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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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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Tomografía Computarizada Craneal en la intoxicación por Metanol.

We report two cases of patients who ingested methanol and suffered a cerebral affectation demonstrated in the cranial CT as hypodensities in the putaminal nucleus and cerebral white matter, compatible with necrosis.

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Síndrome de Deleción del Brazo Corto del Cromosoma 18 (18p) por Traslocación Balanceada 6p/18p Materna asociada a Holoprosencefalia Alobar.

We present a case of alobar holoprosencephaly and cebocephaly in a newborn girl associated to a short arm deletion chromosome 18, as a result of a maternal balanced traslocation 6p/18p.

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La neurología en el Ecuador.

Not Available.

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Mielitis Crónica por HTLV-1: Resultados en 45 Casos Estudiados.

ntroduction: HTLV-I myelitis is a chronic disease, endemic in several countries, with well-established clinical and diagnostic criteria.

Methods: Etiology and immunology are reviewed based on 45 patients diagnosed and followed up in our neurology service since 1992. We evaluate the inclusion criteria , assessment scale (Osame`s motor disability scale) and blood, CSF, radiological (specially MRI) and neurophysiological studies for the diagnosis.

Results: Motor, sensory and long tract signs predominated. Patients resulted positive for HTLV1 by ELISA and Western Blot in blood and CSF. These results were similar as those previously reported.

Conclusions: We confirm the existence of chronic HTLV-1- associated myelitis in Ecuador, with an endemic focus in Esmeraldas region. It is necessary to perform new neuroepidemiological studies in order to obtain a better prevention and control of this disease in medical centers, blood banks and to monitor infected nursing mothers.

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Concurrent Validity of the Hamilton Depression Rating Scale and the Beck Depression Inventory versus the ICD-10 Diagnostic Criteria among Patients with Parkinson’s Disease.

Objective: To examine the concurrent validity of the Hamilton Depression Rating Scale and the Beck Depression Inventory for quantifying depression in patients with Parkinson’s disease, using the ICD-10 Diagnostic Criteria as the gold standard, and to determine if the somatization items considered are pertinent.

Methods: The study involved one hundred and forty consecutive PD patients –102 men and 38 women– with a mean age of 68.7 years and mean disease duration of 6.7 years. Sensitivity, specificity, positive and negative predictive values and likelihood ratios were obtained with a 95% CI. ROC Curves (AUC) were also performed.

Results: Based on ROC measurement of discriminative ability, our results suggest that both scales were poor at recognizing mild depression, somewhat better at recognizing moderate depression and adequate for distinguishing severe depression, though with poor specificity. Comparisons of HDRS-21, HDRS-12, BDI-21 and BDI-16 to determine concurrent validity all gave similar results for each depression level and no important differences between the complete scales (all 21 items) and abbreviated forms (without somatic items) were noted.

Conclusions: We conclude that both scales possess similar psychometric properties, but our results cannot be compared with those of other studies that used DSM-IV criteria as their gold standard. These observations led to the following conclusions: (1) the evaluation scales and criteria that comprise them were not designed for PD; (2) the somatic items observed in our patients were a product of PD; and (3) as the severity of the illness increased, so did the number of items that were confused as elements of depression.

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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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Ependimoma Mixopapilar Extradural Subcutáneo simulando un Quiste Pilonidal.

Objective: To describe a case of a subcutaneous, extradural, retro sacral, myxopapillary ependymoma which presented as a pilonidal cyst.

Case description: A 6-year old boy presented with a painful intergluteal mass. The histopathologic examination revealed an ependymal neoplasm with conspicuous myxopapillary appearance.

Conclusion: These tumors are extremely unusual in extradural locations, and their biological behavior is more aggressive than those cases of similar histogenesis localized in the conus medullaris-filum terminale region.

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Enfermedad Celiaca. Alteraciones Neurológicas y Enteropatía Asociada a Linfoma de Células T.

In celiac disease, gluten ingestion results in intestinal mucosal swelling, cryptic hyperplasia, and villi atrophy in genetically predisposed individuals. We report an unusual case of celiac disease, neurological complications and enteropathy associated T cell lymphoma in the same patient.

