Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative adult onset disease. Presently, ALS is considered a complexed multifactorial disorder of unknown etiology. Consequently, optimal treatment of ALS requires an integral multidisciplinary approach. Early care and palliative support in ALS lead to an improvement in survival and quality of life of patients. A multidisciplinary treatment approach is essential to provide adequate management of symptoms, respiratory problems, psychological, and social support. This review is based on literature with evidence-based practice parameters, which substantiate recommendations to guide clinical decisions in the treatment of ALS. The focus is mainly on how information is provided to the patient in regards to diagnosis and prognosis, symptomatic treatment, decisions about nutrition and gastrulation, respiratory assistance, and palliative care.
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Enfermedad de Moyamoya: Revisión de la Literatura.
Moyamoya disease is an idiopathic cerebrovascular disorder, uncommon in non-Asian population, which is characterized by bilateral stenosis of the internal carotid arteries and their major branches, as well as by the neoformation of vessels at the base of the skull (rete mirabile) and branches originated from the external carotid artery, as a compensatory mechanism due to a chronic ischemia. Its peak age is biphasic appearing around 5 and 40 years of age. In children the predominant pictures are of ischemic types while, in adults it presents itself as hemorrhagic pictures. The digital angiography is pointed out as the gold standard among all the diagnostic methods, although similar results can be obtained with an angio-MRI and angio-CT. The disease’s treatment is merely surgical characterized by a revascularization achievement, either made in a direct or indirect form. The prognosis of these types of patients is not well defined yet; but it has been observed that its worst in which the disease appeared at an early age compared to those in whom it appeared later on.
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Monitoreo Neurofisiológico Intraoperatorio: Utilidad y Ventajas en cirugía de columna.
Injuries to neural structures such as the spinal cord, peripheral nerves, nerve roots, and important vessels often occur during spinal surgery despite the experience and knowledge of the surgeon. Intraoperative neurophysiologic monitoring is an important tool to protect the integrity of those structures. This monitoring is possible due to the electric properties of the nervous system. By the continuous recording of this electrical data over key anatomical regions and periodic stimulation of neural structures to verify neurologic function, the monitoring staff can relay crucial information about the neurological status of the patient to the operating physician. The use of this information by the operating surgeon can help prevent post-operative neurological deficit and improve patient outcomes. Due to the protective and preventative nature of IONM, in some European countries and in the United States of America this test is considered the Gold Standard of the procedures that prevent and minimize neurologic complications during spinal surgery. The incorporation of the neurophysiologic monitoring duringneurological surgeries has begun in many Latin American countries and is just emerging in Ecuador. We attempt in this article to introduce and explain the importance of this support in Neurosurgery, Traumatology and other surgical specialties.
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Sobre Neurociencia y Proceso Judicial.
Neuroscience is a relatively new field of study that is introducing challenges in different fields of knowledge and has begun to generate interest in legal scholars and philosophers. The dialogue and debate between Law and neuroscience has become urgent. Neurociencia y proceso judicial [traducción libre: Neuroscience and judicial process], a work published in 2013, gives a good account of that. Its eight essays written by legal scholars and legal philosophers explores a wide spectrum of issues and suggestive connections between procedural law, philosophy of evidence, legal philosophy, scientific and technological advances on the study of the brain and its functioning, and some of the postulates from certain neuroscientific currents. This article contains a review of that work.
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Estimulación Cerebral Profunda y Enfermedad de Parkinson: Documento Instructivo para el Manejo Multidisciplinario en el Hospital Regional Dr. Teodoro Maldonado Carbo
Neuromodulation by deep brain stimulation (DBS) in the treatment of Parkinson’s disease has developed new research in its pathophysiology. By electrical stimulation of established surgical targets, clinical response can be modified and optimized, improving quality of life. Even though its efficacy, DBS is not indicated for all patients. Selection criteria must be considered and a multidisciplinary team -with roles and responsibilities clearly defined- must evaluate the patient.
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Partial Thenar Atrophy as a Physical Manifestation of Martin Gruber Anastomosis.
Martin Gruber anastomosis is a frequent finding on electrodiagnostic examination and has three common variants. Much has been written about these variants such as the anatomic course of crossover fibers and the electrodiagnostic findings. However, little has been written on associated physical findings that might suggest such a diagnosis. In this report the physical examination findings clearly supported a diagnosis of a Type III Martin Gruber anastomosis that was initially established through electrodiagnostic testing. Awareness of this pattern on physical examination could provide an early clue to the possible presence of anomalous innervation.
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Meningitis Crónica por Angiostrongylus Cantonensis.
Two patients with chronic meningitis due to the helmint Angiostrongylus cantonensis are reported. Two years after the primary infection from a previous outbreak, both patients had pain in lumbosacral region, leg weakness, headache and sleep disturbances among other symptoms. Intrathecal synthesis of IgA, IgM and IgG was found. Nuclear magnetic resonance showed temporal atrophy in one patient and in the other one a T2 hyperdense lesion zone in right parietal region at white zone level. It is the first report of chronic disease because of this parasite in the Americas.
