The Benign Epilepsy of Childhood with centrotemporal Spikes (BECCTS) constitutes the most common form of idiopathic epilepsy in the paediatric population.
We present 80 cases with this diagnosis analyzing its clinical and electrophysiological characteristics, and particularly its atypical evolution. Results: 71% were male (relation 2.5:1), the partial motor seizures were the most frequent (40%) followed by the partial generalized secondarily (35%). 75% of the seizures appeared during sleep. The 66% of our patients were not medicated and a patient showed an electro-clinical picture of BECCTS and of Occipital Benign Epilepsy, Panayiotopoulos type, in concomitant form. 5% evolved in atypical form: a patient as atypical BECCTS with compromised language and hyperactivity, another developed a continuous point-wave syndrome of slow sleep and a patient debuted as focal epileptic state in hemi face with anarthria. Conclusions: The present study remarks its differential diagnosis and alerts about the phenomena of secondary bisynchronism that can appear in this type of epilepsy, the role that antiepileptic drugs play in its probable appearance and the proper and adequate changes to avoid future cognitive compromise. However, in 95% of our cases, the evolution of the BECCTS was favourable.
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Estudio de ochenta pacientes con Epilepsia Benigna de la Niñez con Espigas Centrotemporales.
Valor de la pulsioximetría nocturna en pacientes tetrapléjicos.
Sleep breathing disorders are common in quadriplegic patients and can be accompanied by significative oxygen desaturations. The purpose of this investigation was to evaluate the behavior of the oxygen saturation during sleep in 20 patients diagnosed as traumatic cervical tetraplegia admitted at the Julio Diaz National Rehabilitation Center, considering the intensity of the injury according to ASIA scale as well as the behavior of demographic, anthropometric, spiro metrical, oxymetrical variables and the muscle strength in both complete and incomplete injuries.
Ventilatory function and nocturnal oxygen saturation were studied, with findings of higher percent of incomplete injuries (70%) toward the complete ones (30%). There were not significant differences between both groups age, body mass index, neck circumference, neck length, time of injury, espiratory volume in the first second, forced vital capacity, espiratory volume in the first second/forced vital capacity index, vital capacity, maximal inspiratory pressure, and maximal espiratory pressure.
All patients showed a restrictive ventilatory disorder. Though significant oxyhemoglobin desaturations were not found in any case; complete injuries displayed a higher rate of desaturations after an hour sleep than the incomplete ones and they showed a significant negative correlation between the desaturation episode per hour of sleep index, and mean oxygen saturation.
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Malformaciones vasculares de la médula espinal.
Congenital vascular malformations are largely known as rare entities. The prevalence of vascular malformations of the spinal cord is around 16%. Spinal arteriovenous malformations (AVM) are a heterogeneous group of lesions that may impair spinal cord function, and can be considered as a treatable cause of myelopathy.
According to localization they are classified as: dural, intradural, extradural, and complex, each one with a different arterial nutrition. According to the pathological anatomy it classifies in: cavernous angiomas, capillary hemangiomas and AV lesions.
The clinical manifestations of the spinal AVM are acute neurological deficits caused mainly by bleeding, or a subacute myelopathy, presenting as an insidious onset paraparesis associated with sensorial, intestinal, sexual and urinary dysfunction, based on the level (cervical to lumbar) of the lesion. The most common spinal vascular lesion in adults and elderly are dural arteriovenous fistulas prevalent in men and representing 56% of spinal vascular malformations.
After clinical suspicion, diagnosis is made by magnetic resonance imaging (IRM) which leads to selective medullary angiographies in order to determine type and subtype of medullary vascular malformation and, therefore, establish the most appropriate treatment.
Early rehabilitation permits acceptable functional recovery.
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Mioclonías benignas de la infancia temprana: Revisión de la literatura a propósito de dos casos clínicos.
Benign myoclonus of early infancy (BMEI) is a non epileptic paroxystic disorder unknown in our pediatric population. We analyze the clinical characteristics in two patients with BMEI, and emphasize their differential diagnosis in four groups: encephalopathies with a non epileptic myoclonus, myoclonus without encephalopathy and without epilepsy, epileptic encephalopathies with myoclonus seizures and myoclonic epilepsy without encephalopathy. We consider to the BMEI as an autolimited, age-dependent, non epileptic paroxystic disorder without encephalopathy with onset in the early infancy. This study could be useful in order to identify the BMEI and avoid the incorrect use of antiepileptic drugs in our patients.
