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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Paraproteinemic Neuropathies: A Review for Neurologists Neuropatías. Paraproteinémicas: Revisión de tema para Neurólogos

As part of the workup of polyneuropathy, neurologists often encounter patients with paraproteinemias, also called monoclonal gammopathies. Given the relatively rising prevalence of both polyneuropathy and paraproteinemias in our aging population, this coexistence is frequently due to chance. Still, certain types of paraproteinemias have a well-defined causal role in the development of polyneuropathy, and therefore has implications in their subsequent work-up and management. Once a monoclonal gammopathy has been deemed as “benign” or of undetermined significance, looking at the heavy chain subtype is paramount in deciding whether it is related to a polyneuropathy. Conversely, polyneuropathies associated with monoclonal gammopathies tend to have a distinct clinical, serological, and/or electrodiagnostic phenotype that helps in establishing an association. Recognizing this association will determine approp riate workup and management.

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Embolismo gaseoso cerebral: a propósito de un caso de ictus masivo. Cerebral air embolism: about a case of massive stroke

Introduction: Cerebral air embolism is a rare but potentially fatal entity, that occurs due to the entry of air into the cerebral blood circulation and is caused by multiple causes, including t hose associated with medical procedures.

Clinical case: We present the case of an 81-year-old male patient who, after removal of the high-flow hemodialysis catheter, begins with a deterioration of the level of consciousness, which worsens progressively. Multiple hypodense lesions, compatible with gas embolism, are evidenced in the cerebral tomography. Due to his slow evolution, he was transferred to the intensive care unit and died a few hours later.

Conclusion: It is essential to know the preventive measures to avoid this complication and the general and specific measures to adopt when it occurs.

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Psicosis orgánica, tipo trastorno esquizofreniforme, posterior a hemorragia subaracnoidea. Organic psychosis, schizophreniform disorder type, after subarachnoid hemorrhage

Organic psychosis refers to a group of diseases currently classified as “mental disorders due to a medical illness”, unlike the absence of specific organic causality that exists in primary disorders. These are secondary to long-term processes , which is necessary to re-evaluate frequently due to the association between age-related deterioration, comorbidity and the evolution of psychosis itself. We present the case of a patient with subarachnoid hemorrhage secondary to a ruptured arteriovenous malformation, who suffered from a slight change in behavior and decreased cognitive functions with subsequent evolution to dissociative symptoms, episodes of hypersomnia and disconnection from the environment, through assessment joint of specialties was reached the diagnosis of organic psychosis type schizophreniform disorder.

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Infarto cerebeloso bilateral simultáneo en un paciente con fibrilación auricular paroxística no valvular: una presentación atípica. Simultaneous bilateral cerebellar infarction in a patient with paroxysmal atrial fibrillation: an atypical presentation

Introduction: Cerebellar infarcts usually occur in the territory of the posterior inferior cerebellar artery (PICA) and tend to be unilateral. Simultaneous bilateral involvement is extremely rare.

Case report: We present the case of a 67-year-old male who developed an acute cerebellar syndrome secondary to acute infarction in the territory of both PICA confirmed by nuclear magnetic resonance imaging. Non-valvular paroxysmal atrial fibrillation was the most plausible etiology after digital cerebral angiography ruled out vascular abnormalities.

Conclusion: This case shows that bilateral infarction in the territory of both PICA can occur in the context of cardiac embolism, even in the absence of an anomalous common PICA.

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Infarto cerebral en mujeres. Stroke in women

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Disfunción neurológica y exposición a agrotóxicos. Neurological dysfunction and exposure to agrotoxics

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Plantas medicinales: Comprendiendo sus beneficios para la salud y advirtiendo sobre sus efectos psicoactivos y neurotóxicos. Medicinal plants: Understanding their health benefits and warning about their psychoactive and neurotoxic effects

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Beneficios de la musicoterapia sobre el lenguaje. Benefits of music therapy on language

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Embarazo y esclerosis múltiple: ¿Existe una relación positiva entre ambas entidades? Pregnancy and multiple sclerosis: Is there a positive relationship between the two?

