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

Artículos de Revisión

 

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.

 

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Algunas Reflexiones sobre la Microcirugía de los Aneurismas Intracraneales.

A correlation between the authors´s surgical experience clipping brain aneurysms and current endovascular techniques is made.
The authors recommend early repair of aneurysms micro surgically or endo vascularly. Best results in microsurgical treatment are possible in young patients with small anterior circulation aneurysms operated in good clinical grade, without evidence of clinical or radiological vasospasm. The endovascular treatment is recommended in poor grade patients, elderly people, and as a complementary mean in the management of giant and complex aneurysms.

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Neuroprotección en la Isquemia Cerebral. Experiencias con la Eritropoyetina Humana Recombinante.

The current therapeutic strategies are oriented to reestablish the cerebral blood flow and to protect the nervous cells during cerebrovascular disease. Searching neuroprotective agents has been guided to block some of the molecular events that nervous cells suffer as a consequence of ischemia. Human recombinant  erythropoietin constitutes a recent proposal, demonstrating to have neuroprotective mechanisms of action at  more than one level, appearing to be a short term promising option. Its erythropoietic action can represent an convenience for chronic treatments or in secondary prevention. The use of an erythropoietin with low content of sialic acids –with neuroprotective activity but not erythropoietic– may be a good option.  This molecule should be administered by a non systemic route as is the intranasal in order to prevent the hepatic degradation. Intranasal administration of human recombinant erythropoietin has shown to  be quick and safe in accessing the brain, it does not stimulate erythropoiesis in acute treatments and it shows  therapeutic effectiveness in  several models of cerebral ischemia in rodents. This proposal can become a therapeutic option in cerebrovascular diseases.

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Aspectos Neuropsicológicos de la Resonancia Magnética Funcional.

Cognitive neuropsychology and functional magnetic resonance (RMf) have marked a particularly fertile period in the development of neurosciences. Their clinical value, although incipient, is unquestionable. Integration of these two disciplines allows the registration  of cerebral images related to the neuronal activation of specific areas, providing structural and functional information of the human brain  under normal and pathological conditions. The RMf has important clinical and research applications. For example, pointing the brain  areas needed to be avoided during surgery, or observing the neurofunctional changes that take place with a  pharmacological treatment or neuropsychological rehabilitation. It also permits increasing the knowledge of  neurofunctional bases of different cerebral pathologies. It  is of special interest and importance the selection and implementation of the activation paradigm on functions to be studied, condition that  is required for the correct interpretation of the results. The procedure for RMf allows the study of a specific component in the  cognitive process in response to a specific task. It doesn’t identify the function as a unique feature, but it  identifies neurofunctional patterns. In this  article, the basic theoretical-methodological neuropsychological aspects are described for the implementation of studies by RMf.

 

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Neurogénesis en el Cerebro Adulto: Promesas y Posibilidades en la Enfermedad de Parkinson.

Dopamine is an important neurotransmitter implicated in the regulation of mood, motivation and movement. Here, we have reviewed recent data suggesting that dopamine, in addition to being a neurotransmitter, also plays a role in the regulation of endogenous neurogenesis in the adult mammalian brain. In addition, we  approach a highly controversial question: can the adult human brain use endogenous neurogenesis to replace the dopaminergic neurons in the substantia nigra that are lost in Parkinson’s disease?

 

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Activación Plaquetaria: Aspectos básicos, participación en la Enfermedad Cerebrovascular y Proyecciones Terapéuticas.

Platelets activation processes comprise very complex signalling pathways which involve a G protein system, coupled to effect enzymes such as phospholipase A2, phospholipase C, guanilil and adenilil cyclases. Closely related functional properties and special estructural  characteristics of platelets confer them a very high trombogenic potential, which play a role in the development of stroke, from  the primary hemostasis.  Basis of platelet aggregation, activation, degranulation and platelet participation in cerebrovascular disease were reviewed. Usage of antiplatelet agents, and novel anti-membrane-glycoproteins therapies were emphasized.

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Meningoencefalitis Eeosinofílica por Angiostrongylus Cantonensis. A un cuarto de siglo de una enfermedad emergente en las Américas.

Introduction: 25 years ago an emergent disease: eosinophilic meningoencephalitis due to Angiostrongylus cantonensis larvae. was first reported in Cuba and in the western hemisphere.