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Epilepsia Benigna Occipital tipo Panayiotopoulos y Epilepsia Benigna con espigas centrotemporales concomitantemente en el mismo paciente.
The Panayiotopoulos type benign childhood occipital epilepsy, (PBCOE) and the benign childhood occipital epilepsy with centro-temporal spikes (BCECT) are two forms of focal idiopathic epilepsy recognized by the International League Against Epilepsy. Several authors have reported that some patients with PBCOE evolve later in life toward a BCECT, remarking the fact of the morphological similarities of their paroxysms and the spikes migration phenomena. However, few cases of patients with PBCOE have been described concomitantly clinical characteristics of a BCECT in the same evolving process. One clinical case with the mentioned concomitant manifestation is shown, analyzing its clinical characteristics, differential diagnosis and treatment. Finally it is postulated that the PBCOE could be an early form of the BCECT
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Resolución clínica de un hematoma subdural crónico en un niño con quiste aracnoideo conocido.
Chronic subdural haematoma is a well-known and relatively frequent entity in the elderly. It is originated by a slight trauma which provokes subdural veins to rupture after suffering stretching due to cortical atrophy.
We report the case of a 11 year old patient with a previous known arachnoid cyst who was diagnosed as chronic subdural haematoma without association to cranial trauma and with slight neurological impairment. He responded successfully to pharmacological therapy. The following clinical case permits review pathophysiology of chronic subdural haematoma and considers subarachnoid cyst as a risk factor.
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Esclerosis Lateral Amiotrófica Secundaria a Infección por VIH.
Motor neuron diseases are not frequently associated to Human Immunodeficiency Virus (HIV) infection, although some reports have mentioned this relationship, suspecting a viral involvement in the pathogenesis of this disease. A 43-years-old male with HIV diagnosis is described, who started with progressive weakness of his legs, showing further worsening 6 months later, with upper extremity and bulbar involvement. Neurophysiological studies demonstrated upper and lower motor neuron compromise in all the extremities and bulbar muscles. Other causes were ruled out. Auto-antibodies against Human Herpes Virus type 8 were positive for IgG. There were no anomalies on image studies. A change in the antiviral scheme stopped temporarily the progression of the clinical features. However, posterior withdrawal of medications due to toxic hepatitis led to worsening of signs and symptoms. This case, among the previously reported worldwide, suggests that the association between motor neuron diseases and HIV is not coincidental, this must raise suspicion in every subject with risk factors for HIV and coexistent motor neuron disease.
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Algunos aspectos sobre la Hemorragia Intracerebral Espontánea en el Puerperio.
Primary hemorrhagic cerebrovascular accident in brain parenchyma is known as spontaneous intracerebral hemorrhage. It uncommonly occurs during pregnancy and puerperium. We briefly describe this clinical entity, although it is rare, is important to know its existence in order to give a good treatment.
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Cavernomas: a propósito de dos casos.
Cavernomas are uncommon vascular lesions, with presenting symptoms after third decade of life. They are asymptomatic in 20% of cases, rising to 40% in familiar cases. Clinical picture is variable but seizure is the most common presenting feature. We report, briefly, two clinical cases and comment some data from the literature.
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Programa de Riesgo Vasculocerebral Multifactorial orientado a la Comunidad. Policlínica Buenaventura 2005.
Community-oriented multifactorial cerebrovascular risk program is a community health project for cerebrovascular risk factors control. Primary objective was to reduce morbimortality of this group of patients as risk was stratified.
Material and method: This intra-group controlled study included 101 patients, any gender, older than 45 years with a a past history of hypertension. A multidisciplinary group applied a Cerebrovascular Risk Evaluation Scale, which divided patients in low, moderate and high risk. Results were compared before and after intervention over modifiable risk factors. Statistical significance was considered at 95%.
Results: Modifiable risk factors control was beneficial in case of arterial hypertension. Lack of knowledge in the population stroke management was high. There was not good adherence to different treatments including diet, drug therapy and routine exercises. All these indicators were considered positive and were validated for the scale.
Conclusions: Clinical and administrative indicators in the evaluation of program effectiveness permitted to validate the applied instrument as an alternative in primary care assistance.
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Prevalencia y Tendencia de Trastornos Mentales en el Instituto Nacional de Neurología y Neurocirugía.