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Encefalomielitis Aguda Diseminada: Reporte de un Caso con Afectación Selectiva de Tallo Cerebral.
Introduction: Acute Disseminated Encephalomyelitis (ADEM) is an autoimmune demyelinating disease of the central nervous system´s white matter; it usually has a monophasic course with an idiopathic cause or following infection or immunization processes.
Objective: This article has the purpose to present a clinical case with selective involvement of brainstem. We describe a 28 year old woman who developed ADEM. Her clinical presentation, neuroradiological findings and treatment are reported.
Conclusions: ADEM is an infrequent disease which can be fatal. MRI findings confirm the diagnosis and steroid therapy appears to be the most effective treatment, although the disease may spontaneously improve.
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Apoplexy of Rathke Cleft Cyst: A Case Report and Literature Review.
Symptomatic Rathke cleft cysts (RCCs) are rare sellar and suprasellar lesions, and apoplexy is one of the most unusual presentations. Only a few cases of hemorrhagic apoplexy of RCCs have been reported and their pathogenesis is still poorly understood. In order to present a diagnostic thread to reduce misdiagnosis rate preoperatively, we reported one case of RCC apoplexy and reviewed the associated published literature. we also summarized the clinicopathological relationship on clinical symptoms, imaging features and intraoperative visualization of intracystic content.
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Hemangioblastoma Sólido de Cerebelo. Presentación de un Caso y Revisión de la Literatura.
Hemangioblastoma is an uncommon benign and highly vascular tumor that is usually observed in the brain, spinal cord and retina. It may be solid or cystic and approximately 25 to 40% of them are associated with von Hippel-Lindau syndrome. Clinical manifestations of hemangioblastoma are nonspecific and they depend on the location and growth pattern of the tumor. Despite its great vascularity, they rarely manifest themselves with hemorrhage and when they do, intraparenchymal bleeding, intramedullary hemorrhage or subarachnoid hemorrhage may be observed. The definitive diagnosis of HB is the pathologic exam. The treatment of choice for hemangioblastoma is microneurosurgery in order to make a complete resection. Preoperative endovascular embolization of feeding vessels may be useful in selected cases as the main risk of this surgery is intraoperative bleeding. We present a case of a 25 years old female diagnosed with right cerebellar hemisphere hemangioblastoma and hydrocephalus, therefore treated through various surgical stages: placing of a medium pressure valve, suboccipital craniectomy, supraselective embolization of feeder vessels and finally extirpation of the tumor mass.
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Epidemiología de la Enfermedad Cerebrovascular en Latinoamérica
Esclerosis Mùltiple, Virus del Herpes Humano Tipo 6 y Chlamydophila Pneumoniae
Background and objectives: An increasing number of papers are showing a relationship between two infectious agents, human herpes virus 6 (HHV6) and Chlamydophila pneumoniae (Cp), and multiple sclerosis (MS), although these results remain controversial. The objectives was a meta-analysis of clinical series published in literature until now. Material and Methods: An electronic database search (Medline, Excerpta Medica and IME) was performed with MS relapsing remitting and the infectious agents as keywords. A strict selection criteria was applied. 16 studies were selected who complained with the methodological criteria. Results: After statistical analysis, we found that HHV6 was related with MS by detection of DNA in plasma and antibodies in serum and cerebrospinal fluid, but not with DNA in cerebrospinal fluid or peripheral blood white cells. Cp was related with MS by detection of DNA in cerebrospinal fluid, antibodies and the isolation of bacteria of cerebrospinal fluid, but not with serum antibodies. Conclusions: A study with enough number of patients and samples, prospective, controlled with healthy subjects and other neurological diseases, with multiple microbiological techniques in the same sample and patient, and correlated with clinical and paraclinical activity parameters, are needed.
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Descripción y Fundamentos de la Tomografía Computada en el Diagnóstico de la Enfermedad Cerebrovascular
Descripción y Fundamentos de la Resonancia Magnética en el Diagnóstico de la Enfermedad Cerebrovascular
Descripción y Fundamentos de las Técnicas de Imagen Vasculares en el Diagnóstico de la Enfermedad Cerebrovascular
Descripción y Fundamentos del SPECT y el PET en el Diagnóstico de la Enfermedad Cerebrovascular
Mecanismos Moleculares y Métodos Diagnósticos de la Atrofia Muscular Espinal Infantil
The spinal muscular atrophies are a group of disease characterized by loss or degeneration of the neurons of the horn previous of the spinal marrow. The bad operation of the same ones makes that the nervous impulse cannot be transmitted correctly so that the movements like the muscular tone are affected. The SMA is classified in five groups: SMA proximal, variants of the childhood, non proximal, paralysis bulbar and espinobulbar Kennedy type. In turn, the Infantile Spinal Muscular Atrophy (SMAi) that is a variant of the childhood, it is classified in three groups in dependence of the appearance age and clinical severity. One of the genes responsible for this disease is known as gene of the survival of the neuron (SMN), it is located in the chromosome 5 (5q 11.2 – 13.3), it presents two copies one in the region telomérica (SMN t or SMN1) of the chromosome and another in the centromérica (SMNc or SMN2) and it codes to the protein Smn. Until the moment a good therapy is not known for this illness, so that the molecular diagnosis is of great importance to improve the quality of life of the affected families.