 

Objectives: To collect in a summary form the accumulated experience and the findings of the study of this parasitosis during the period.

Results: It has been collected the first evidences, the parasitological and malacological findings, the experimental and molecular studies on immunology and neuroimmunology, the clinical-pathological findings in children and adults with the particularities of this diseases in our environment with special accent in the never-before scientific findings reported.

Conclusions: It has been documented the testimony, the main findings among the malacological studies, the role of IgE and the mechanism involved in the central nervous system, the intrathecal synthesis patterns of immunoglobulins and the clinical elements in children and adults.

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Aspectos Moleculares de los Trastornos Cognitivos Ligados al Cromosoma X que Conllevan una Disfunción del Lenguaje.

Molecular characterization of X-linked cognitive disorders in which language impairment is a prominent symptom decisively contributes to a better understanding of the genetic programme involved in the development of the language organ. Proteins encoded by genes mutated in these disorders are structurally and functionally diverse: (i) helicases, (ii) homebox proteins, (iii) transcriptional regulators, (iv) translational regulators, (v) posttranslational regulators, and (vi) phosphoproteins. These proteins ultimately modulateneural development and function, as far as they seem to be involved in the regulation of (i) neural migration, (ii) neural identity, (iii) axonal growth, (iv) dendritic proliferation, (v) synaptogenesis, (vi) myelinization, (vii) neural plasticity, (viii) long-term potentiation and (ix) neural survival, but also in the modulation of basic cellular processes, like (x) chromosome segregation. Though these genes clearly make up the genetic programmes responsible for the emergence of different cognitive modules, they should be taken necessarily into account for an accurate molecular characterization of the language organ, since it is only programmes, but not genes, that should be properly regarded as idiosyncratic (i.e. most genes cannot reasonably be characterized as “linguistic”, unlike the programme itself related to the language organ).

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A strange case of Comorbidity in a 60-year-old Portuguese war veteran: War Post Traumatic Stress Disorder, Early Fronto- Temporal Cerebral Atrophy, and Strong Neuropsychological Symptomatology. A Neuropsychological Review.

The Post-Traumatic Stress Disorder is a psychiatric disorder that derives from traumatic events. Although the literature and clinical reports of PTSD is known along centuries, only in 1980 was recognized by the American Psychiatric Association as a diagnostic category of the Diagnostic and Statistical Manual – III. The present article reports the case study of a man with Post-Traumatic Stress Disorder, initial signs of fronto-temporal dementia and other neuropsychological and neuropsychiatric symptomatology. Decurrent of the neuropsychological evaluation was possible to detect a variety of alterations in the humour and personalistic sphere, as well as deficits in orientation, memory, attention, concentration and also in the functions regulated by pre-frontal cortex. This study suggests not only the importance of a deep neuropsychological evaluation, as the necessity to integrate the deficits showed by the patient with the evidence from literature.

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Terapia Celular en la Enfermedad de Parkinson.

The pathological hallmark of Parkinson’s disease is a gradual loss of nigrostriatal dopamine-containing neurons and is responsible for cardinal motor symptoms of the disease. Current therapeutic strategies are mostly based on pharmacological enhancement of the dopaminergic neurotransmission. This therapeutic approach has several long-term side effects, such as dyskinesias and fluctuations of response and is therefore limited in its use. Transplantation of fetal dopaminergic precursor cells has provided the proof that a cell replacement therapy can ameliorate clinical symptoms in affected patients. Novel therapies aiming at a stimulation of an endogenous dopamine production within the brain at a continuous rate might provide a more physiological and elegant way to overcome the dopaminergic deficiency in parkinsonian brains. This article will review recent studies demonstrating the potential of these alternative cell graft sources for treating Parkinson’s disease.

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Aspectos moleculares de las enfermedades metabólicas que conllevan trastornos del lenguaje.

 

Molecular characterization of metabolic diseases in which language impairment is a prominent symptom decisively contributes to a better understanding of the molecular effects and ontogenetic context on the development of the language organ. Main etiological feature in these diseases is actually diverse, as it may be disturbed in: 1) hormone homeostasis; 2) sugar, creatine, carnitine, lipid, sulphur, organic acid, or aminoacid metabolism; 3) different cellular processes (lysosomal storage and mitochondrial function). Metabolic disturbances ultimately lead to structural and functional anomalies in different brain regions, which positively correlate with the linguistic and cognitive impairments. Functional cloning has greatly helped to identify genes involved in such metabolic diseases, and so to a better understanding of the innate programme involved in the development of the linguistic module.