A mental disorder is the result of an imbalance between biological and social aspects that reflect an alteration of cerebral functions which intervene in the life and productiveness of each individual. In this study the prevalence and trend of mental disorders were estimated in the National Institute of Neurology and Neurosurgery in Mexico City. This was a transversal study. The information was obtained during 1995 to 2004 of clinical records of patients admitted by first time in the Institute with diagnosis of mental disorder according to ICD-10, and different epidemiological variables were identified.
One thousand four hundred eighty three cases were identified. 56% were woman. The more frequent group of age was 20-29 years. In first place was Schizophrenia (22%), followed by Depressive Episode (16.5%) and in third place Bipolar Affective Disorder (8.6%). The prevalence was higher in 1995. In general, a tendency to decrease of mental disorders was observed, although it was not statistically significant.
Previous reports affirm that the number or patients with neuropsychiatric disorders will increase around the world in next years. Because most patients are assisted in the beginning by general or alternative medicine, it is essential for cases to be promptly identified and be addressed to specialized institutions for their attention.
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Predictores del nivel de somnolencia en pacientes con Apnea Obstructiva del Sueño.
Excessive daytime sleepiness (EDS) is the obstructive sleep apnea syndrome (OSAS) most frequent symptom. The relationship between the level of daytime sleepiness and standard polysomnographic variables (i.e., apnea/hypopnea index [AHI] and oxygen saturation SaO2) has been the subject of a number of studies. To date, the results have been inconsistent. The goal of this study is to assess the variables significantly related with daytime sleepiness severity. The variables chosen were: AHI, percentage of total time that the subject remains with arterial oxygen saturation level below 90% (SaO2<90%), minimum oxygen saturation (SaO2 m), body mass index (BMI), and age. The study sample was composed of 51 obstructive sleep apnea patients selected from a medical centre.
Subjects completed using the Epworth Sleepiness Scale (ESS) to determine daytime sleepiness. The OSAS was diagnosed by conventional polysomnography.
The results indicated significant correlations between level of daytime sleepiness and age (r=.302; p<.05), BMI (r=.339, p<.05), SaO2 m (r=-.393, p<.01) and SaO2 < 90% (r=.492, p<.01). Significant correlations were also found between AHI and SaO2<90% (r=.314, p<.05), BMI and SaO2<90% (r=.387; p<.05), and SaO2 m and SaO2<90% (r=- .746; p<. 01). No significant correlation was detected between AHI and level of daytime sleepiness. According to the results, the percentage of total time that the subject remains with arterial oxygen saturation level below 90% (SaO2< 90%) is the best predictor of daytime sleepiness severity.
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Síndrome de apnea obstructiva del sueño y accidentes de tráfico.
In recent years many studies have shown that Obstructive Sleep Apnea Syndrome is one of the biggest risk factors for drivers. The most severe patients report that they become sleepiness not only while driving on the highway or over long distances – during which the driver may stop the vehicle to sleep for a short time – but also during short distances around town. The interest raised by this problem has caused several studies to centre their objective on identifying the factors that are responsible for this increase in traffic accidents. Polysomnographic testing has verified that the sleep of these patients is fragmented which causes an excessive daily sleepiness and a sensation of tiredness and fatigue, which, up to a point can cause traffic accidents. Current work is revising some the studies which have been undertaken in order to evaluate the prevalence of traffic accidents in patients with Obstructive Sleep Apnea Syndrome according to the statistics. At present the nasal Continuous Positive Airway Pressure is the treatment of choice of this syndrome. For that reason and in relation to the problem that we are studying, the traffic accidents in patients with Obstructive Sleep Apnea Syndrome, we have recently revised some of the studies that have been undertaken to evaluate up to what point the therapy of Continuous Positive Airway Pressure diminishes the frequency of traffic accidents in patients with Obstructive Sleep Apnea Syndrome.
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La evaluación neuropsicológica en la Cirugía de Epilepsia.
Since the decade of the sixties objective systems of evaluation of the superior functions have been developed with the purpose of being able to establish the cognitive state from the patients candidates to epilepsy surgery, for that, the neuropsychological evaluation is made by means of the use of suitable instruments that allows to identify the cerebral dysfunction and taking into account the following aspects: a) to establish the global cognitive state, b) to guide in the lateralization of the cerebral dysfunction, c) to predict the risk of deterioration or cognitive improvement, with base in the preserved functions and in the altered functions, and d) after the surgery, to describe the patient’s cognitive state by means of periodic evaluations, with the purpose of having an evolutionary control of the neuropsychological functioning, providing in a precise and integrated way the effects that the surgical intervention produces in the cognitive functioning of the patients. The neuropsychological evaluation is an integrated process that requires of several hours to be completed, for that, it is important that this is carried out without the previous knowledge of the discoveries of image and/or electroencephalographic methods, since this could slant the exploration and the results, when it is not possible the identification of other possible cognitive alterations, or, one runs the risk of supposing the existence of cerebral pathology where there is not. In this article the theoretical-methodological aspects of the neuropsychological exploration are described in the area of epilepsy surgery.
