Trombosis senovenosa cerebral en un recién nacido con mutación MTHFR C677T tratado con enoxaparina / Cerebral sinovenous thrombosis in a newborn with mutation of MTHFR C677T treated with enoxaparin

Resumen: Introducción: La trombosis senovenosa cerebral neonatal (TSVC), es una patología rara y generalmente grave, de la cual se conoce poco sobre los mecanismos fisiopatológicos responsables y, aunque controvertido, se ha sugerido que la trombofilia genética, puede desempeñar un rol en la patogénesis. Debido a los temores de un sangrado intracraneal el tratamiento anticoagulante con heparina de bajo peso mole cular es controvertido. Objetivo: presentar un recién nacido con una trombosis senovenosa cerebral neonatal, discutir los factores de riesgo trombofílico, y el manejo con heparina de bajo peso molecu lar de la trombosis venosa cerebral. Caso Clínico: Recién nacido de término que debutó a los 8 días de vida con convulsiones clónicas, rechazo al pecho más hipoactividad motora. La neuroimagen con RM mostró una TSVC involucrando múltiples senos venosos, un infarto hemorrágico talámico dere cho y congestión venosa de la sustancia blanca frontal. El estudio de trombofilia puso de relieve una mutación homocigota del gen MTHFR C677T. El tratamiento con heparina de bajo peso molecular se asoció a repermeabilización del seno sagital superior a los 23 días de iniciada la terapia. Conclusio nes: La presentación clínica de la TSVC en el neonato es inespecífica, probablemente en relación con la extensión y gravedad de la lesión y el desarrollo de complicaciones asociadas, como infartos he morrágicos venosos intraparenquimatosos o hemorragia intraventricular. Estas complicaciones son detectables mediante Ecografia o Resonancia Magnética, y deben hacer sospechar una TSVC. En esta experiencia el tratamiento anticoagulante mostró ser seguro y prevenir la extensión de la trombosis.

Abstract: Introduction: Neonatal cerebral sinovenous thrombosis (CSNT) is a rare and generally serious con dition about which there is little knowledge of the responsible pathophysiological mechanisms and, although controversial, it has been suggested that genetic thrombophilia may play a role in its patho genesis. Out of concern for intracranial bleeding, the anticoagulant treatment with low-molecular- weight heparin is controversial. Objective: To present a case of a newborn with neonatal CSNT, to analyze the thrombophilic risk factors, and the management of cerebral venous thrombosis with low-molecular-weight heparin. Clinical Case: Full-term newborn who presented at eight days of life breastfeeding rejection, clonic seizures, and locomotor hypoactivity. The MRI neuroimaging showed a CSNT involving multiple venous sinuses, a right thalamic hemorrhagic infarction, and venous congestion in frontal white matter. Thrombophilia study highlighted a homozygous MTHFR C677T mutation. Treatment with low-molecular-weight heparin was associated with repermeabilization of the superior sagittal sinus after 23 days of starting therapy. Conclusions: The clinical presentation of CSNT in the neonate is nonspecific, probably related to the extent and severity of the injury and the development of associated complications, such as venous hemorrhagic infarctions and intraparenchymal or intraventricular hemorrhage. These complications are detected through ultrasound or MRI, and they should make us suspect a CSNT. In this experience, the anticoagulant treatment proved to be safe and prevents thrombus propagation.

Papiledema como primera manifestación de trombosis de senos venosos en un paciente pediátrico. Reporte de caso y revisión de literatura / Papilledema as an initial finding in venous sinus thrombosis in a child. Case report and literature review