 

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La Memoria de Trabajo y su relación con otras funciones cognitivas en la Enfermedad de Parkinson.

Parkinson’s Disease (PD) is a neurological illness due to degeneration of the dopaminergic neurons of the nigro-striatal pathway. Each of the five frontal-subcortical circuits contributes in a different way to PD’s symptoms. The clinical manifestations are variable; they include non cognitive deficits like: rigidity, tremor, bradykinesia, and other motor deficits like hypomimic (mask-like face), hypophonia, dysarthria, dystonia and abnormal postures among others. The emotional disorders in PD include depression and anxiety. The frontotemporal dementia in PD has been described. The neuropsychological examination of the PD patient is done considering whether the main symptoms are bradykinesia and rigidity, since these patients show more severe cognitive decline. We must examine the attention, which is between the normal limits in simple tasks, but it shows deficits in more complex tasks; memory and learning are disturbed, orientation is intact; in verbal functions we evaluate vocabulary, syntaxes and grammar which are relatively intact, although the sentence length tend to be reduced; the visuospatial disorders are frequent in PD; thinking and reasoning must also be examined, in both of them there is normal performance and they show a realistic appreciation of their condition and limitations; the executive functions are evaluated, there are consistent failures in tests that require concept formation and thinking flexibility. PD treatment can be medical using L-Dopa, or surgery through techniques by lesion or stimulation to different surgical targets like the internal globus pallidus, the subthalamic nucleus, or the ventral medial thalamic nucleus, unilateral or bilaterally. The PD repercussions in working memory (WM) will be studied related to executive functions, speech, cronometraje, saccades and attention.

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Neuroimagen de la reorganización cortical en pacientes con lesión medular.

Introduction: Spinal cord injury promotes an afferent and efferent disconnection from the cortex causing neurologic deficits. Many imaging studies have shown that neuroplasticity occurs after effective treatments in patients with spinal cord injury.

Objective: The purpose of the study was to make a bibliographic review of the treatments applied after spinal cord injury, studying neuroplasticity and how supraespinal structures react to these treatments as shown by neuroimaging tools such as functional magnetic resonance imaging (fMRI).

Development: Treatments including cell transplantation, antibodies against myelin inhibitors or against antagonists of their receptors, surgical rebuilding methods and motor rehabilitation, can compensate the loss of function of the areas with injury. This improvement is characterized by cortical activation changes.

Conclusion: We conclude that the treatments currently used produce good results in relation to the sensorimotor reorganization in patients with spinal cord injury. fMRI is a valuable tool for the study of cerebral changes in spinal injury.

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Accidente Cerebrovascular en poblaciones situadas a grandes alturas: Revisión y análisis de los factores de riesgo.

Despite our understanding about cerebrovascular accident, also known as stroke, its risk factors, treatment and prevention, it remains the leading cause of death of both men and women worldwide. Several risk factors have been associated with higher incidence of stroke such as systolic or diastolic hypertension, diabetes, hypercholesterolemia, cigarette smoking or obesity. Other such as cocaine abuse, heavy alcohol consumption, AIDS, Vitamin D and protein C deficiency are also important non traditional risk factors, associated with increased prevalence of stroke. Nevertheless, high altitude exposure and potential impact of hypoxia as a stroke risk factor –in people living or visiting high altitude environments– has not been clearly established yet. This review will analyze the available evidence concerning about the association of risk factors with higher prevalence of stroke during acute or chronic high altitude exposure, either in high altitude dwellers or new sojourners. The author will examine the available evidence behind the possible physiopathology of ischemic and hemorrhagic stroke in those living at high altitudes. We conclude that living or visiting high altitude locations is a potential non traditional risk factor associated with higher incidence of ischemic stroke, especially in young otherwise healthy men. The hypobaric hypoxia level or the altitude in which stroke commonly starts is very difficult to establish. However, it is well known that as soon as hypoxia causes acclimatization, risk becomes higher. The evidence suggests that high altitude exposure and consequent hypobaric hypoxia are important environmental factors to be considered. The time length of exposure, high levels of hematocrit and polycythemia are the most prevalent risk factors associated with higher incidence of stroke in subjects exposed to chronic or acute hypoxia.