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Bases anatómicas y fisiológicas del sueño.
Sleep study has rarely been important until the past few years, although its pathology deserves special attention from the medical point of view. The present work intends, with update knowledge, to review in a summarized and practical way, the anatomical basis and mechanisms involved in sleep, its types, and the regulation sleep-wakefulness cycle. This will be helpful for a better understanding of its function and will permit an adequate management of its pathology.
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Epilepsia Occipital Benigna de la Niñez tipo Panayiotopoulos Presentación de cinco casos clínicos y revisión de la literatura.
Occipital Benign Epilepsy of Childhood, Panayiotopoulus Type (OBECP) is the more frecuent idiopathic epilepsy following the Benign Epilepsy Childhood with Centrotemporal Spikes. We present five cases with this diagnosis analyzing its clinical, electroencephalografics, therapeuthics and evolutives characteristics. This study remarks our low incidence, perhaps due to of poor knowledge and the most cases are underdiagnosted in the paediatric population. Our objetive is alert about this type of Epilepsy, its early diagnosis, adecuated treatment and the respective family assessment because this pathology had a favourable evolution.
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Síndrome de Guillain Barré imitando Muerte Encefálica.
Guillain Barré Syndrome is a neurological disease frequently admitted to the Intensive Care Unit, especially if there is associated hemodynamic or ventilatory compromise. Brain death is the irreversible cessation of all functions of Central Nervous System, and rarely, Guillain Barré Syndrome can mimick it, with subsequent recovery in the next few days or weeks. We report a case admitted to the Intensive Care Unit, with this type of evolution.
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Síndrome de Tolosa-Hunt: Hallazgos en Resonancia Magnética Cerebral antes y después del tratamiento con Terapia Corticoidal Sistémica.
Tolosa-Hunt syndrome is characterized by painful ophthalmoplegia due to a granulomatous inflammation in the cavernous sinus. Corticosteroid therapy dramatically resolves both clinical and radiological findings. We report a case of a patient 55 years old, initially managed as cavernous sinus thrombophlebitis, and by means of magnetic resonance imaging studies, was then diagnosed as Tolosa-Hunt syndrome. After receiving adequate therapy, clinical symptoms improved. We recommend serial magnetic resonance imaging studies when Tolosa-Hunt syndrome is suspected in order to differentiate it from other cavernous sinus lesions that can mimic it clinically and radiologically.
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Una propuesta para la evaluación de la Prueba de Wada en pacientes analfabetos: Presentación de un caso.
In the preoperative neuropsychological evaluation of certain patients with epilepsy it is important to determine the hemispheric dominance; particularly in those of doubtful laterality, due to the high postoperative risks of affectation of the language and the memory. The test of Wada allows guiding the neurosurgeon about the possible impact of the surgical procedure in the hemispheric areas related to the language and the memory. Although it is well-known that the execution in diagnostic neuropsychological tests is under the influence of an important series of variables, as the culture and the educational level, until now there are not specific protocols for this kind of exploration in illiterate patients. In this study we describe the experience in the test of WADA of an illiterate patient with epilepsy diagnosis and cerebral congenital left hemiatrophy, of left-handed manual laterality. With base in the protocol of Trenerry and Loring the language tasks and memory were implemented according to the patient’s cultural context. It was found a right hemispheric representation for the language and the memory. These results confirm the effects of the cerebral plasticity in the reorganization and /or compensation of the cognitive functions, as the language and the memory in the cerebral hemisphere not affected.
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Post-stroke hemiballismus and Contralateral tremor: Report of two cases.
Involuntary abnormal movements may occur as part of the symptomatology of acute stroke or may be delayed or progressive. We report two cases of post-stroke hemiballismus and contralateral tremor. Both patients presented acute hemiballismus. In one of the patients, the tremor started with acute symptomatology and the other was delayed. We discuss the possible pathogenic mechanisms for their movement disorders. To our knowledge, this is the first report of two cases with the unusual presentation of post-stroke hemiballismus and contralateral tremor.
