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Descripción y Fundamentos de las Técnicas de Imagen Vasculares en el Diagnóstico de la Enfermedad Cerebrovascular
Palabras clave: enfermedad cerebrovascular,
Descripción y Fundamentos del SPECT y el PET en el Diagnóstico de la Enfermedad Cerebrovascular
Palabras clave: 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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Palabras clave: atrofia muscular espinal infantil,
atrofias musculares espinales,
mecanismos moleculares,
Keywords: spinal muscular atrophies,
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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Palabras clave: Enfermedades Neurológicas,
estudio epidemiológico,
Keywords: epidemiological survey,
neurological disorders,
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.
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Improvement of Movement Disorders with Mirtazapine: A Preliminary Open Trial
We performed a non-controlled open trial in 22 patients who had movement disorders. Patients received 30 mg of Mirtazapine per day. Twenty patients (90.9%) had a favorable response and their scores on the severity and functional scale improved after treatment. The time needed to control abnormal movements was 30 days in almost 70% of the subjects. Further randomized controlled trials could determine the effectiveness of Mirtazapine for movement disorders.
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Hemorragias Cerebrales Lobares. Resultados Quirúrgicos
Stroke is the third cause of death in Cuba and in developed countries and intracerebral hemorrhage represent between 15-30% of all acute stroke. An observational descriptive study was conducted in 20 patients who had lobar spontaneous intracerebral hemorrhages and underwent surgery in our Service from January to December,2000. The patients were evaluated at admission by Kanaya system and the results by Glasgow outcome scale (GOS). arterial hypertension was the commonest risk factor (71.42 %). Computed axial tomography (95%) and carotid angiography (75%) were the diagnostic tests performed during the first 24 hours after receiving the patients in our emergency services. All the patients had lobar hemorrhages between 20-60 cm3. The etiology was unknown in 70% of cases,10% showed intracranial aneurysms, 10% brain tumours and 10% haematologic disturbance. Open surgical techniques were used in 80% of patients. Recombinant streptokinase was used twice during surgery to attain the total lysis of the clot . There were 4 deaths(20%) in the reported cases.
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Síndrome de Guillaín-Barré. Experiencia en el INNN. Búsqueda de Factores del Mal Pronóstico.
Objectives: This article describe the Guillaín-Barré syndrome in a Mexican population. Materials and Methods: We review the charts of 40 patients admitted to the National Institute of Neurology and Neurosurgery “Manuel Velásco Suárez” in México City, and we contact them by telephone in order to know their current outome. Results: We find pathological antecedents in 62% of patients. The clinical patern was classical in 62.5%. The functional grade at admission was II in 17.5%, III in 55% and IV in 27.5%. Electrophisiologic tests showed 72% of secundary axonal dammage, of them, 35% was severe. Twenty-two patients (55%) were admited to the Intensive Care Unite, with 70% of them requiring mechnical ventilation, and with a mean admission time of 17.8 days. 22.5% of patientes were treated with immunoglobulins, 7.5% underwent plasma exchange and 70% did not recive specific treatment. Average total hospitalization was 31.8 days. The twelve months outcome was good (Class A) in 77.5%, regular (Class B) in 15%, and bad (Class C) in 7.5%. Conclusions: The electrophysiologic pattern was directly related with the outcome. This was perhaps the same situation of the time of admition and later management with the outcome. The specific treatment and the outcome did not show any correlation. In the group of Class A patients, 50% were treated with IG vs 37.5% without treatment and 12.5% with plasma exchange. Thie study is limited by its retrospestive characteristic and by the lack of disponibility to specific treatment.
