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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Estesioneuroblastoma: Presentación de un Caso y Revisión de la Literatura

A 34-year-old man presente wiith progressive anosmia, epistaxis, and intracranial hypertension related to a nasal tumor with intracranial extension. After surgical resection of the lesion, inmunohistochemical and electrical microscopy study of the biopsy material stablished the diagnosis of esthesioneuroblastoma or olfactory neuroblastoma. This is a rare embrionary tumor derived from neuroblasts of the olfactory sensorial system. That portion of the olfactory tract is located at the upper half of the nostrils, above the cribiform plate. Esthesioneuroblastoma shows a bimodal incidence with a first peak in the second decade, and a second and most important peak during the third and fifth decades of live. Individuals from both sexes are similarly affected. Pollipoid mass with epistaxis or nasal obstruction and chronic anosmia are the most common clinical manifestations of this tumor. It is invasive and frequently causes regional and distant metastasis; however, some tumors could stay without recognition until its intracranial growing through the cribiform plate causes neurologic symptoms.

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Tratamiento de la Paraparesia Espástica Tropical con Pentoxifilina: Estudio Piloto 

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Short course of albendazole therapy for neurocysticercosis: A prospective randomized trial comparing three days, eight days and the control group without albendazole

Antihelminthic therapy with albendazole for parenchymal cerebral cysticercosis, despite its widespread acceptance, is still the subject of controversy. In this prospective, randomized clinical trial, we compared the effectiveness of two regimens of albendazole therapy for neurocysticercosis against each other and against symptomatic therapy alone. A first group (27 patients) received albendazole for 3 days, a second group (27 patients) received albendazole for 8 days, and a third group (29 patients) received only symptomatic treatment. Effectiveness of albendazole was 85.8% with no difference between the 3 and 8-day groups of treatment. Improvement of the patients in the control group was 34.4%. Complete resolution of cysts was obtained in 77.7% of the patients who received albendazole. Two years after therapy, there was no difference in the number of patients free of seizures, when comparing the three groups of treatment. The ultra-short course of treatment with albendazole for 3 days was effective in our patients. Therapy with albendazole for 8 days did not provide additional benefits.

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Amnesia global transitoria y factores de riesgo vascular

Introduction. Transient global amnesia (TGA) is a clinical syndrome determined by an episode of less than 24 hours consisting of temporo-spatial disorientation with retrograde and anterograde amnesia followed by complete recovery. Objectives. To determine whether the presence or absence of vascular risk factors (VRF) in patients with TGA is associated with different clinical data and/or examination findings. Patients and methods. A retrospective study was made of two groups of 13 and 12 patients with TGA, who presented with and without VRF, respectively. The following variables were determined: VRF, age, a previous history of migraine, triggering factors, duration and repetition of the episodes, associated neurological symptoms and findings obtained by neuroimaging, eco-Doppler of the supra-aortic trunks and transcranial Doppler. The data were subjected to statistical analysis by univariate analysis with Fischer’s exact probability test. Results. The statistical studies showed no significant differences between the variables obtained in the two groups of patients. Conclusions. Transient global amnesia has been particularly related to migraine, epilepsy and cerebral vascular pathology, although its aetiology has not been fully determined. In this study we compare clinical data between the two groups of patients with and without VRF who have had TGA. The lack of significant differences between them tends to rule out a vascular aetiology as the sole cause of this  syndrome. Recently Leao’s propagated depression has been suggested as the physiopathological mechanism involved. According to this theory, the vascular pathology might act as the trigger but probably not as the aetiological factor. The findings of our study may support this thesis.

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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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Progresive Dementia and Behavioral Changes: Pick’s Disease: A rare disease or an underdiagnosed disorder?

Pick’s disease is a nosological entity with frontal dementia, early cortical dementia with severe frontal lobe disturbances, absence of apraxia, and absence of gait disturbance at onset. This disorder is underdiagnosed in clinical practice. When specific criteria for the clinic, imaging and neuropathologic diagnosis including the presence of Pick bodies, are used, the diagnosis of Pick’s disease is achieved. However, taking into account that the definitive diagnosis of PD is achieved only with pathological study, in vivo diagnosis requires of the combination of neuroimaging techniques.