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Factores asociados al tiempo libre de discapacidad en pacientes con Esclerosis Múltiple de un instituto de neurología en Medellín, Colombia. Factors associated with disability-free time in patients with Multiple Sclerosis of a neurology institution in Medellin, Colombia

Introduction: Multiple Sclerosis is the leading neurological cause of disability in young adults. The objective of this study was to describe the time free from disability since diagnosis and to analyze associated factors.

Method: Retrospective follow-up study of a cohort based on records of patients who consulted the Neurological Institute of Colombia between 2013 and 2021. Progression to disability was defined as the time to obtain a value greater than 3.5 on the EDSS scale. Parametric survival model for interval censored data with Weibull distribution was used to estimate survival function and Hazard Ratios.

Results: 216 patients were analyzed. The median disability-free time was 12 years (95% CI 7.56-17.66). Cerebellar complications (aHR = 8.18; 95% CI 4.09-16.35), vision (aHR = 2.25; 95% CI 1.25-4.05) and age at diagnosis over 40 (aHR = 1 .28; 95% CI 0.68-2.4) were associated with shorter time to progression, other initial symptoms (aHR = 0.35; 95% CI 0.18-0.68), stem lesions (aHR = 0.6; 95% CI 0.33-1.1), MSRR (aHR = 0.17; 95% CI 0.08-0.33) and women were associated with a longer time to progression.

Conclusion: Clinical and imaging factors allow identifying patients at high risk of rapid progression to disability.

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Correlación entre variables clínicas sugestivas de hipertensión intracraneal y alteraciones en la neuroimagen en trauma craneoencefálico. Correlation between clinical variables suggestive of intracranial hypertension and alterations in neuroimaging in traumatic brain injury

Methods: Retrospective observational cohort study where patients admitted for TBI to the ICU area of the HLV were included. 

Results: We included a total of 297 subjects. The most common neuroimaging lesion was multiple lesions (35.4%). We found that there is a significant correlation between the presence of lesion in neuroimaging and the presence of fixed pupils at admission (p = <0.001), score ≤ 8 on the Glasgow scale (p = <0.001) and need for orotracheal intubation (p = <0.001). Similarly, the same 3 variables were significant when related to the score ≥ III on the Marshall scale. In the logistic regression model, pupil fixation was the only one that was shown to increase the risk of a score ≥ III on the Marshall scale (OR: 3.50, 95% CI 1.53-7.99). 

Conclusion: The clinical variables: pupil fixation, need for endotracheal intubation and Glasgow ≤ 8 are related to the development and severity of lesion on neuroimaging in patients with TBI.

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Entrenamiento sensoriomotor en casa en personas mayores con Enfermedad Parkinson durante el periodo de confinamiento por COVID-19: Estudio piloto. Sensorimotor training at home in elderly people with Parkinson’s Disease during the period of confinement due to COVID-19: A pilot study

The pandemic caused those therapeutic activities associated with people with Parkinson’s disease to be suspended (PD) with the aim of preventing COVID-19 infections by increasing motor and non-motor symptoms, due to social isolation and stress. Therefore, there was a rise of care based on telemedicine to continue the treatment in the context of a health emergency, promoting training at home. The objective of this experimental research was to document the effects of a sensorimotor training program (NM-FITT®) in people with PD through weekly telematic follow-up during the COVID-19 pandemic. A quasi-experimental study was carried out with pre, inter and post test evaluations that characterized the physical, functional condition and quality of life, in an intervention group (n=5) and a control group (n=5) of elderly people (69.5 ±4.5 years) with PD in Hoehn & Yahr stages 1 to 3, for 18 weeks, through an exercise manual, video calls and telephone follow-up. Favorable and significant effects were demonstrated on all the parameters studied after the application of the intervention in detriment of the health condition of the persons who did not participate in the home training program.