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Demencia y el Vaso Sanguíneo
Palabras clave: demencia,
Enfermedad de Alzheimer,
Evaluación Pulmonar en la Esclerosis Lateral Amiotrófica
Palabras clave: Esclerosis Lateral Amiotrófica,
Aspectos Históricos del Tratamiento Farmacológico de la Epilepsia
In the present work, we make an historical revision on the different treatments that had been employed for epilepsy since ancient times through the present. This article reviews different periods such as: the believe between the Incas and the Aztecas that the cause of epilepsy was the action of malignant spirits and the basements of the treatments in magical and religious methods; the starting of pharmacological treatment in the XIX century with the use of bromides, the discovery of the antiepileptic drug phenobarbital by Alfred Hauptmann in 1912, phenytoin by H. Houston Merrit and Tracy Jackson Putnam in 1937, through the introduction of new antiepileptic drugs such as lamotrigine, tiagabine, vigabatrine, levetiracetam, etc. in the nineties.
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Aneurisma Gigante de la Bifurcación de la Arteria Cerebral Media: Reconstrucción con Clipaje y Microsutura
The key for the treatment of giant intracranial aneurysms is its exclusion from the circulation and restoration of the normal anatomy. This report describes the technique of the middle cerebral artery bifurcation reconstruction using microsuture aneurysmorraphy and clipping.
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Tratamiento Sistematizado de la Hemorragia Subaracnoidea
Hemorrhages account for 20% to 25% of stroke cases in our population, Among these patients, subarachnoid hemorrhage accounts for 7% to 10% of cases, and represent the main cause of stroke-related death. In the present study we present a model for systematic treatment of this condition with particular emphasis of early diagnosis and agressive therapeutic approach. We performed an observational study of 117 patients with subarachnoid hemorrhage admitted between January 1995 and August 1999 who were managed according to internationally accepted algorithms. Arterial hypertension and smoking were the most common risk factors. While angiography of intracranial vessels was performed in every patient, we could only find saccular aneurysms in 32 patients (27.36%). About 60% of those patients were operated on, with a mortality of less than 5%. Mortality was directly related to the clinical status of the patient on addmision, and aggessive therapy reduced the chance of death.
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Neuroprotección en isquemia cerebral aguda. Estado actual e importancia clínica de la cascada isquémica.
Knowledge of physiophatological mechanisms of cerebral ischemia permits understanding the mechanisms of action on which many aspects of treatment are based and the clinical and neuro-radiological changes. The only treatment with clinically demonstrated success is the use of thrombolytic to restore the cerebral circulation. A cellular and molecular mechanisms such as free radical production, lipid peroxidation, excitotoxicity and calcium ion overload constitute the important therapeutic targets of neuroprotection and it is now known that interventions such a delivering neuroprotective agents can participate to salvage a portentially reversible ischaemic region known as the ischaemic penumbra. We review the vascular and biochemistry that produce necrosis of neurons after a cerebrovascular occlusion and actual evidence of neuroprotective drugs.
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Enfermedad Vascular Cerebral en los Trópicos
Objectives. To review the clinical manifestations, diagnosis, and therapy of diseases causing cerebrovascular disease (CVD) in the tropics. Development. Most prevalent conditions causing CVD in the tropics include: sickle cell disease, Takayasu’s arteritis, cysticercosis, infective endocarditis, Chagas’ disease, viral hemorrhagic fevers, gnathostomiasis, leptospirosis, snake bites, cerebral malaria, puerperal venous thrombosis, and tuberculosis. These conditions may cause cerebral infarcts or hemorrhages, and in most instances are related to either vascular damage secondary to angiitis or hemorrhagic diathesis with bleeding in other organs. In some patients, the severity of the neurological picture makes impossible to identify an specific stroke syndrome and cerebrovascular complications are only recognized on neuroimaging studies or autopsy. Conclusions. There is a group of tropical infectious and non-infectious diseases that may cause cerebral infarcts or hemorrhages. Prompt diagnosis and therapy are needed to reduce the severity or brain damage and to avoid recurrent strokes.