More than 100 years ago, Arnold Pick described several patients who presented with progressive behavioral changes (apragmatism, outbursts of rage, and later stages, mutism) and who, at autopsy, had characteristic frontal or temporal lobar atrophy [1]. Alloys Alzheimer histologically characterized the disorder when he described “argentophylic globes” in the cytoplasm of neurons and the presence of ballooned neurons and spongy cortical wasting in the absence of neurofibrillary tangles or plaques [2]. Pick’s disease is considered a relatively rare neurodegenerative disorder, affecting subjects in their 60s with the progressive development of frontal lobe type features (e.g., difficulty planning, reasoning, abnormal social behavior), language disturbances (decreased fluency followed by echolalia, mutism), later followed by memory and gait abnormalities and occasional parkinsonism [3]. In autopsy studies of progressive dementia, only about 5% are due to PD. The underlying cause is not know, but there does appear to be a hereditary component, with clear autosomal dominant transmission in some families. The disease usually progresses inexorably over 2 to 5 years to death. At the present time there is no specific treatment available [4]. We report our findings in a patient with the purpose of to attract attention about this entity with the aim to avoid unnecessary, expensive and dangerous treatment when this kind of patients received a wrong diagnosis.

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Neuritis Optica: Análisis de 30 pacientes

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Cirugía de Aneurismas Intracraneales

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Mucopolisacaridosis en Cuba

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Características Clínicas del Temblor Esencial en México

We studied the clinical and genetic characteristics of 100 patients with definite diagnosis of essential tremor (ET), evaluated at the clinic of movement disorders of the National Institute of Neurology of Mexico. All patients hab laboratory (metabolic and endocrine tests), neurophysiologic studies, and neuroimaging studies to evaluate the presence of other potential causes of movement disorders. The study included 100 patients with definitive diagnosis of ET, 33 men and 67 women. The mean age at the time of diagnosis was 45,92 years (40.92 in men and 48.4 in women). Mean time from the onset of tremor to diagnosis was 10.60 years (13,71 in males and 9.07 in females). Family history of tremor was found in 56% of the patients. Patients with maternal inheritance had lower age of onset (35.61 years vs. 46.11 years, p<0.05). Five percent of the patients exhibited almost a total lack of capacity in writing and in fine and precise activities. This study showed that patients with ET inherited frim the mother exhibit a lower age of onset. A narrow relationship between the sex of the progenitor and ET probably exists, The torpid evolution of the tremor was noted in 5% of the cases, emphasizing the need for early therapy before deterioration becomes incapacitating.

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Manejo Electivo de los Aneurismas de Circulación Anterior Mediante Abordaje Pterional – Basal

During the last three years we employed a pteriono-basal approach to 26 aneurysms of anerior circulation. The approach consist of a pterional craniotomy removed in block with the orbitozygomatic complex, resection of the superolateral orbit and anterior clinoidectomy. Using this approach, multidireccional viewing is possible with minimal brain retraction. We recomend this approachfor most of anterior circulation aneurysm, especialy those situated in the paraclionid area.

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Cirugía en Hernias Discales Lumbares: Comparación de Técnicas

We studied 468 cases of lumbar disk hernias. They were surgically operated following standard clinical and radiological criteria. Of the 468 cases, 319 were operated using standard techniques and 149 cases were operated with microsurgery. Comparative study between these two techniques and results were evaluated. The results were excellent in 82.3% of cases when used standard techniques, and were excellent in 91.4% with microsurgery. We concluded, that microsurgery in lumbar disk hernias is a good option to decrease the risk of surgical trauma and to obtain a better postoperative prognosis.