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Las enfermedades neurológicas en el Ecuador: Un llamado a la acción.
Not Available.
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Descripción y Valoración Pronóstica de la Hemorragia Cerebelosa no Traumática.
Spontaneous cerebellar hematomas (CH) represent 5%–10% of intracranial hemorrhages The purpose of this study was to describe CH characteristics in patients admitted to the critical care unit, to determine the prevalence of risk factors, to describe the clinical presentation, the treatment and the clinical situation at discharge. Thirty four consecutive patients were included. The mean age was 67±15 years. In the univariant analysis, only Glasgow Coma Score was associated with high mortality. An hematoma diameter > 3 cm., intraventricular hemorrhage and hydrocephalus were associated with mortality, but without statistical significance.
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The incongruities of the NPI−Q score obtained by the caregiver versus that obtained directly from the non−demented patient with Parkinson’s disease.
Background: Neuropsychiatric symptomatology frequently accompanies Parkinson’s disease. Purpose: In this work (a one point time analytical study), we wished to explorer the Neuropsychiatric Inventory in its shorter format (NPI–Q) in seventy consecutive patients. To find out in what way information obtained from the caregiver (CG) is correlated with that which the patients provide. If this tool, designed to evaluate patients with dementia, can be applied to non-demented subjects with Parkinson’s disease (NDP).
Method: The study, the NPI–Q Spanish version was employed, first with the CG alone and subsequently, with the individual patient alone by a researcher with no knowledge of the previous result from the CG. The weighted kappa correlation coefficient was measured to evaluate the CG–NDP consistency rate (Test–retest reliability; obtained by judging the reproductibility or stability of a instrument over time; two or more observers; or two or more times); a value of 0.7 or higher was accepted as significant. Because the correlation results were found to be insignificant, a post–hoc analysis was performed. We study the convergent validity (Validity–Construct–related, include examining the logical relations that should exist with other measures, know too as convergent validity) analyzed using the Spearman rank correlation statistic values greater than (r <0.29 are weak correlations; r <0.3– .58 are moderate correlations and; r < 0.59 are high correlations the convergent validity).
Results: The consistency inter-rater (CG–NDP), were without any significant agreement; in the inter- tems correlations the best values were for the patients; and in the Spearman’s correlations (a measure of converging validity), the values obtained on the NDP were more significant.
Conclusions: We can assume that the patient’s NDP is the best source of information, and hence for these patients, we consider that the NPI–Q (reported by the CG) is not the best tool to evaluate the Neuropsychiatric symptomatology of NDP sufferers.
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Aneurismas intracraneales múltiples Presentación de un caso y revisión de la literatura.
Prior to review of literature, we present the case of a 51 year old woman, with arterial hypertension, who presented with a hemorrhagic stroke due to multiple intracranial aneurysms rupture (totally 4). Family history reported a sister died because of the same cause. The patient underwent aneurysms clipping, with a non-complicated after–surgery period. She was discharged in satisfactory general and neurological conditions. Nowadays, she undergoes a normal life, with no neurological deficit.
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Migraña y anticonceptivos.
Migraine is a pathology predominant in women; being 2 to 3 times more prevalent than in men. It’s frequently associated with physiological changes in ovarian steroids levels, during the menstrual cycle and in menopause. Despite this strong association, few studies have examined the mechanism through the ovarian steroids influence the neural system in migraines. Clinical data demonstrate that high levels of estrogens increase the probability of having migraine. Paradoxically the sudden descent of estrogens during menstruation increases the probability of having a migraine attack.
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Síndrome de Rett Revisión de la literatura y presentación de seis casos en el Hospital Pediátrico Dr. Roberto Gilbert E.
Rett syndrome is one of the most common causes of mental retardation in females. It is an X linked dominant inherited disorder characterized by regression of language and motor milestones, acquired microcephaly, severe mental retardation and loss of purposeful use of hands. Its hallmark is a repetitive “hand-washing” movement. In the majority of cases, defects on genes MECP2 and CDKL5 have been identified. On the following report we present our first experience involving Rett Syndrome patients, analyzing their main clinical and electroencephalographic features, progression of disease and its treatment. We also make an update of genetics and treatment options. We believe that this study will provide the necessary tools to make an early diagnosis in our environment, reach the most accurate treatment and the appropriate genetic counseling for the families.
