RESUMO
Moyamoya disease (MMD) is characterized by progressive stenosis of the distal portion of the internal carotid artery and its two main branches, the middle cerebral artery, and the anterior cerebral artery. Clinically, MMD can present with ischemic or hemorrhagic cerebrovascular events. The term Moyamoya syndrome (MMS) is used when the characteristic Moyamoya vasculopathy presents in association with other conditions such as Graves' disease (GD). We report a case of a 34-year-old, right-handed male patient of Amerindian descent. He presented to the emergency room with a two-month history of palpitation, fatigue, and weight loss associated with sudden-onset left hemiparesis, facial asymmetry, and dysarthria. His workup was remarkable for elevated levels of thyroid hormones with the presence of autoantibodies and radiological findings typical of MMS. Moyamoya syndrome in association with Graves' disease has increasingly been noted in Latin American patients and should be considered in the differential diagnosis in the appropriate clinical context.
RESUMO
Carotid web (CW) is an atypical form of intimal fibromuscular dysplasia that occurs at the level of the carotid bulb. It is associated with ischemic strokes. The first report of this association was in 1967 and it is currently known to represent a significant percentage of cryptogenic stroke. We report the case of a young female patient with a history of transient ischemic attack who presented a cerebral infarction of the territory of the left middle cerebral artery. The diagnosis of CW was suggested by the findings of the ultrasound carotid duplex and was confirmed by digital subtraction angiography. Likewise, brain magnetic resonance angiography showed an incipient alteration in the morphology of the wall of the left internal carotid artery in its intracranial segment. Aspirin treatment was started and there was no recurrence up to two years of follow-up. CW represents a diagnostic challenge; it should be suspected in young adults with ischemic stroke. In them, studies of the supra-aortic vessels should be performed. Ultrasound carotid duplex can be a useful diagnostic tool.
RESUMO
Reversible cerebral vasoconstriction syndrome (RCVS) is an underdiagnosed cause of convexal subarachnoid hemorrhage, characterized by thunderclap headache associated with focal and segmental intracranial vasoconstriction. It can appear complications such as intracerebral hemorrhage, seizures, posterior reversible leukoencephalopathy, or ischemic stroke. Our objective is to present the case of a 51-year-old woman with an RCVS diagnosis, who had a normal digital subtraction angiography at the illness onset. We highlight the high diagnostic value of thunderclap headache and convexal subarachnoid hemorrhage. We also highlight the importance of repeating the angiographic studies in the second week when there is strong diagnostic suspicion.
RESUMO
La fistula carótido-cavernosa es una comunicación anómala entre la arteria carótida y el seno cavernoso que provoca un shunt arteriovenoso patológico y cuyo origen es traumático o espontáneo. Las manifestaciones clínicas están relacionadas con el cambio de dirección del drenaje venoso y del flujo sanguíneo a través de la fístula e incluyen síntomas oculares tales como quemosis, exoftalmos y soplo orbitario, además de manifestaciones neurológicas como infartos venosos y hemorragias intracerebrales. Su presencia se confirma mediante la angiografía por sustracción digital (ASD) y la clasificación generalmente aceptada se basa en la propuesta por Barrow. El tratamiento endovascular ha sido el enfoque terapéutico preferido durante las últimas dos décadas; sin embargo, el tratamiento quirúrgico sigue siendo una opción cuando aquél falla o no está disponible. Se reporta el caso de una paciente de 71 años de edad, con antecedente de traumatismo cráneo-encefálico en la que se diagnósticó fístula carótido-cavernosa izquierda y síntomas neuro-oftalmológicos bilaterales asociados a un infarto venoso a nivel temporal izquierdo. La paciente fue intervenida quirúrgicamente y presentó una evolución clínica favorable.
The carotid-cavernous fistula is an abnormal communication between the carotid artery and the cavernous sinus that induces a pathological arterio-venous shunt, and whose origin may be traumatic or spontaneous. Its clinical symptoms are related to changes in the direction of the venous drainage and blood flow through the fistula, and include ocular symptoms as chemosis, exophthalmos and orbital murmur besides neurological manifestations such as venous infarctions and intracerebral hemorrhage. Its presence is confirmed by digital subtraction angiography and the generally accepted classification is based on Barrow'se proposal. Endovascular treatment has been the preferred treatment approach for the past two decades; however, surgical treatment remains an option when the former fails or is not possible. The case of a 71 year-old woman with a history of traumatic brain injury is reported: the patient was diagnosed with carotid-cavernous fistula and bilateral neuro-ophthalmic symptoms associated with left temporal venous infarction, and underwent surgical treatment with a favorable clinical outcome.
RESUMO
La monoparesia motora pura (MMP) es un déficit motor aislado en una extremidad, superior o inferior, producido por un evento vinculado a enfermedad cerebrovascular. Es un trastorno raro y podría confundirse con otras causas de déficit motor por lo que la diferenciación con otras etiologías se debe realizar a través de una adecuada evaluación clínica y de estudios de neuroimágenes. Se reporta el caso de una paciente de 56 años de edad con antecedente de hipertensión arterial, que debuta súbitamente con una monoparesia motora pura braquial izquierda. En la IRM cerebral se evidenció hiperintensidad en los protocolos de FLAIR y difusión e hipointensidad en el coeficiente de difusión aparente (ADC) en el territorio de arteria cerebral media derecha, segmento M4, correspondiente al área prerolándica en la distribución somatotópica del miembro superior. Se discuten diferentes aspectos etiológicos y de tratamiento de este cuadro. En pacientes que presenten déficit motor agudo de una extremidad, con historia de factores de riesgo vascular, debe considerarse activamente la posibilidad diagnóstica de MMP.