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Caracterización histológica de las dendritas de la amígdala por alto consumo de fructosa en un modelo experimental de isquemia cerebral. Histological features of dendrites in the amygdala due to high fructose consumption in an experimental model of cerebral ischemia

Introduction. High fructose consumption has been shown to magnify cerebral ischemic injury in the ischemic focus and penumbra region. However, ischemia also produces changes in exofocal areas such as the amygdala, an important structure in emotional processing. Therefore, the objective of the investigation was to characterize the histological changes of the amygdala dendrites caused by high fructose consumption in an experimental model of cerebral ischemia.

Method. Wistar rats fed with standard food were used; the control group was given water and the fructose (HDF) group was given add libitum 20% fructose in water for 11 weeks. Some rats were subjected to cerebral ischemia. Therefore, there were four experimental groups: Sham control, Sham HDF, Ischemia control, Ischemia HDF. 50 um coronal sections of the brains were made and microtubule-associated protein 2 (MAP2) immunohistochemistry was performed. Images were captured and processed in Image J software.

Results. Loss of dendrite immunoreactivity was found in ischemic groups, and also cluster-type MAP2 immunoreactivity in HDF groups.

Conclusion. According to the above, both ischemia and high fructose consumption generate dendritic alterations in the amygdala.

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Relación entre factores de riesgo y la distribución topográfica en niños con parálisis cerebral. Relationship between etiological factors and topographic distribution in children with cerebral palsy

 Introduction: Cerebral palsy is a health condition that seriously impacts the life condition of children and their family. Different risk factors are becoming increasingly important in its etiology.

Objective: The aim of this study was to analyze the most frequent risk factors for cerebral palsy and their association with topographic distribution in children aged 0 to 15 years in the city of Barranquilla.

Methods: An analytical observational cross-sectional survey type study was conducted with a sample of 78 children diagnosed with cerebral palsy in the city of Barranquilla, where the association of the most frequent risk factors of this condition with the topographic distribution was analyzed.

Results: The risk factors associated with cerebral palsy in order of importance were: perinatal hypoxia, gestational history of maternal infections, maternal trauma and congenital malformations. There was statistically significant association between gestational weeks at birth and GMFCS level (p<0.05) and between gestational weeks and topographic distribution.

Conclusions: Subjects with higher gestational age presented greater involvement at the level of motor function and a more extensive topographic distribution.

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Hipernatremia como factor pronóstico de mortalidad en trauma encefalocraneano severo. Hypernatremia as a prognostic factor of mortality in patients with severe traumatic brain injury

Background: A severe traumatic brain injury is a frequent pathology, it implies a non-negligible morbidity and mortality. Hypernatremia could be considered a factor associated with poor prognosis in order to optimize treatment in the group of high-risk patients. We aim to determine if hypernatremia is a prognostic factor of mortality in patients with severe traumatic brain injury in a public hospital which is a local reference center in trauma.

Material and Methods: We performed a non-probabilistic sampling of adult patients of both sexes with severe traumatic brain injury from the Intensive Care Unit of the Hospital Regional Docente de Trujillo, Perú during the 2015–2018 period.

Results: Mortality in the group with and without hypernatremia was 38% and 6% respectively (p=0.001). The difference in natremia mean values between the group with and without mortality was 21,86 (95% CI 18.2-25.5) (p=0.000). In the multivariate analysis, we found hypernatremia with adjusted OR of 16.73 (95% CI 1.96-142.82) (p=0.01). The ROC curve showed adequate performance to predict mortality, with an AUC 0.878 (95% CI 0.77-0.97) (p=0.00).

Conclusion: Hypernatremia is a prognostic factor of mortality in patients with severe traumatic brain injury.