Introducción: la trombosis de senos venosos (TSV) en niños, sin factores de riesgo es una patología rara pero potencialmente fatal. La cefalea es el síntoma principal y muy pocos pacientes se presentan sin este. El papiledema como hallazgo inicial se presenta sólo en el 9% de los pacientes. Presentamos el caso de una menor de 13 años, sin factores de riesgo para TSV, cuya manifestación inicial fue el papiledema en ausencia de cefalea. Objetivo: describir el caso clínico de una paciente menor de edad sin comorbilidades, factores de riesgo ni cefalea, con papiledema como única manifestación de TSV. Diseño de estudio: reporte de caso. Resumen del caso: menor de 13 años, sin antecedentes, con cuadro clínico de 12 horas de evolución de dolor en ojo derecho de instauración progresiva, tipo punzada, asociado a visión doble y un episodio emético. Al examen físico con agudeza visual (AV) 20/20 en ambos ojos, con papiledema; sin hallazgos positivos al examen neurológico ni en otros sistemas. Conclusión: La TSV en pacientes pediátricos sin factores de riesgo ni cefalea es rara. El papiledema es uno de los hallazgos principales de esta patología y en pocas ocasiones es la manifestación inicial. Es importante conocer los síntomas y signos oft almológicos de la TSV, ya que pueden ser el único hallazgo en esta patología.

Background: venous sinus thrombosis (VST) in children, without risk factors, is a rare but potentially fatal pathology. Headache is the main symptom and only few patients present without it. Papilledema as an initial finding occurs only in 9% of patients. We present the case of a 13 years old girl, without risk factors for VST, with papilledema as initial manifestation in the absence of headache. Objective: to describe a clinical case of with venous sinus thrombosis without comorbidities or risk factors, who had papilledema as first symptom. Study design: case report and literature review. Case summary: 13 years old girl, without medical history, with a clinical picture of 12 hours of right eye progressive pain, prick type, associated with double vision and an emetic episode. Physical examination with visual acuity (VA) 20/20 in both eyes and papilledema; without positive findings in the neurological examination or in other systems. Conclusion: venous sinus thrombosis in pediatric patients without risk factors or headache is rare. Papilledema is one of the main fi ndings of this pathology and in few cases it is the initial manifestation. It is important to know the symptoms and ophthalmological signs of VST because it could be the only finding in this pathology.

Trombosis de Senos Venosos, experiencia en un hospital pediátrico en Buenos Aires, Argentina / Venous sinus thrombosis: Experience at a pediatric hospital in Buenos Aires, Argentina

Introducción: La trombosis de senos venosos cerebrales (TSVC) es infrecuente en pediatría. Está asociada a condiciones como infecciones, deshidratación, fallo renal, traumatismo de cráneo, neoplasias, trastornos hematológicos, etc. Cefalea, vómitos, alteración del sensorio y hemiparesia son los síntomas más frecuentes. El diagnóstico es confirmado por TC con angio y/o RM con angio. La anticoagulación es el tratamiento de elección. Los pacientes suelen evolucionar favorablemente. Materiales y Métodos: Estudio descriptivo observacional de pacientes con TSVC atendidos en el Hospital Garrahan desde 2010 a 2017. Las variables registradas fueron: edad, sexo; manifestaciones clínicas, factores de riesgo; estudios diagnósticos, tratamiento y evolución. Resultados: Se describen 34 pacientes con TSVC. Los adolescentes fueron el grupo mayor. La cefalea fue el síntoma más frecuente. Angio TC, RM y/o angio RM confirmaron el diagnóstico; los senos transverso, sagital superior y sigmoideo fueron los más comprometidos. 21 pacientes tenían patología oncológica y 14 procesos infecciosos. El tratamiento de elección fue la anticoagulación. Tuvieron buena evolución el 82%. Conclusiones: Debemos sospechar esta entidad en dos grupos: el primero formado por lactantes y pre-escolares con patología infecciosa; y un segundo integrado por escolares y adolescentes con patología oncológica, especialmente aquellos que reciben L-ASA.Es importante resaltar el valor de la TC y angio TC para hacer diagnóstico oportuno, resultando accesible las 24 horas en el hospital

