first time in Portuguese patients, the presence of major deficits in terms of cognitive function and symptoms of depression and anxiety.

Conclusion: This article strengthens the argument of the necessity to pay attention in psycho-educational, psychotherapeutic and cognitive stimulation as well as neuropsychological intervention in these types of patients.

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Esclerosis Lateral Amiotrófica: Criterios de El Escorial y la Electromiografía en su Temprano Diagnóstico.

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.

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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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Demencia y Parkinsonismo como Síntomas Iniciales en un Paciente con SIDA.

Dementia and Parkinsonism as early symptoms of AIDS are infrequent. We report the case of a male patient 62 years old, with a history of diabetes – insulin dependent and illicit drug use, who began his symptomatology with dementia and parkinsonism two months before his death.

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Enfermedad de Osler-Weber-Rendu y Neuroinfección: A Propósito de un Caso.

A 68 years old man with a previous diagnostic of Hemorragic Hereditary Telangiectasia (HHT) or Osler-Weber-Rendu Syndrome has been transferred to the emergency room of Metropolitano´s Hospital of Quito because of altered mental status, fever and nuchal rigidity. Complementary studies reported images of ventriculitis and left temporal abscess.

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Epilepsia Parcial Benigna Atípica de la Infancia: Presentación de un Caso Peculiar y Revisión de la Bibliografía.

Introduction: Atypical benign partial epilepsy of childhood is a rare form of epilepsy characterized by a combination of partial seizures, as observed in Partial Benign Epilepsy of Childhood, as well as with centre-temporal paroxysms and generalized seizures; showing furthermore, a continuos peak wake electroencephalographic pattern characteristic of NREM sleep.

Clinical Case: Patient presenting with partial seizures is admitted to the hospital with EEG findings compatible with continuos peak wave electric status of NREM sleep.

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Enfermedad de Creutzfeldt-Jakob. Presentación de un Caso Clínico y Revisión de la Literatura.

Creutzfeldt-Jakob disease is the prototypic rapidly progressive dementia. Esporadic CJD is the most common presentation. We report a 65 year-old patient with primary visual complaints, afterward psychosis with memory and higher cerebral functions complaints. After 4 months of evolution, the patient showed akinetic mutism and myoclonus. In the MRI, anormal hyperintensity was seen on T2 sequences in the subcortical and cortical regions of the occipital lobes. EEG revealed periodic generalized triphasic waves. We present this patient with the Heindenhain variant of sporadic CJD, in order to consider it when were are faced with a patient with rapidly progressive dementia (RPD). We emphasize the importance of early diagnosis for prognostic value and for minimize the risk of accidental iatrogenic transmission.

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Investigación y Universidad.

Not available.

 

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The “8-foot Up and Go” Test as a Physical Performance Measurement in Parkinson’s Disease: A Pilot Study.

Blackground: Parkinson disease (PD), is a movement disorder, so physical assessment should be a key component in the diagnosis of individuals suffering from this disease. There is a neeed to assess the utility of some motor tests in PD, specially those which include assessment of straight line walking and turning.

Objective: This pilot study aimed to asses the utility of the “8-foot up and go” test as a physical performance measurement in PD, as well as the relationship between functional mobility and quality of life in this population.

Material and Methods: Twenty-four patients with a diagnosis of idiopathic Parkinson’s disease volunteered and finished the study. They performed the “8 foot up-and-go” test and answered the 9-item Parkinson’s Disease Questionnaire.

Results: The motor test’s total score correlated significantly with different sub-scales of the 9-item Parkinson’s Disease Questionnaire, especially with the mobility sub-scale and with the total score.

Conclusions: The “8 foot up-and-go” test is useful and easy to perform by Parkinson’s disease patients and it has turned out to be a clear indicator of the way the illness affects their quality of life.

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Efectos de la Subtalamotomía en la Inducción o Modificación de Disquinesias en Pacientes con Enfermedad de Parkinson Idiopática.

We performed a longitudinal, analytic and retrospective study in order to evaluate the effect of subthalamotomy in the induction or modification of dyskinesias in patients with Parkinson’s disease. Was study the motor behaviour, the cardinal features of Parkinson’s disease, the mean daily Levodopa dose and the dyskinesias induced by Levodopa or subthalamotomy in 50 patients with Parkinson’s disease that were operated between 1995 and 2005 in our center. Evaluations were performed in the ‘off’ and ‘on’ drug states before surgery and at least for one year. Patients were assessed according to the CAPIT (Core Assessment Program for Intracerebral Transplantation) protocol. In our study the cardinal features of the disease improved in more than 50 %, it were evaluated through the Unified Parkinson’s Disease Rating Scale (UPDRS), as well as the mean daily Levodopa dose was reduced by 40% compared with baseline. The dyskinesias induced by Levodopa were reducing too, and we think that it’s not explainable only for the reduction of the Levodopa dose. Subthalamotomy induced contralateral dyskinesias in approximately 50% of our patients but were generally mild and short lasting. The risk of persistent and severe dyskinesias postoperative was smallest. This study indicates that the presence of severe preoperative dyskinesias and the dorsal location of the lesion are relevant for the development of chorea after subthalamotomy in patients with Parkinson’s disease. These find must to be confirm in a prospective randomized study.