Case report: A 49 year-old woman with celiac disease who failed to respond to a strict free-gluten diet. She developed intestinal perforation, T cell lymphoma associated to enteropathy and neurological features.

Conclusion: An uncommon case of celiac disease with associated neurological manifestations and malignancy. In a single patient, this uncommon association has not been reported. It is still to be determined, whether genetic and/or immunological factors are responsible for the development of this features.

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Meningioma Quístico: Reporte de Caso y Revisión de Literatura.

Cystic meningiomas are uncommon tumors easily confused as cystic-component glial tumors or metastases. There is controversy regarding about cyst wall origin. Magnetic resonance has improved diagnosis showing dural adhesion of the tumor. We report a case of a patient diagnosed with a cystic meningioma tumor, treated in our neurosurgical service.

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Hipoplasia-agenesia de la arteria carótida interna.

Hypoplasia of the internal carotid artery is an infrequent congenital anomaly, rarely reported by medical literature. We report a case of stroke in the left medium cerebral artery territory, in which an agenesia-hypoplasia of the right carotid artery was diagnosed. After performing a doppler echography, the following findings led to suspect it: a significant difference between the flow speeds of the internal  carotid arteries through continuous doppler study on the hypoplastic side through the submandibular window; an early disappearance of the spectrum; and the  persistence of an invariable blood flow in the medium cerebral artery on the hypoplastic side when compressing the common carotid artery. With the study of the multi-slice helicoidal computerized tomography, the diagnosis  was confirmed corroborating the existence of hypoplasia of the carotid hole in the skull base. Agenesia was also  found in the distal portion of the internal carotid artery. The left medium cerebral artery was supplied by the basilar  artery through the posterior communicating artery. The relationship with the stroke seems to be casual as shown by the characteristics of neuroimaging.

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Trombosis Venosa Cerebral en una Paciente con Enfermedad de Hodgkin y Embarazo: Presentación de Caso Clínico y Revisión de la Literatura.

Cerebral venous thrombosis is an uncommon problem with serious medical consequences. It presents with a wide range of signs and symptoms as any cerebral syndrome where neuroimaging plays a key role. Among the conditions that produce or predispose to venous thrombosis are hypercoagulable states, inherited or acquired. We report a case where the association of pregnancy and malignancy (Non-Hodgkin Lymphoma) induced the development of cerebral venous sinus thrombosis.

 

 

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Chagas Disease: Complementing Supplements.

Trypanosoma cruzi infection is far for being solved in Latin America. Recent research is strongly biased to basic investigations mostly at molecular levels putting aside important clinical involvement and epidemiological findings. Here, we present evidence that Trypansoma cruzi affect neural systems including the autonomous one. Oral and genital routes of transmission of this parasite as well  as the cross-reactions and false-positives described with different serological tests including those used to test HIV must be checked out before putting the almost always fatal diagnoses of Chagas disease.

 

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Pálido-Ansotomía Lenticular: Un blanco quirúrgico efectivo en la Enfermedad de Parkinson.

Briefly we described the posteroventral pallidotomy technique introducing a new target situated more anteriorly to the classic Laitinen´s pallidotomy, in this way we obtain relief in the whole motor signs of Parkinson´s disease. We believe it is the result of the interruption of efferents fibers from pallidum to motor thalamus, and coagulation of the majority of somatomotor cells of the posteroventral pallidum.

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Hamartoma Hipotalámico.

The association between hypothalamic hamartoma and gelastic epilepsy has been described several years ago. This type of tumor, usually asymptomatic, when it is related to epilepsy, can cause intractable seizures and a particular type of laughter crises. A brief analysis of the clinical features and treatment is performed.

 

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Farmacogenomia psiquiátrica.

Not Available.

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Tratamiento del Blefaroespasmo y Síndrome de Meige con toxina botulínica. Experiencia y seguimiento en 18 casos.

Treatment with botulinum toxin (BT) is a good therapeutic alternative for patients with blepharospasm and Meige’s syndrome. We report follow up results of 18 patients with blepharospasm and Meige’s syndrome treated with botulinum toxin, to asses therapy response. 67 injection sessions were performed with a mean dose of 40.2 ± 26.5 UI. The duration of effect was 120 ± 50 days and the global assessment of the results on a scale from 0 to 4 (0 no results to 4 very good results) was an average of 3.1 ± 0.72. We can say that botulinum toxin therapy was good or very good in 14 (77.7%) patients with blepharospasm and Meige’s Syndrome. Only 3 cases (16.6%) had significative complications, reversible in all cases.