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Medical Therapy for Cysticercosis: Indications, Risk, and Benefits

Neurocysticercosis is a pleomorphic disease that causes serveral neurological syndromes and pathological lesions. Therefore, a unique therapeutic shceme can not be useful in every patient. A proper characterizcion of the disease in terms of viability of cysts, degree of the host’s immune response to the parasites, and location of the lesions is of major importance for a rational therapy. Therapy include a combination of symptomatic drugs, cysticidal drugs, surgical resection of lesions, ana placement of ventricular shunts.

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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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Recomendaciones a los Revisores de los Manuscritos Sometidos para Publicación en las Revistas de la Federación de Revistas Neurológicas en Español.

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Neurocisticercosis en un Hospital General en San Luis Potosí, México

Cysticercosis is the most common parasitic disease of the CNS and the main cause of secondary epilepsy in developing countries. We present the number of patients with neurocysticercosis evaluated at a general hospital in San Luis Potosí, Mexico, on the basis of the files of the department of neuroimaging. The prevalence of neurocysticercosis that we found in this sample (28% of patients with secondary epilepsy) confirm that this parasitic disease is a common cause of secondary epilepsy and a major public health problem in our city.

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Pseudotumor cerebral: el lado maligno del espectro clínico

Introduction. The pseudotumor cerebri syndrome (PTC) is a clinical entity of unknown etiology affecting mainly young, obese women. The major morbidity factor in this condition is the possibility of vision loss, initially considered to be low, but now known to affect a significant proportion of patients. Clinical case. We describe a case of PTC in a young, obese black women who presented with headache, transient visual blurring and diplopia. Besides obesity, there where no other relevant clinical findings, namely other pathologies and history of medication use. An extensive analytical workup was negative and the CSF showed no abnormalities, aside from increased pressure. Brain MRI was also normal. The clinical course was progressively worse, consisting of rapid vision loss associated with an intracranial pressure of over 850 mmH2 O, despite treatment with diuretics (acetazolamide, furosemide and spironolactone) in therapeutic dosage. Eventually, the patient needed the rapid implantation of a lumboperitoneal shunt to prevent blindness, and went on to make a nearly full recovery. Conclusions. In all major series of PTC, loss of vision has been reported to occur in a significant percentage of patients, and cases have been described in which a catastrophic course with rapid progression to near blindness has led to the introduction of the designation of ‘malignant’ pseudotumor cerebri. We believe that such cases, although rare, need to be recognized as the malignant end of the spectrum of PTC, and that their management constitutes a neurological emergency.

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Diagnóstico de la Enfermedad Cerebrovascular Aguda.

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Anti-Human Herpes Virus 6 Type B Antibodies Make Up the Oligoclonal Bands in Multiple Sclerosis

Background: There is considerable Oepidemiological evidence that a latent or slow infection with human herpes virus 6 is associated with the etiology of multiple sclerosis. However, techniques for detecting anti-human herpes virus 6 antibodies in the cerebrospinal fluid had been lacking up to the time of this study. Preliminary results were presented at the Second International Conference on Human Herpesviruses 6, 7 and 8 (Italy, Pisa, May 8-11, 1997). Methods: We therefore adapted the enzyme-linked immunosorbent assay in order to make this determination.

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Microcraniectomía Asterional: Una Opción Quirúrgica Para la Patología del Angulo Ponto-Cerebeloso.

Current tendency is towards minimally invasive surgical approaches that offer short-term recovery and short hospital stays, reducing the costs of treatment. In cranial neurosurgery, minimally invasive surgery is based on the Key-hole concept, that is, small surgical incision that allow an approach to the lesion using natural microsurgical corridors at the subarachnoid space. The technique that we present in this paper may be carried out with basic surgical equipment and instruments, and do not depend on sophisticated technology. In this article, we present our experience in 250 patients with the technique of key-hole surgery of the cerebelopontine angle. We had excellent results that were similar to those reported in the literature, since we had a low postoperative morbidity, fast recovery, fast recovery, reduced offers the advantage of reduced costs for both patients and institutions.

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Gliosarcomas Cerebrales: Aspectos Clínico-Quirúrgicos, Correlación con Estudios de Neuroimagen, Hallazgos inmunohistoquímicos y Pronóstico.