Introduction: Cerebral venous sinus thrombosis (CVST) is uncommon in children. CVST is associated with conditions, such as infections, dehydration, renal failure, head trauma, cancer, and hematological disorders. Headache, vomiting, sensory alterations, and hemiparesis are the most common symptoms. Diagnosis is confirmed by angio CT and/or MRA. Anticoagulation is the treatment of choice. Outcome is generally good. Material and Methods: An observational, descriptive study of patients with CVST seen at Garrahan Hospital between 2010 and 2017. The following variables were recorded: age, sex; clinical manifestations, risk factors; diagnostic studies, treatment, and outcome. Results: 34 patients with CVST were studied. Most patients were adolescents. Headache was the most common symptom. Angio CT, MRI, and/or MRA confirmed the diagnosis; the transverse, superior sagittal, and sigmoid sinuses were most frequently affected. Of the patients, 21 had oncological disease and 14 infections. Anticoagulation was the treatment of choice. Outcome was good in 82%. Conclusions: CVST should be suspected in the following two groups: A first group consisting of infants and preschool children with infections and a second group of school-age children and adolescents with cancer, especially those receiving L-ASA. It is important to highlight the role of CT and angio CT for early diagnosis as the study is available day and night at the hospital.

Dural sinus thrombosis following epidural analgesia for delivery: a clinical case / Trombose de seios durais após analgesia peridural para parto: caso clínico

Abstract Background and objectives: Neurological complications of spinal anesthesia are rare conditions. Headache caused by low pressure of the cerebrospinal fluid is one of the most frequent, which occurs after post-dural puncture. A comprehensive history and physical exam must be carried out before making the diagnosis of Post-Dural Puncture Headache (PDPH) and additional tests are necessary to exclude the possibility of developing serious neurological complications such as Dural Sinus Thrombosis (DST). According to the Case Report a differential diagnosis between Dural Sinus Thrombosis with PDPH is discussed. Case report: A 22 year-old lady, ASA Physical Status Class I was admitted at 39 weeks of gestation for delivery. For labor pain relief she requested epidural for analgesia, but unfortunately accidental dural puncture occurred. She developed an occipital headache and neck pain in the second day postpartum which was relieved by both lying down and supporting treatment such as rehydration, analgesics and caffeine. On day third postpartum she was discharged without complaints. On day fifth postpartum the pain returned and became more intense and less responsive to oral analgesics. She was admitted to the hospital to do a complete neurological and image investigation that showed a lesion consistent with the diagnosis of cortical vein thrombosis and Dural Sinus Thrombosis (DST). She was treated with oral anticoagulants. After two days, a repeated magnetic resonance image (MRI) showed partial canalization of the central sinus thrombus. The patient was discharged from hospital five days after her admission without any of the initial symptoms. Conclusion: The report describes a patient who developed severe headache following continuous epidural analgesia for delivery. Initially it was diagnosed as PDPH, however with the aid of MRI the diagnosis of DST was later established and treated. DST is a rare condition and is often underdiagnosed. Because of its potentially lethal complications, it should always be considered in acute headache differential diagnosis.