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Morphological Changes Induced by Three Aminoglycosides on the Cochlear Stria Vascularis.

Aminoglycosides are efficient antimicrobials commonly used in clinical practice. Their effects on the cells of the organ of Corti and the spiral ganglion neurons have been extensively studied. However, there are only a few reports concerning aminoglycoside-induced morphological changes on the stria vascularis. The purpose of this study was to describe morphological and morphometrical changes on the rat stria vascularis after a profound sensorineural hearing loss induced by three aminoglycosides (kanamycin, gentamicin and amikacin) followed by furosemide. To analyze the stria vascularis area and the number of blood capillaries per stria vascularis, cochleae from fourteen rats sampled at eight weeks after deafness and from four control animals were processed. Serial semi thin cochlear sections from the apical, upper middle, lower middle and basal turns were examined under a light microscope. The cochlear damage degree depended on the aminoglycoside. Mean stria vascularis areas for both kanamycin and gentamicin groups were lower than controls. The mean number of blood capillaries per stria vascularis was reduced for the three aminoglycoside-deafened groups as compared to control animals. For both variables, the most severe damage was observed for gentamicin-deafened animals, followed by kanamycin and amikacin.

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Efectos del Tratamiento Rehabilitador y el “Treadmill” en Pacientes con Ataxia.

Ataxia is a syndrome related to gait disorders that makes necessary a wide range of possibilities in rehabilitation in order to improve the disorder. The treadmill can be useful because it combines motor learning and sensory tricks. This study included 15 patients with ataxia from CIREN clinic. Treatment including treadmill was applied in 30 sessions, one hour each one. Motor function was evaluated pre and post treatment using ICARS scale, velocity, number and width of paces and Hausser index. Comparing results before and after intervention showed statistically significative improvement in ICARS scale and LEIS (p<0.001 and p<0.041 respectively). Rehabilitation treatment including treadmill has favorable effects over gait disorders in patients with ataxia.

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Neurofobia entre los estudiantes de la Carrera de Medicina de sexto a décimo semestre en la Universidad Católica Santiago de Guayaquil.

Background: Neurophobia is defined as fear to neuroscience and clinical neurology. By the impact of neurological diseases worldwide, management of these disorders could be affected. No data exist of this phenomenon in Ecuador. Objectives: To evaluate the difficulty, knowledge, confidence and interest in neurology, neuroscience specialties, affinity factors for difficulty in neurology and neurological training among medical students.

Methods and Materials: A descriptive study was conducted at the Catholic University of Santiago de Guayaquil, surveying: difficulty, knowledge, confidence and interest among specialties; affinity to neuroscience as a specialty, factors contributing neurology´s perception as difficult, and neurological undergraduate training.

Results: The response rate was 78.32% (401/512). Neurology was the most difficult (3.01±0.048), the second with less clinical confidence (2.01±0.056), and third with less knowledge (1.96±0.058). The differences were significant (p <0.001). 70.82% did not plan to pursue a specialty related to neuroscience, 59.85% for absent personal affinity; 10.97% for poor teaching; Contrary, 29.18% want a related specialty in neuroscience, 19.95% by personal affinity and 9.23% for good teaching. Need to know basic neuroscience was the most important for the perception of difficulty (2.76±0.055) followed by poor teaching factor in neurological involvement (2.68±0.57). Training in Neurological Semiology the lowest (1.70±0.061) followed by Neuroanatomy (1.98±0.58) and Neurology (2.52±0.81). Neurologic semiology had the highest percentage of inadequate training (20.45%) compared to Neuroanatomy (9.47%) and Neurology (6.16%).

Conclusions: There is neurophobia among our students. Research and modifications in teaching must be developed to prevent neurophobia and contribute to “neurophilia.”

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Desarrollo Psicomotor y Variables Medioambientales en Dos Poblaciones del Ecuador.

Objective: To determine the influence of some environmental variables associated with psychomotor development in children from an indigenous community and mestizo community in Ecuador. Material and methods: For the observation and measurement of variables were used EEDP scale and a registration form of environmental factors. Data was analyzed using logistic regression test. The methods of observation and measurement of variables through the EEDP scale and a registry of environmental factors were used. Data was analyzed through a logistic binary regression. X2 omnibus test was applied; the same statistician was estimated by the Hosmer-Lemeshow test with global adjustment. Also, it was estimated -2 log likelihood (-2LL) in order to check the veracity of the results, the Cox and Snell R2, Nagelkerke R2 and Wald statistic. Results: The findings indicate that some of the independent variables were related to motor development and coordination in the populations studied. Furthermore, it was found that breastfeeding and type of housing have influence in the social and language development of both populations.

Conclusions: Only some of the studied variables exert influence on psychomotor development of two populations: breastfeeding, age and housing type.