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Control de calidad de las publicaciones científicas: Aspectos a considerar en nuestra revista y en revistas foráneas.

Not Available.

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Esclerosis Múltiple y neuroplasticidad: Eventos en la patogenia y proyecciones terapéuticas.

Several forms of neural plasticity can occur in the adult Nervous System activated by experience and regulated by the interaction among the environment, stress, physical exercises, and physiological conditions (i.e. pregnancy). Recent studies demonstrate that specific neuroplastic events occur in Multiple Sclerosis (MS) and its experimental animal model, as well as some punctuate failures in neuroplastic mechanisms. Basic pathogenic mechanisms of MS related to axonal and myelin integrity loss and associated to inflammation processes and the dual roll of inflammation in the neurodegeneration/neuroprotection relation are reviewed. We emphasize in the actual therapies trends in those topics and in the current evidences of neuroplastic changes in MS.

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El marcapasos del parpadeo en un abrir y cerrar de ojos.

Clinical and functional neurological studies have suggested that the motor cortex, M1, does not control facial muscles, mostly the superior ones. Recent studies including current transcranial magnetic stimulation, showed that neural structures related with the cingulate cortex is the main brain region controlling facial muscles in humans. These facts will help clarifying discrepant findings in clinical neurology related to the well known -but not well understood- central facial palsy, among other applications.

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La Atención Involuntaria: Aspectos clínicos y electrofisiológicos.

One of the brain functions presenting more interest within the study of cognitive neurosciences is attention, which has a voluntary and an involuntary component. Involuntary attention is related to the detection of potentially relevant stimulus but originally not attended. It is considered that involuntary attention is made up by at least three stages: a) an automatic mismatch detection of a stimulus related to a given sensory context, b) an involuntary attention change associated to the orientation response, and c) a re-orientation towards the originally carried out task. The three-stage model of involuntary attention has been originated mainly based on the results obtained by Event Related Potentials (ERP) technique, by which three electrophysiological components associated with each stage of the theoretical model have been identified: mismatch negativity (MMN), P3a, and reorientation negativity (RON). This work reviews describing experimental, morphological, and topographic characteristics of each one, as well as their appearance through the development, and their alteration in clinical populations, such as traumatic brain injury, alcoholism and attention deficit disorder, is made. Finally, research perspectives are defined for future studies.

 

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Infarto cerebral y síndrome antifosfolípido primario.

Antiphospholipid syndrome (AFS) is one of the most frequent acquired thrombophilias, is characterized by venous thromboembolism, and/or arterial thromboembolism, and/or pregnancy morbidity, together with the presence of antiphospholipid antibodies. Thrombosis can be at both venous and arterial level, are usually recurrent and frequently affect cerebral circulation. Cerebral ischemia associated with antiphospholipid antibodies is the most common arterial manifestation and the only neurological manifestation accepted for diagnosis. Although it is difficult to predict which patients with antiphospholipid antibodies will develop thrombosis, once a thrombotic event has taken place, secondary prevention with anticoagulation is mandatory. We review the main epidemiological and diagnostic aspects and secondary prevention treatment in patients with ischemic stroke secondary to AFS.

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Utilidad del Levetiracetam como Terapia Adjunta en Pacientes con Crisis Parciales Refractarias.

We conducted a metanalysis of controled trials on the efficacy of levetiracetam (LVT) as add-on therapy for patients with refractory partial seizures. A MEDLINE search revealed five trials comparing the efficacy of different doses of LVT over placebo. Combined data confirmed the efficacy of LVT over placebo for the 50% reduction of seizures during therapy (x2=96.9; p<0,0001; OR=3.73; 95% CI=2.02-4.95), as well as for the complete resolution of seizures (x2=27.01; p<0,0001; OR=7.31; 95% CI=3.08-21.09). Only two trials showed a dose-related (1000 mg/d, 2000 mg/d o 4000 mg/d) efficacy of LVT. This suggest that the initial dose of LVT in these patients should be 1000 mg/d, and that the dose may be increased only in those who do not respond to therapy.

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Lesión del Nervio Espinal Accesorio. Importancia de los Estudios Electromiográficos.

The spinal accessory nerve (XI cranial nerve) injury is an unusual clinical entity and, sometimes, of complex diagnosis. The objective of this study is to describe the syndromic picture attending to its main etiologic factors, the different forms of presentation and the value of the neurophysiological studies, especially of electromyography in its diagnosis. The information that neurophysiology brings is of great value at the moment of establishing a precocious diagnosis as well as in the evolution and prognosis of the lesion. There are few available data in the literature that describe neurophysiological techniques for its correct management.