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Rizotomía Prepontina para el Tratamiento de la Neuralgia Trigeminal Secundaria a Infarto de Tronco Cerebral
A 62-year-old woman presented with severe pain in the distribution of the second and third branches of the left trigeminal nerve. Pain was related to an infarct located in the brainstem at the entry of the trigeminal nerve. As pain was refractary to medical therapy, we performed a selective prepontine trigeminal rhizotomy. The procedure resulted in total relief of pain with preservation of sensation in the distribution of the trigeminal nerve.
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Palabras clave: nervio trigémino,
Rizotomía Prepontina,
Keywords: prepontine trigeminal rhizotomy,
trigeminal nerve,
Epidemiología de la enfermedad cerebrovascular hemorrágica en la región central de Cuba
Introduction. Cerebrovascular diseases are the third most important cause of death in Cuba. Among the programmes given priority by the Cuban Ministry of Public Health and the World Health Organization (WHO), hemorrhagic disorders are the most ‘lethal’. Objective. To determine the ‘behaviour’ of hemorrhagic cerebrovascular disease in the central region of Cuba. Patients and methods. An exploratory, descriptive, retrospective study in which 1,401 clinical histories were reviewed. The variables being investigated were then processed using the computer data processor EPINFO 6.0 to determine the mean, standard deviation and chi squared. Results. An annual incidence rate of 84.03 per 100,000 persons was seen for hemorrhagic cerebrovascular disease; 54.16 and 29.86 per 100,000 persons respectively for intracerebral hemorrhage and subarachnoid hemorrhage; and total mortality of 68.95%. The disorder was associated with possible meteorological factors and the highest percentage was due to intracerebral hemorrhage with 64.45%. Arterial hypertension was the most intensely studied risk marker (p< 0.01). Most cases were elderly (p< 0.001). Surgical treatment was given to 81 patients with subarachnoid hemorrhage, with a predominance of malformations of the middle and anterior cerebral arteries. Treatment was based on depletion using Manitol and calcium antagonists. A considerable number of patients required mechanical ventilation and vaso-active drug support. Most were attended in Intermediate Polyvalent Treatment Units and the main complication was sepsis. Conclusions. There is a high mortality from hemorrhagic cerebrovascular disease in the central region of Cuba, and once the diagnosis has been made the prognosis is usually poor. The patients included in the programme of attention for subarachnoid haemorrhage showed promising results.
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Pseudotumor cerebral: análisis de nuestra casuística y revisión de la literatura
Introduction. Pseudotumor cerebri (PC) is a complex syndrome characterized by increased intracranial pressure in the absence of any space occupying lesion, usually self-limiting, but often relapsing. Diagnosis is based on a record of intracranial pressure of over a limit of 250 mmH2O. Morbidity is basically due to possible loss of vision associated with atrophy of the optic nerve. When the aetiology is unknown this clinical condition is known as idiopathic intracranial hypertension. Patients and methods. We describe the principal characteristics of a series of patients who were admitted to our department with the diagnosis of PC and review the relevant literature. Results. Altogether 14 patients (12 women and 2 men) were admitted with the diagnosis of PC. In 12 patients the disorder presented with headache, with or without associated symptoms of disorders of vision. The main visual symptoms were transient darkening of vision, scotomas, photopsias and reduction in visual acuity. The headache was holocranial, continuous and well-tolerated or alternatively uni- or bilateral, pulsatile, moderate, with or without photophobia, and with or without nausea and vomiting. Bilateral papilloedema was seen in all patients. In one case there was also bilateral facial palsy and neck rigidity. In four cases visual field measurement were abnormal (inferoneasal scotoma, bilateral concentric reduction). Progress was poor in only one case. Conclusion. In spite of being theoretically benign, and there being many forms of treatment, PC may cause considerable morbidity of vision. Therefore, early diagnosis and close follow-up attention is essential.
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Palabras clave: presión intracraneal,
pseudotumor cerebral,
Keywords: intracranial pressure,
Pseudotumor cerebri,
Aspectos Prácticos de las Infecciones Bacterianas del Sistema Nervioso Central
Palabras clave: meningitis piógena,