Gliosarcomas are maling, rare, and dimorphic neoplasms formed by glioblastoma associated with sarcomatous components that may develop from the malignant transformation of hyperplastic vascular elements. We report three patients with gliosarcoma to analyze the correlation between neuroimaging and surgical findings, and prognosis. Clinical manifestations had a sudden onset, in previously healthy patients, and was characterized by a syndrome of intracranial hypertension of acute onset related to the development of an intratumoral hemorrhage. In two of our patients the tumors were observed as intra-axial lesions having large areas of necrosis and peripheral enhancement of contrast material. This finding is similar to that observed in patients with glioblastomas. The other patient presented with a well-defined and homogeneous hyperdense lesion the resembled a meningioma. In our series the patients with the longest survival was the one who had a lesion resembling a meningioma, in whom the sarcomatous component of the lesion predominated.

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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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Problemas en el Diagnóstico de la Demencia Vascular

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Sleep Patterns and their Relation to Psychological Traits in Women

The number of hours of sleep, approximately 8, is an average that varies little from one culture to another. However, there are individual differences for this variable, which has permitted the establishment of certain sleep patterns. Many studies have focused on the relationship between psychological variables and sleep patterns, which have produced contradictory results. The purpose of the present investigation is to evaluate the extent of dimensions of extraverted and neurotic personalities and state-trait anxiety levels in women with different sleep patterns. In a sample of 300 young healthy adults, 34 women were selected, 17 women showed a long sleep pattern, 9 showed an intermediate sleep pattern and 8 showed a short sleep pattern (age X = 20.29 SD = 1.55). The results indicate that there are no significant statistical differences between psychological variables and sleep patterns, which concludes that there exists no relation between sleep duration and personality and anxiety factors.

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Hematomas Epidurales Temporobasales

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Epilepsias Mioclónicas

Objective: To analyze the frequency of myoclonic epilepsies (ME) according to the classification of the International League Epilepsy (ILAF) and workshop of the Comission on Pediatric Epilepsy (Royaumont-France 1997), considering the possibility to include or modifi myoclonic epileptic syndromes. Patients and Methods:Clinic histories of 113 patients (56 men and 57 women) with diagnostic of ME evaluated between January 1993 and July 1998 were reviewed. Early epileptic encephalopathy, progressive and photosensitive epilepsies, as well as other epilepsies presenting with myoclonic seizures during their evolutive course, were excluded. Results: We recognized the following syndromes: a) Idiophatic: 1) Bening ME of Infancy, 10 cases (8.8%), 2) Reflex ME of Infancy, 2 cases (1.8%), 3) Eyelid myoclinia with absence (EMA), 3 cases (2.6%), and 4) Juvenile ME, 29 cases (25.6%); b) Cryptogenic: 1) Myoclonic-Astatic Epilepsy (MAE) of favorable course, 21 cases (18.5%) and unfavorable course, 10 cases (8.8%), 2) Severe ME of infancy, 25 cases (22.1%), and 3) Myoclonic absence epilepsy, 2 cases (1.8%); and c) Symptomatic: 1) MAE, 2 cases (1.8%), 2) Severe ME of Infancy, 2 cases (1.8%), 3) Myoclonic Status in non-progressive encephalopathies (MSnPE), 4 cases (3.5%), and 4) others, 3 cases (2.6%). Conclusion: Cryptogenic (51.3%) and idiopathic (38.9%) seizures were the most common types of ME in our study. In the idiopathic group, the most frequent syndrome was juvenile ME, while in the cryptogenic group, was the Myoclonic-Astatic epilepsy. We consider that EMA should be included in the new classification of epilepsies as an idiopathic syndrome. We also suggest that Reflex ME of Infancy should be discussed as a new syndrome of ME or as a variant of benign ME of Infancy. Finally, whether MsnPE is a new syndrome or a peculiar evolution of symptomatic epilepsies needs further discussion.

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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.

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