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Estudio piloto de tratamiento de pacientes con Esclerosis Múltiple con el uso de una formulación homeopática de la Biomodulina T.

25 patients with defined exacerbating-remitting Multiple Sclerosis (MS), with a score between 1 and 5.5 according to Kurtzke’s disability scale, were treated with Biomoduline T at 6 ch during 15 months. After concluding this treatment 83% of the patients were clinically stable according to the disability score. Due to the immunoregulating and anti-inflammatory effects of this product, the possibility of employing this homeopathic variant for the treatment of MS is suggested, considering its low cost of production and the fact that no adverse reactions were referred.

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Pathological changes induced by ototoxicity on spiral ganglion neurons and its peripheral processes.

Deafness is one of the most widespread disabilities in the world. Its most frequent cause is death of cochlear hair cells (located in the organ of Corti), which induces degeneration of spiral ganglion neurons and their peripheral processes innervating the organ of Corti. In a previous light microscopy study using a rat model of ototoxicity, a loss of spiral ganglion neurons was observed since the eighth week of deafness and peripheral processes degeneration since the fourth week. In order to determine the onset of ultrastructural degenerative changes, a transmission electron microscopy study of spiral ganglion neurons and their peripheral processes was undertaken. Rat cochleae sampled after 2, 4, 8 and 16 weeks of deafness and healthy controls were analyzed. Since the fourth week of deafness, Type I spiral ganglion neurons and the myelin sheaths of their peripheral processes showed progressive degenerative changes. Most of the remaining neurons exhibited complete demyelination at sixteen weeks of deafness, resulting in the pathological type III spiral ganglion neurons. These results show ultrastructural degenerative changes of the spiral ganglion neurons and their peripheral processes, before both undergo significant losses.

 

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Alteraciones del reflejo H, reflejo de parpadeo y neuroconducción en pacientes alcohólicos crónicos.

Introduction: The objective of this study was to determine alterations of the Blink-reflex (BR), H-reflex (HR) and nerve-conduction in a group of alcoholic patients. Methods: A cross-sectional study was carried out on 21 alcoholic patients with an average age of 43.5 years. The history of alcohol abuse was 22 ± 7.4 years. At the time of the study, none of the patients had ingested alcohol for more than 30 days. Results: BR component alterations were as follows: R1 right 19.0%, R2 right 57.1%, R2 right contralateral 71.4%, R1 left 9.5%, R2 left 61.9% y R2 left contralateral 52.4%. Only the R2 right and left ipsi and contralateral latancies were significantly prolonged (P < 0.05). The H-reflex was absent in 90.5% of the patients. There was no significant difference in the comparison of sensitive or motor nerve-conduction speed between alcoholics and healthy subjects. Conclusions: The absence of H-reflex was the most frequently found electrophysiological alteration in this group of alcoholic individuals.

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Conversión de Deterioro Cognitivo Leve a Demencia.

Great social efforts and sanitary conditions have decreased child mortality rates, decreased birth rates and increased life expectancy. Consequently, the proportion of adults has increased. The increased incidence of neurodegenerative disease such as dementia occurs in parallel to this demographic transition. Dementia may be associated with several risk factors. However, few studies have examined the rate of cognitive and functional decline.

Objective: To determine the prevalence of cognitive decline and dementia, risk factors and report results of cognitive and functional decline in patients in the National Institute of Neurology and Neurosurgery.

Methods: We included all outpatients aged ≥ 50 years attending between 1999 and 2000. We sought to asses the Petersen criteria for the mild cognitive impairment (MCI). Patients were assessed using the Consortium to Establish a Registry for Alzheimer’s Disease, Diagnostic and Statistical Manual of Mental Disorders (4th edition), and the National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer’s Disease and Related Disorders Association criteria. We followed for up to 8 years.

Results: A total of 239 individuals were included. The conversion of MCI was of 25.6% per year and 56.4% at the eight years. The consumption of alcohol were associated with dementias (p<0.01).

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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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