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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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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Enfermedad de Lafora: Diagnóstico histopatológico de un caso por biopsia de piel.

Lafora disease constitutes one of the most common types of progressive myoclonic epilepsy in South Europe, especially in Spain. Its onset is between 10 and 18 years old, with myoclonus which can be segmentary, erratic, intentional and associated frequently with focal occipital seizures. There could also be primary or secondary generalized tonic-clonic seizures. A progressive dementia is evident as is the presence of intra citoplasmic inclusion bodies (Lafora bodies) in neurons and other glucose consuming body cells. The diagnosis can be done by identifying Lafora bodies in the sweat glands of the underarm skin. There are two genes fundamentally implicated in this disease: the EPM2A in 6q24 and the EPM2B in 6p22. We present, to our knowledge, the first documented case in Ecuador, diagnosed by histopathological material obtained by skin biopsy, and analyze its clinical, electroencephalographic and evolving characteristics.  Patient’s family tree is analyzed in detail and an exhaustive literature update related with this disease is performed.

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Charcot: Superando el tiempo.

Not Available.

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Georges Gilles de la Tourette: El hombre y su tiempo.

Not Available.

 

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Verdaderos valores del ejercicio profesional.

Not Available.

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Valor del Potencial Evocado Auditivo de Latencia Media en el estudio de personas con Esclerosis Múltiple Forma Brote–Remisión.

A prospective study was carried out to establish the utility of Auditory Middle Latency Response (AMLR) in the evaluation of patients with relapsing- remitting multiple sclerosis. Twenty subjects were evaluated with the multimodal battery of auditory, visual and somatosensory evoked potentials, AMLR, and motor evoked potential by transcraneal magnetic stimulation. The results showed the following  abnormalities: 60 % in the AMLR, (only 50 % of them with clinical symptoms), 25% in the auditory brainstem response, 85 % in the visual response and 90 % in somatosensorial and motor potentials. We found significant differences between the auditory tests and the  rest of the electrophysiological techniques (rate comparison, p<.05). Those differences disappeared when auditory tests were considered together. There was a significant association between anatomical and functional tests in the evaluation of the auditory pathway, and a positive  correlation between the absolute latency of Na, Pa, and Pb components and the temporal course of the disease. The results suggest the convenience of including AMLR in the battery of evoked potentials for the study of relapsing- remitting multiple sclerosis patients.

 

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Subtipos de Enfermedad Cerebrovascular: Análisis del registro de la Unidad de Ictus del Hospital Clínica Kennedy.

The main goal of this study was to determine the relative incidence of the different subtypes of stroke in a group of people from Guayaquil, considered of a relatively high socioeconomic stratus, to establish a relationship that was previously proposed as a probable cause of the etiologic stroke subtype variation found in our city. We conducted a retrospective study, in which were identified 100 consecutive patients with acute  first-ever stroke admitted to the stroke unit of Hospital-Clínica Kennedy. The results were: male prevalence (57% vs 43%), mean age of onset 70 years for infarcts and 54 years for hemorrhages. Sixtythree patients had a cerebral infarction and 37  had an intracranial hemorrhage. As conclusion, we found that this relatively high prevalence of hemorrhages were related to rupture of  intracranial aneurysms, and that most infarcts were related to hypertensive vasculopathy. We also found that the main risk factors were  arterial hypertension and cardiopathies (p<0.05). Is important to resolve the controversies that were provoke in the last years about onset  variability in types and subtypes of stroke, for which more studies and further are required.

 

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Cadena de supervivencia del Ictus: Un desafío de la medicina actual.

A quasi experimental study was carried out in patients with cerebrovascular disease, through the stroke survival chain at the Municipality Holguín, in order to characterize the access getting of these patients to the secondary care unit at the Hospital Provincial Docente V. I. Lenin, from May to October 2006. Neurological evaluation and cerebrovascular risk scales were applied, obtaining the following results: ages from 60 to 74 years were the more affected, predominantly in male. Atherotrombotic cerebral infarcts were the more frequent. Only 8 patients (23,5%) arrived to the hospital during the first three hours of the event. High blood pressure, history of cerebrovascular  disease and cardiopathies were the more frequent risk factors. Glasgow coma scale above 11 points was observed in all the cases. We conclude that, in order to reestablish cerebral blood flow after stroke, it is necessary to participate in a whole performance survival chain.

 

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Efectos de la Terapia con Campos Magnéticos en los Niveles de Fatiga de las personas con Esclerosis Múltiple.

Introduction: Multiple Sclerosis (MS) is a demyelinating and neurodegenerative disease of the Central Nervous System. It is the major cause of neurological disability in the young adult. One of the most frequent and refractory symptoms to treatment is fatigue. Objective: To evaluate the efficacy of treatment with Magnetic Fields for fatigue produced by MS.

Material and Method: An almost experimental-type intervention study was performed in 14 patients evaluated at the Psychomotor Evaluation Lab and diagnosed with MS (Mc Donald et al) who presented fatigue. The Impact Fatigue Scale (IFS) was applied before and after the treatment with 1750-l magnetic bed was conducted for 4 weeks and 20 minutes, 50 Gauss. Statistics tests used were Spearman and ANOVA within the statistics program. The level of significance was p<0.05.

Results: The sample was composed by women (86%). The most frequent clinical form of MS was the Secondary Progressive (64.3%). Fatigue was found in 92.9% of cases, with an evolution period of 6 months or more. This is one of the symptoms that produced greater disability in these patients. After treatment with magnetic fields there was fatigue improvement in 92.9% of cases according to the evaluation of the fatigue impact scale. Tolerance to treatment was excellent.

Conclusion: Therapy with magnetic fields produced improvement of fatigue symptom in this group of patients with MS, especially in the progressive type.

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La Creatinfosfokinasa tiene utilidad en la Evaluación Pronóstica Temprana de Discapacidad en el Infarto Cerebral.

Introduction: Cerebral infarction is the third cause of morbidity and mortality in the occidental world. The diagnosis is usually based on clinical and imaging studies, like computerized axial tomography. However, the prognostic value is limited especially in the first 48 h after the event. Currently, creatinphosphokinase tests are not known as a prognostic tool.

Objective: To study the creatinphosphokinase serum levels in patients with cerebral infarction in the first hours after the event, to determinate prognosis and establish a relation with disability.

Patients and methods: Forty hospitalized patients with cerebral infarction were studied. Levels of creatinphosphokinase in serum were measured at 12, 24 and 48 hours after infarction and were associated with disability using the Rankin modified scale.

Results: The creatinphosphokinase concentration increased during the first 12 hours after infarction, was higher at 24 hours and, at 48 hours, it began to decrease. The levels of creatinphosphokinase are correlated with disability results in the Rankin scale.

Conclusions: The creatinphosphokinase can be a useful tool in the prognostic evaluation of disability in patients suffering brain infarction, in the first hours after the event takes place, and preceding tomographic alterations.

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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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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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