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Trastorno Obsesivo-Compulsivo: Hacia Un Modelo Integrador.

The main purpose of this paper is to present a general view of the advances in the last three decades concerning the conceptualization and treatment of the OCD phenomena. This disorder is more frequent and heterogeneous than it was thought before. Biological and psychological aspects suggest a crucial task for both, clinicians and researchers: to develop integrated models. Emphasis should be made on the importance of combined treatments to increase well being, social and personal functioning and quality of life.

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Origins of Addictive Behavior: A Nature Heritage Or A Neuropsychiatric Nurtured Reality?

In this paper we present a revision on the origins of Addictive Behavior and related neural basis. Although there are abundant specific bibliography and professions focusing on addictions treatment, the reasons why people started with addictive behaviors and consequently maintain are still not well understood. This article explores the construction, meaning, and impact of addiction in historical and psychological perspective. The challenge to the usual concept of addictions causes has significant implications for medical practice and for treatment programs. Furthermore, with this article we present the phenomena of Multifactorial Polygenic Heritage as a possible theory for the explanation of addictive behavior, being consequently important for its prevention and treatment. The roles of social, cultural, and population differences, as they may relate to addictive behaviors, are explored.

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Muerte Encefálica: Revisión de la literatura y actualización del tema.

One of the most complex situations that neurologists face is brain death. It is a painful state for the patient’s family, but by the other hand, it constitutes the hope for patients waiting for organ donation. Therefore it is essential to have a clear concept to achieve a correct diagnosis and management of this delicate situation.

 

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Emociones y Neurociencia. Una aproximación desde la productividad científica.

The neuroscientific approach to the emotion has been termed affective neuroscience. This multidisciplinary approach has emerged as an influential source of data and theory for the basic, clinical and applied sciences. The aim of this study was to perform a bibliometric analysis over the scientific production in the affective neuroscience field. As in most scientific areas, the scientific productivity in affective neuroscience is lead by North American research centers. Latin-American research in the field is marginal. This asymmetrical observation is a warning to the Latin-American researchers as well as an incentive to improve the scientific productivity in the affective neuroscience field.

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Miopatías asociadas al uso de estatinas: Un enfoque actual.

Statins are the drugs of choice for the treatment of hyperlipidemia, cardiovascular disease, and coronary disease, among others. Their safety and tolerance allows its use on multiple branches in medicine. However, its long- erm use is associated with adverse effects that affect the quality of life of patients, and therefore, their adherence to treatment. Among these, myopathies are described. They represent a variety of clinical presentations ranging from mild myalgia to fatal rhabdomyolysis. In this literature review, the safety of statin use is assessed objectively in order to determine what attitude should be taken regarding the therapy for muscle disorders.

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Utilidad del doppler transcraneal en la evaluación del stroke criptogénico.

Cryptogenic stroke accounts for 40% of patients with ischemic stroke and it is considered that in which after conducting a thorough investigation, a cause cannot be determined. For several years there has been a close association between cryptogenic stroke and paradoxical embolism through right-to-left shunt, which occurred specially in patients with patent foramen ovale (PFO), whose prevalence is significantly higher in patients with cryptogenic stroke than in patients with stroke of a definite cause. The study of this type of cardiac abnormalities has been strengthened today with the advent of new technologies including transesophageal echocardiography (TEE) which remains the gold standard in the diagnosis of PFO, however, there are increasing reports of the usefulness of transcraneal doppler in the evaluation of these patients in a way that makes its recommendation comparable to TEE. This review is intended to expand the knowledge available on this specific procedure according to the current existing evidence.

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La Contribución de la Evaluación Neuropsicológica a la Atención Médica Primaria.

The present review offers a revision of neuropsychological assessment and the contribution that it makes to the diagnosis, prognosis, and treatment of various medical, neurological and psychiatric disorders. Neuropsychological assessment provides the clinician with information regarding the interaction between the patients’ neurologic, psychological and behavioral functioning, which frequently determines clinical management and outcome. This revision presents a summary of the contribution of neuropsychological assessment as a useful clinical tool, with the goal of helping clinicians determine the utility of such an assessment for individual patients.

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