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Psychiatrists available in the Ecuadorian public health system and psychiatry residency programs in Ecuador – A cross-sectional analysis. Psiquiatras disponibles en el sistema de salud público y programas de residencia en psiquiatría del Ecuador – Un análisis transversal

Introduction: In 2008, Ecuador’s mental health profile displayed deficiencies due to the lack of psychiatrists and psychiatry residency programs. The same year, access to universal health care, was instituted as a right in the Constitution. There are no studies on the Public Health System’s capacity to provide mental health services, hence an updated analysis of the number of psychiatrists and psychiatry training programs available is required.

Objective: To determine the number of psychiatrists available through the Public Health System and to calculate the ratio of psychiatrist per 10,000 population; to establish the number of residency programs available.

Methods: Cross-sectional analysis of the data available from the Ecuadorian Public Health System and the Higher Education Council of Ecuador. Ratios were calculated using data from the National Institute of Statistics and Censuses.

Results: Ecuador’s psychiatrists ratio is 0.08:10,000 and the adjusted ratio (psychiatrists and psychologists) is 0.65:10,000. The Public Health System has 138 psychiatrists, concentrated in Pichincha (n=51) and Guayas (n=27). Three provinces had no psychiatrists available. There are two psychiatry training programs in Quito.

Conclusion: Ecuadorian Public Healthcare holds a shortage of psychiatrists which inhibit the minimum ideal ratio fulfillment, along with a noticeable lack of psychiatric training programs availability.

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Alteraciones en el sistema nervioso producido por la obesidad. Alterations in the nervous system produced by obesity

Introduction: The central nervous system as well as the cardiovascular system are target organs, susceptible to endogenous and exogenous injuries. In this sense, the role that obesity has in relation to the direct or indirect affection towards the nervous tissue is little known, however, there is evidence that suggests that the decrease in cerebral blood flow as well as the exaggerated production of pro-inflammatory cytokines could be associated with damage to cellular tissue including gray matter and white matter.

Objective: The objective of our study was to review the available scientific literature on the macro and microstructural changes caused by obesity in the nervous system.

Methodology: Searches were made in English and Spanish in PubMed, GoogleSchoolar and Scielo using the following search terms: “OBESITY” OR “OVERWEIGHT” AND “BRAIN STRUCTURE” OR “GRAY MATTER” OR “WHITE MATTER” OR “BRAIN VOLUME”. We included articles since 2000 to 2021.

Results: The results show that obesity is associated with a decrease in brain tissue volume, possibly at the expense of a decrease in white and gray matter, which also causes demyelination and diffuse axonal damage in patients with body mass index > 30.

Conclusions: There are different mechanisms that affect the nervous system, producing macro and microscopic changes in obese patients, among which are decreased blood flow, alteration of the blood-brain barrier, production of proinflammatory cytokines, production of reactive oxygen species, among others, which lead to nerve cell apoptosis, decreased white matter volume white and gray matter volume, axonal damage among others.

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Construcción teórica neuropsicológica de las funciones ejecutivas. Theoretical neuropsychological construction of executive functions

Executive functions (EF) constitute a large conglomerate of cognitive abilities that, as their name indicates, carry out decisive actions in decision-making and human behavior. They are of a cognitive and socio-emotional nature, they coordinate the input and output information to regulate the behaviors, thoughts, and emotions of a task to be performed. Alexander Luria raises its conceptualization at the beginning of the seventies, being Muriel Lezak the one in charge of its scientific dissemination. In this article, a bibliographic compilation on EFs, their best known theoretical models, their neuropsychological evaluation process, the most common disorders and the most relevant studies in recent years is made.

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Calcificaciones ganglio basales simétricas bilaterales (Enfermedad de Fahr) en pediatría: Reporte de caso. Bilateral symmetric basal ganglia calcifications (Fahr’s disease) in pediatrics: Case report

Introduction: Fahr syndrome is defined as the presence of predominantly bilateral symmetric calcifications in the basal ganglia (mainly striated nucleus). It is considered primary when the etiology is genetic, which is also known as Fahr’s disease; and secondary if metabolic alterations, endocrine alterations, exposure to radiation, infections, vascular disorders, mitochondrial diseases or others, are found, being the most frequent: hypoparathyroidism and pseudo-hypoparathyroidism. Fahr’s disease is a pathology of genetic origin, rare in pediatrics, that is associated with movement, neuropsychiatric and cognitive disorders. 