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Cambios Fisiológicos en el Sueño.

Not available.

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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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Sobre Neurociencia y Proceso Judicial.

Neuroscience is a relatively new field of study that is introducing challenges in different fields of knowledge and has begun to generate interest in legal scholars and philosophers. The dialogue and debate between Law and neuroscience has become urgent. Neurociencia y proceso judicial [traducción libre: Neuroscience and judicial process], a work published in 2013, gives a good account of that. Its eight essays written by legal scholars and legal philosophers explores a wide spectrum of issues and suggestive connections between procedural law, philosophy of evidence, legal philosophy, scientific and technological advances on the study of the brain and its functioning, and some of the postulates from certain neuroscientific currents. This article contains a review of that work.

 

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Estimulación Cerebral Profunda y Enfermedad de Parkinson: Documento Instructivo para el Manejo Multidisciplinario en el Hospital Regional Dr. Teodoro Maldonado Carbo

Neuromodulation by deep brain stimulation (DBS) in the treatment of Parkinson’s disease has developed new research in its pathophysiology. By electrical stimulation of established surgical targets, clinical response can be modified and optimized, improving quality of life. Even though its efficacy, DBS is not indicated for all patients. Selection criteria must be considered and a multidisciplinary team -with roles and responsibilities clearly defined- must evaluate the patient.

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Avances en el Manejo de la Patologia Neuroquirúrgica en Ecuador.

Not available.

 

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Partial Thenar Atrophy as a Physical Manifestation of Martin Gruber Anastomosis.

Martin Gruber anastomosis is a frequent finding on electrodiagnostic examination and has three common variants. Much has been written about these variants such as the anatomic course of crossover fibers and the electrodiagnostic findings. However, little has been written on associated physical findings that might suggest such a diagnosis. In this report the physical examination findings clearly supported a diagnosis of a Type III Martin Gruber anastomosis that was initially established through electrodiagnostic testing. Awareness of this pattern on physical examination could provide an early clue to the possible presence of anomalous innervation.

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Meningitis Crónica por Angiostrongylus Cantonensis.

Two patients with chronic meningitis due to the helmint Angiostrongylus cantonensis are reported. Two years after the primary infection from a previous outbreak, both patients had pain in lumbosacral region, leg weakness, headache and sleep disturbances among other symptoms. Intrathecal synthesis of IgA, IgM and IgG was found. Nuclear magnetic resonance showed temporal atrophy in one patient and in the other one a T2 hyperdense lesion zone in right parietal region at white zone level. It is the first report of chronic disease because of this parasite in the Americas.

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Encefalomielitis Aguda Diseminada: Reporte de un Caso con Afectación Selectiva de Tallo Cerebral.

Introduction: Acute Disseminated Encephalomyelitis (ADEM) is an autoimmune demyelinating disease of the central nervous system´s white matter; it usually has a monophasic course with an idiopathic cause or following infection or immunization processes.

Objective: This article has the purpose to present a clinical case with selective involvement of brainstem. We describe a 28 year old woman who developed ADEM. Her clinical presentation, neuroradiological findings and treatment are reported.

Conclusions: ADEM is an infrequent disease which can be fatal. MRI findings confirm the diagnosis and steroid therapy appears to be the most effective treatment, although the disease may spontaneously improve.

 

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Apoplexy of Rathke Cleft Cyst: A Case Report and Literature Review.

Symptomatic Rathke cleft cysts (RCCs) are rare sellar and suprasellar lesions, and apoplexy is one of the most unusual presentations. Only a few cases of hemorrhagic apoplexy of RCCs have been reported and their pathogenesis is still poorly understood. In order to present a diagnostic thread to reduce misdiagnosis rate preoperatively, we reported one case of RCC apoplexy and reviewed the associated published literature. we also summarized the clinicopathological relationship on clinical symptoms, imaging features and intraoperative visualization of intracystic content.

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Hemangioblastoma Sólido de Cerebelo. Presentación de un Caso y Revisión de la Literatura.

Hemangioblastoma is an uncommon benign and highly vascular tumor that is usually observed in the brain, spinal cord and retina. It may be solid or cystic and approximately 25 to 40% of them are associated with von Hippel-Lindau syndrome. Clinical manifestations of hemangioblastoma are nonspecific and they depend on the location and growth pattern of the tumor. Despite its great vascularity, they rarely manifest themselves with hemorrhage and when they do, intraparenchymal bleeding, intramedullary hemorrhage or subarachnoid hemorrhage may be observed. The definitive diagnosis of HB is the pathologic exam. The treatment of choice for hemangioblastoma is microneurosurgery in order to make a complete resection. Preoperative endovascular embolization of feeding vessels may be useful in selected cases as the main risk of this surgery is intraoperative bleeding. We present a case of a 25 years old female diagnosed with right cerebellar hemisphere hemangioblastoma and hydrocephalus, therefore treated through various surgical stages: placing of a medium pressure valve, suboccipital craniectomy, supraselective embolization of feeder vessels and finally extirpation of the tumor mass.

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