Resumo Justificativa e objetivos: As complicações neurológicas da raquianestesia são condições raras. A cefaleia causada pela baixa pressão do fluido cerebrospinal é uma das mais frequentes e ocorre após a punção dural. Anamnese completa e exame físico geral devem ser feitos antes de fazer o diagnóstico de cefaleia pós-punção dural (CPPD) e testes adicionais são necessários para excluir a possibilidade de complicações neurológicas graves, como trombose de seios durais (TSD). De acordo com o relato do caso, discutiremos o diagnóstico diferencial entre TSD e CPPD. Relato de caso: Paciente de 22 anos, estado físico ASA I, foi admitida com 39 semanas de gestação para o parto. Para alívio da dor do trabalho de parto, a paciente solicitou analgesia peridural, mas infelizmente ocorreu uma punção dural acidental. A paciente desenvolveu cefaleia occipital e dor cervical no segundo dia pós-parto - ambas aliviadas com repouso e terapia de suporte, como reidratação, analgésicos e cafeína. No terceiro dia pós-parto, a paciente recebeu alta sem queixas. No quinto dia pós-parto, a dor retornou e ficou mais intensa e com pouca resposta aos analgésicos orais. Ela foi admitida no hospital para uma completa investigação neurológica e de imagem que mostrou uma lesão compatível com o diagnóstico de trombose venosa cortical e TSD. A paciente foi tratada com anticoagulantes orais. Após dois dias, a repetição de ressonância nuclear magnética (RM) mostrou canalização parcial de trombo do seio central. A paciente recebeu alta hospitalar cinco dias após a admissão, sem quaisquer dos sintomas iniciais. Conclusão: O caso descreve uma paciente que desenvolveu cefaleia grave após epidural contínua para o parto. Inicialmente ela foi diagnosticada como CPPD, contudo com o auxílio da RNM foi estabelecido o diagnóstico tardio de TSD. TSD é uma condição rara e frequentemente subdiagnosticada. Ela deve sempre ser considerada como diagnóstico diferencial de cefaleia aguda em decorrência de suas complicações potencialmente letais.

Neurobrucellosis and venous sinus thrombosis: an uncommon association

Abstract: Brucellosis is a commonly diagnosed zoonosis and neurological involvement is rare. A 30-year-old woman presented with a pulsatile headache that was exacerbated by the Valsalva maneuver and refractory to analgesic therapy. The patient also had nausea, cough, and coryza that evolved over 7 days. The neurological examination was unremarkable. Thrombosis of the lateral and sigmoid sinus and ipsilateral internal jugular vein were diagnosed and anticoagulation therapy was started. Brucella spp was identified in a sample of cerebrospinal fluid (CSF); five months after treatment with rifampicin and doxycycline, CSF was sterile. Cerebral venous thrombosis is a very uncommon sign of brucellosis.

Trombosis de senos venosos cerebrales: epidemiología, características clínicas, imaginología y pronóstico / Cerebral venous sinus thrombosis: Epidemiology, clinical characteristics, imaging and prognosis

Introducción. La trombosis de senos venosos cerebrales representa entre 0,5 y 1 % de las enfermedades cerebrovasculares en adultos, y sus factores de riesgo son diferentes a los del resto de dichas enfermedades. Objetivo. Determinar la epidemiología, las características clínicas e imaginológicas, así como los resultados en pacientes con trombosis de senos venosos cerebrales, y explorar los aspectos asociados con los puntajes desfavorables en la escala modificada de Rankin. Materiales y métodos. Se llevó a cabo un estudio retrospectivo de corte transversal en el Instituto Neurológico de Colombia entre marzo de 2006 y junio de 2011, periodo en el que se analizaron las historias clínicas de 37 pacientes con diagnóstico confirmado por neuroimágenes. Resultados. El 86,5 % de los pacientes eran mujeres, con una edad promedio de 41 años. El síntoma más frecuente fue la cefalea (86,5 %), el cual se presentó como único síntoma en el 40,5 % de los casos. El 68 % tenía, por lo menos, un antecedente de riesgo para trombosis de senos venosos cerebrales, siendo el más frecuente la obesidad (24,3 %), seguido del uso de anticonceptivos hormonales (21,9 %). El examen neurológico fue normal en el 43,2 %. El hallazgo más frecuente en la tomografía fue la hiperdensidad de los senos venosos (33 %) y, en la resonancia magnética, el infarto venoso (37,5 %). El promedio de senos comprometidos fue de 2,27+1,3, siendo más frecuente el compromiso de los transversos. La estancia hospitalaria promedio fue de 7,8+3,6 días. El 92 % de los pacientes presentaba resultados funcionales favorables al dárseles de alta. La mortalidad hospitalaria fue de 5,4 % y se relacionó directamente con la trombosis de senos venosos cerebrales. Conclusiones. La trombosis de senos venosos cerebrales difiere de los otros tipos de enfermedad cerebrovascular en cuanto a su perfil epidemiológico, factores de riesgo, presentación clínica y pronóstico. Es una condición que exige un alto grado de sospecha diagnóstica, pues su presentación clínica es inespecífica.