Clincal case: We present the case of a 9-year-old boy with evidence of bilateral symmetric basal ganglia calcifications on brain computed tomography (CT) scanning. In the literature there are very few descriptions of this finding in pediatrics. Clinically, the child presented WITH epilepsy, right hand dystonia, mild cognitive deficit and progressive impairment of speech and gait. He was clinically diagnosed as a Fahr’s disease, given the abscense of other metabolic or endocrinological alterations, without genetic studies, and treatment with Levodopa-carbidopa was started for the management of dystonia with clincal evidence of improvement, an a comprehensive rehabilitation program was indicated.

Conclusions: The finding of bilateral symmetric basal ganglion calcifications should lead to rule out endocrinological or metabolic alterations; In the event that they do not occur, all possible related manifestations in the cognitive, psychiatric, motor, speech and other fields should be handled in a comprehensive manner.

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Encefalitis autoinmune por anticuerpos anti Iglon5, reporte de caso clínico. Autoinmune encephalitis due to anti Iglon5 antibodies, clinical case report

Introduction: New clinical entities related to the discovery of autoantibodies either against intracellular antigens or against cell membrane surface receptors or synapses are being identified with increasing frequency. In the latter group, patients with apnea have been described in recent years, REM and NON-REM sleep behavior disorder, respiratory failure and presence in serum and CSF of antibodies against the LON5 antigen. These antibodies appear to alter the interaction with the cytoskeletal system of the neuron and induce accumulation of TAU, conditioning a wide clinical expression.

Clinical case: 76-year-old male patient with eight years evolution of symptoms before diagnosis. He had a mixture of respiratory symptoms, parasomnias, ataxia, movement disorder, autonomic symptoms, cognitive and mood disorder, and associated a chronic demyelinating polyneuropathy. The diagnosis was confirmed by detection of antibodies against IGLON5 in cerebrospinal fluid.

Conclusion: Anti-IGLON5 antibody encephalitis is a unique entity that challenges what we know about autoimmunity and neurodegeneration, leading to a diagnostic challenge due to the wide expression of symptoms and the possibility of improvement with early treatment.

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Enfermedad de Wilson: A propósito de un caso neuropsiquiátrico de diagnóstico tardío. Wilson’s Disease: About a late-diagnosed neuropsychiatric case

The comprehensive assessment of a psychiatric case requires evaluating the symptoms from a comprehensive perspective, without bias, and suspecting unlikely pathologies where appropriate. We emphasize this from a case of Wilson’s disease in which, as is frequent, the diagnosis was late. Clinical case: 36 year-old woman who from the age of 18 presented depressive and later psychotic symptoms, and her diagnosis was paranoid schizophrenia. The three-year diagnostic delay led to a poor evolution with permanent disabilities. The biased diagnostic attribution about parkinsonism as secondary to antipsychotics clouded the adequate diagnostic evaluation. Our case shows the need for a comprehensive neuropsychiatric perspective in the diagnostic evaluation. In a first case of psychosis, Wilson’s disease should be considered within the differential diagnosis.

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Meningioangiomatosis y displasia cortical focal. Meningioangiomatosis and focal cortical dysplasia

The association between meningioangiomatosis (MA) with focal cortical dysplasia (FCD) has been scarcely published. We present the case of 15-year-old adolescent suffering 10 years evolving drug-resistant epilepsy without history of neurofibromatosis. Magnetic Resonance Image showed an increase in the volume of the hippocampus and the right parahippocampal region. The lesion was considered as a possible tumor. A right temporal lobectomy, guided by trans-surgical electrocorticography (EcoG) was performed. Histology of the resected tissue evidenced a FCD type IIIc (MA mainly vascular associated to FCD). The patient has been seizure free (according to the Engel IA scale) after 4 years of post-surgical evolution. When MA is suspected, we recommend trans-surgical ECoG considering the possible association with FCD in the surrounding neocortex. It could increase the incidence and knowledge about these two lesions. The histological study provides the definitive diagnosis.