The pure motor monoparesis (PMM) is an isolated motor deficit in an upper or lower extremity usually related to a cerebro-vascular disease. It is a rare condition that can be easily confounded with other pathologies, reason for which should be carefully differentiated through clinical assessment and neuroimaging studies. We report the case of a 56 year-old woman who suddenly presented pure left brachial monoparesis as a manifestation of a right middle cerebral artery stroke. Brain MRI showed a hyperintensity in FLAIR and diffusion protocols, and a subintensity in the apparent diffusion coefficient (ADC) in the territory of M4 segment of the right middle cerebral artery, corresponding to the prerolandic area in the somatotopical distribution of the upper limb. Different etiologies of an management strategies for this condition are discussed. The diagnosis of PMM due to ischemic stroke should be considered in patients presenting an acute motor deficit in one extremity, braquial or crural, and with history of vascular risk factors.
RESUMO
Objetivos: Precisar la etiología del infarto cerebral en adultos jóvenes hospitalizados en el Departamento de Enfermedades Neurovasculares del Instituto Nacional de Ciencias Neurológicas (INCN) durante el periodo 2010 al 2013. Material y métodos: Estudio descriptivo, retrospectivo, de corte transversal; en paciente jóvenes con diagnóstico de infarto cerebral, hospitalizados en el INCN, en el periodo de estudio que tuvieron tomografía y/o imagen de resonancia magnética cerebral. Resultados: Un total de 43 pacientes cumplieron los criterios de inclusión. El 55,8% fueron mujeres. La media de edad fue 37,6+/-6,4 años. El 69,8% provenían de Lima. El 44,2% de los casos fueron de etiología indeterminada, 23,3% cardioembólica. La media de edad para la etiología atero-trombótica fue de 41,8 años, y para la etiología indeterminada de 34,7 años. El territorio vascular mayormente comprometido fue el de la circulación anterior con 65,1%.Conclusiones: Se describen las características clínicas, sociodemográficas y la etiología del infarto cerebral en adultos jóvenes. Los más frecuentes fueron: indeterminada y cardioembólica. Las enfermedades metabólicas y el uso de anticonceptivos orales fueron los factores asociados más frecuentes. No hubo mortalidad. El territorio vascular cerebral mayormente comprometido fue la circulación anterior.
Objectives: Clarify the etiology of cerebral infarction in young adults hospitalized in the Department of Neurovascular Diseases at the National Institute of Neurological Sciences (INCN), during the period 2010 to 2013. Material and Methods: Descriptive, retrospective, cross-sectional study in a sample of young patients diagnosed with cerebral infarction, hospitalized during the study period and who underwent tomography and / or magnetic resonance imaging brain tests. Results: A total of 43 patients, 55.8 % of them female, met the inclusion criteria. The mean age was 37.6 +/- 6.4 years, 69.8% came from Lima, and 44.2% of the cases were of undetermined etiology and 23.3%, cardioembolic. The average age for artery occlusive disease was 41.8 years, and for undetermined etiology, 34.7 years. The vascular territory mostly compromised was that of the anterior circulation with 65.1%. Conclusions: Sociodemographic, clinical characteristics, and etiological factors of cerebral infarction in young adults were described. The most frequent etiologieswerecardioembolic and undeterminated. Metabolic diseases and the use of oral contraceptives were the most frequently associated factors. The cerebral vascular territory mostlyaffected was that of the anterior circulation. No deaths occurred.
Assuntos
Humanos , Masculino , Feminino , Adulto Jovem , Infarto Cerebral/diagnóstico , Infarto Cerebral/etiologia , Epidemiologia Descritiva , Estudos Retrospectivos , Estudos TransversaisRESUMO
La enfermedad de Moyamoya es una patología caracterizada por la estenosis progresiva de la arteria carótida interna y sus ramas principales. Es de etiología desconocida, tiene como forma de presentación a la enfermedad cerebrovascular isquémica o hemorrágica, siendo la primera más frecuente, y afecta en mayor proporción a niños y adultos jóvenes constituyendo un reto diagnóstico. Su presencia se confirma mediante la angiografía por sustracción digital (ASD) y el manejo es médico y/o quirúrgico, siendo el último el que se asocia a un mejor pronóstico. Comunicamos el caso de un paciente peruano de ascendencia japonesa, sin factores de riesgo, con una hemorragia intracraneal cuyo diagnóstico final fue enfermedad de Moyamoya...
Moyamoya disease is characterized by progressive stenosis of the internal carotid artery and its main branches. The cause of the disease is unknown, ischemic or hemorrhagic stroke are the main manifestations (the former is more common) that disproportionately affect children and young adults, and is consider a diagnostic challenge. Its presence is confirmed by digital subtraction angiography (DSA) and the management may be medical or surgical, being the latter associated with a better prognosis. We report the case of a Peruvian male of Japanese ancestry without risk factors, with an intracranial hemorrhage who was finally diagnosed with Moyamoya disease...