Introduction: Cerebral venous sinus thrombosis represents 0.5 - 1% of all cerebrovascular diseases. Objective: The aim of this study was to determine the epidemiological, clinical, and imaging features of the disease, as well as the outcomes of patients with cerebral venous sinus thrombosis, and to explore the characteristics associated with unfavorable patient outcomes. Materials and methods: In this cross-sectional, retrospective study, the medical records of 37 patients with cerebral venous sinus thrombosis were analyzed. Results: Eighty-six percent of the patients were women, and the mean patient age was 41 years. The most frequently reported symptom was headache (86.5%); headache was the single presenting symptom in 40.5% of the patients. Sixty-eight percent of the patients had at least one risk factor, the most frequent of which was obesity (24.3%). A total of 43.2% of the patients had no focal neurological findings. The most common finding on computerized tomography (CT) was hyperdense venous sinuses; on Magnetic Resonance Imaging (MRI), the most common finding was venous infarction. On average, 2.27±1.3 sinuses were involved; most frequently, the transverse venous sinuses were affected. The average hospital stay was 7.8±3.6 days. At hospital discharge, the outcomes were favorable in 92% of the patients, and the mortality rate was 5.4%. Conclusions: Cerebral venous sinus thrombosis is a different type of cerebrovascular disorder, with distinct epidemiology, risk factors, clinical presentations and functional outcomes. The diagnosis is based on clinical suspicion because of the unspecific clinical presentation of the disease.

Trombose do seio dural em idade pediátrica / Dural sinus thrombosis in pediatric age

A trombose do seio dural é uma situação clínica rara, que resulta normalmente da complicação de processos infecciosos dos seios perinasais. Os sintomas e sinais são extremamente variados e inespecíficos sendo o diagnóstico feito através da ressonância magnética nuclear. Esse trabalho relata a ocorrência de trombose do seio dural em um paciente com idade pediátrica. Paciente com 10 anos de idade, sexo masculino, foi enviado ao serviço de urgência devido à diplopia e endotropia no olho esquerdo. No exame oftalmológico foi detectado papiledema bilateral, diplopia binocular e endotropia do olho esquerdo. Apresentava acuidade visual de 10/10 bilateralmente. Diante da suspeita de lesão ocupando espaço do sistema nervoso central, foi realizada ressonância magnética nuclear que confirmou o diagnóstico de TSD. Para avaliar a pressão intracraniana foi efetuada uma punção lombar com manometria, e esta demonstrou uma pressão intracraniana de 20mmHg (normal: <15mmHg). Perante isto a criança ficou internada para tratamento médico (enoxaparina de baixo peso molecular 1,5 mg/kg/dia subcutâneo (60 mg/dia), prednisolona 35 mg/dia oral e acetazolamida 250 mg/dia oral) durante 10 dias. Após 1 mês de follow-up verificou-se agravamento oftalmológico. A realização de nova punção lombar apresentou uma pressão intracraniana de 40 mmHg que não cedia ao tratamento médico. Após discussão multidisciplinar do caso optou-se pela realização de derivação lombo-peritoneal. A necessidade de uma grande dose de suspeição clínica, tanto para o diagnóstico inicial quanto para a monitorização das complicações, tornam a abordagem da trombose do seio dural um processo singular.