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CLIPPERS (Inflamación crónica linfocítica con realce perivascular pontino y respuesta a esteroides) con compromiso longitudinalmente extenso de la médula espinal. Una presentación atípica. CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement and steroid response) with longitudinally extensive spinal cord involvement. An atypical presentation

CLIPPERS (Chronic Lymphocytic Inflammation with Perivascular Pontine Enhancement and Steroid Response) is a recently described neuroinflammatory disorder in which symptoms involving the brainstem predominate, with variable pattern on neuroimaging and a perivascular infiltrate of T lymphocytes on brain biopsy. We present a case of a 50-year-old male who presented symptoms of asthenia, adynamia, weakness in lower limbs extremities, sphincter disorder and ataxia. Patient was extensively studied to rule out infectious, neoplastic, autoimmune, and demyelinating conditions. Diagnosis was made taking into account significant clinical-imaging elements, remarking the spinal cord involvement in this patient. Treatment with steroids and steroid-sparing agents, such as methotrexate, was found to be effective. Very few cases in the literature to date describe CLIPPERS-associated spinal cord involvement.

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Trombectomía mecánica eficaz en paciente con ictus e infección por SARS-Cov-2 con desenlace fatal. Effective mechanical thrombectomy in a patient with stroke and SARS-Cov-2 infection with fatal outcome

Ischemic stroke has been reported in patients with SARS-CoV-2 infection. It is not clear if COVID-19 is causal or simply coexists or triggers the onset of stroke. Stroke is relatively rare in the context of COVID-19 and mostly occurs in the elderly with vascular risk factors. The underlying mechanism of stroke is multiple. We present an 84-year-old male with a stroke due to large vessel occlusion coincident with severe COVID-19 infection, that despite an initial successful mechanical thrombectomy, had a fatal outcome due to respiratory complications and contralateral massive cerebral infarction due to early recurrence. Consequently, vigilance in this type of patients should be extreme since ischemic stroke with active SARS-CoV-2 infection may have a poor prognosis.

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Neuroimagen en síndrome de desmielinización osmótica. Neuroimaging in asmotic demyelination syndrome

The osmotic demyelination syndrome includes pontine and extrapontine myelinolysis, the first being its classic form of presentation. Usually related to rapid corrections of hyponatremia (serum sodium less than 135mEq/L), it has been described in multiple conditions that can alter plasma osmolality. The symptoms are varied and include spastic tetra paresis, pseudobulbar paralysis, coma, seizures or the characteristic “locked in” syndrome, as well as behavioral disorders such as lack of impulse control, aggressiveness, depressive syndromes, catatonia, mutism, and emotional lability. Years ago, it was a very difficult entity to diagnose, but with the introduction of magnetic resonance imaging, many oligosymptomatic or asymptomatic cases have been detected, although there is no known treatment, spontaneous recovery can occur.

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Atrofia multisistémica, estudio por imagen de un caso. Multisystem atrophy, imaging study of a case

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El chico con medio cerebro. Boy with nearly half-of-brain

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A watery eye and an enlarged trigeminal nerve. Ojo acuoso y ensanchamiento del nervio trigémino

A 45-year-old man was evaluated one week after the acute onset of pain and numbness in the right side of the face. On examination, he had a right watery eye, which has been present for several weeks. The patient denied history of eye trauma or previous episodes of inflammatory or infected conditions affecting the lacrimal drainage complex. 

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Neurología fuera de la ciudad capital. Neurology outside the capital city

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Criterios de diagnóstico de la enfermedad de Alzheimer: Aplicaciones prácticas. Diagnostic criteria for Alzheimer’s disease: Practical applications

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