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El Abordaje Subfrontal: Es en realidad Minimamente Invasivo?
Subfrontal key-hole approach has been considered more traumatic to the brain than the traditional pterional approach. We describe some technical tips and make recommendations to choose this approach to some specific target areas. We conclude that the subfrontal key-hole is as minimally invasive as the pterional approach if it is performed in the correct settings.
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Enfermedad de Lyme. Primer caso Reportado en Ecuador.
Lyme disease is a multisystem infectious disease caused by the tick-borne spirochete Borrelia burgdorferi. CNS involvement occurs frequently. Meningitis, multiple cranial nerve palsies, neurophaties, radiculophaties, and encefalophaties are particularly common. The diagnosis should be done carefully; epidemiology, clinical features, and laboratory findings are the basis in the management of disease. In this paper we present a 12 year old Ecuadorian boy who fulfills diagnosis parameters for Lyme disease according to the American Academy of Neurology. This is the first reported case of this disease in Ecuador. It is necessary to carry out epidemiological studies to confirm the occurrence of this condition, and the look for the presence of the transmitting vector and its causal agent in our country.
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Síndrome de Kabuki
Kabuki’s mask syndrome is a rare developmental disease. Most cases are sporadic. Diagnosis is based on five criteria, including slight mental delay slight, skeletal abnormalities, dermatological alterations, slow stature, and typical fascies. We describe the first diagnostic case of Kabuki’s syndrome in our country.
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Estudio Puerta-a-Puerta de Enfermedades Neurológicas en Atahualpa, una Población Rural del Litoral Ecuatoriano — Metodología y Definiciones Operacionales
We describe the methods and operational definitions of an epidemiological door-to-door survey for the detection of neurological disorders in Atahualpa, a rural community of approximately 2,600 inhabitants, located in the coastal region of Ecuador. We will determine the prevalence of epilepsy; the prevalence and incidence of cerebrovascular disease; the prevalence of dementia; the relationship between smoking, alcohol ingestion, arterial hypertension, diabetes mellitus, and stroke; the relationship between cysticercosis and epilepsy; and the prevalence of Taenia solium infection among individuals who tested positive for anti-cysticercal antibodies in serum. We hope that improved epidemiological knowledge on some differences of expression that such diseases may present according to racial, cultural, and socio-economic factors, may improve the control and management of these conditions in Atahualpa.
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Síndrome Febril sin Causa Aparente en Lactantes Menores de 3 Meses: Utilidad de la Punción Lumbar
Febrile syndrome without apparent focus in children under 3 months old is a common problem in clinical practice. Here, we evaluate the role of lumbar puncture in this group of patients to assess the frequency of viral meningitis. We analyzed 253 medical records of patients hospitalized in the Seclusion Service of Roberto del Río Hospital from January 2001 to January 2003. The age ranged from 2 to 87 days; 55.7% of patients were younger that 28 days, and 86.6% were younger than 2 months. Most common manifestations were fever and poor appetite. Laboratory tests showed abnormal WBC in 28% of patients. CRP and ESR were impaired in 1.6% and 10.7% respectively. Blood cultures were positive in 3.2%, and urinalysis were abnormal in 30 patients. We performed lumbar puncture in 223 of 253 patients (88%). It yielded abnormal CSF findings in only 14 children. Viral meningitis was diagnosed in 10 patients and bacterial meningitis 4 patients.Pyelonephritis or occult bacteremia were confirmed in 11.8% and 4.7% respectively. Treatment was started in 125 patients, but only 62 completed it. We conclude that lumbar puncture is a diagnostic tool in febrile infants to rule out diseases having high morbidity and mortality rates, that must be receive prompt treatment. In febrile infants under 3 months of age with no apparent focus, bacterial disease must be suspected
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Relación Entre Patrones de Sueño y Ansiedad / Depresión
In recent years contradictory data as to relationship between the sleep patterns and psychological variables have appeared. This study analyses the relationships of depression and state-trait anxiety levels in subjects with different sleep patterns. From 583 subject sample, 25 long sleep pattern , 25 medium sleep and 25 short sleep pattern were selected. The results show that no relationships between psychological variables and sleep patterns.
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Síndrome de POEMS: Múltiples manifestaciones clínicas durante su larga evolución.
Being a multisystemic disorder, POEMS syndrome has different clinical features.and comprises multiple organs. The referred acronym: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein M and skin changes, results insufficient when this syndrome is described, because of the constellation of multiple signs and symptoms. Neurologically a chronic demyelinating peripheral neuropathy is the common clinical presentation, which is associated with an osteosclerotic myeloma in 50% of cases. Involvement of central nervous system includes papilledema and intracraneal hypertension, less frequently. We report a patient with POEMS syndrome with 11 years follow-up, who has recently developed intracraneal hypertension features. We discuss clinical findings and review literature.