Dural sinus thrombosis is a rare condition, usually results from a late complication of an infection of the paranasal sinuses. The signs and symptoms are extremely varied and nonspecific, being the diagnosis made through magnetic resonance imaging. Ten-year-old male patient that was sent to our emergency department with left endotropia and diplopia. Ophthalmic examination was performed and showed papilledema with margin blurred right and left eye, binocular diplopia and left eye endotropia. Visual acuity was 10/10 bilaterally. Given the suspected space occupying lesion of the central nervous system, the MRI was performed and confirmed the diagnosis of DST. For evaluating the intracranial pressure (IP), a lombar puncture (LP) with manometry was carried out and revealed IP of 20 mmHg (normal values: <15mmHg). Towards this, the child’s was admitted for medical treatment (low molecular weight enoxaparin subcutaneous 1,5 mg/kg/day (60 mg/day), prednisolone 35 mg/per day and acetazolamide 250 mg/per day) over 10 days. After 1 month of follow-up there was deterioration of the ophthalmologic condition. A new LP was made and showed IP of 40 mmHg resilient to medical treatment. After multidisciplinary discussion of the case, it was decided for conducting lumboperitoneal shunt. The need for a great deal of suspicion for both the initial diagnosis and for monitoring complications make DST approach a special process.

Trombosis de senos venosos intracraneales: una complicación infrecuente y grave de la colitis ulcerosa. Primera comunicación de un caso en Uruguay / Intracranial sinus venous thrombosis in ulcerative colitis: Report of one case

Intracranial sinus venous thrombosis (ICSVT) is a rare complication of ulcerative colitis that affects from 1.7 to 7.5% of patients. We report a 22 year-old male with ulcerative colitis in treatment with mesalazine and prednisone presenting with headache and speech disturbances. A magnetic resonance imaging of the brain showed a left temporal hemorrhagic infarct with thrombosis of the ispilateral superficial vein and sigmoid venous sinus. No cause of thrombophilia was detected. Anticoagulation with heparin was started which was changed to oral anticoagulation with warfarin. The patient was discharged ten days after admission.

Trombose de seios venosos em criança com síndrome nefrótica: relato de caso e revisão da literatura / Venous sinus thrombosis in a child with nephrotic syndrome: a case report and literature review

A síndrome nefrótica associa-se a um estado de hipercoagulabilidade, apresentando risco aumentado de complicações tromboembólicas. A trombose dos seios venosos cerebrais é uma complicação rara da síndrome nefrótica, com poucos casos descritos na literatura, mas com diagnósticos cada vez mais frequentes. A verdadeira incidência pode estar subestimada, uma vez que muitos eventos são assintomáticos ou não são diagnosticados a tempo. Descrevemos aqui o caso de uma criança do sexo masculino, de 2 anos e 10 meses, com síndrome nefrótica, que apresentou, na evolução, cefaleia, crises epilépticas e rebaixamento sensorial, com o diagnóstico de trombose do seio sagital superior e transverso. Foi realizada revisão da literatura internacional por meio de estratégia de busca definida, nas bases de dados PubMed, SciELO e Lilacs, utilizando os termos “nephrotic syndrome” e “cerebral sinovenous thrombosis”. O diagnóstico de trombose venosa deve ser considerado em qualquer paciente com síndrome nefrótica que manifeste sinais e sintomas neurológicos, destacando que a suspeita clínica precoce tem relação com um desfecho favorável.

Nephrotic syndrome is associated with a hypercoagulable state and an increased risk of thromboembolic complications. Cerebral venous sinus thrombosis is a rare complication of nephrotic syndrome, with few cases described in the literature, although the disease may be under-diagnosis. The true incidence of cerebral venous sinus thrombosis may be underestimated because many events are asymptomatic or are not diagnosed in time. Here, we describe the case of a male child, 2 years and 10 months old, with nephrotic syndrome presenting with headache, epileptic seizures and sensory inhibition who was diagnosed with superior sagittal and transverse sinuses thrombosis. An international literature review was performed with a defined search strategy in the PubMed, SciELO and Lilacs databases using the terms ‘nephrotic syndrome’ and ‘cerebral sinovenous thrombosis’. The diagnosis of venous thrombosis should be considered in any patient with nephrotic syndrome who presents with neurological signs and symptoms, as early clinical diagnosis promotes favorable outcomes.

Trombosis de los senos intracraneales: Reporte de tres casos y revision de la literatura / Intracranial sinuses thrombosis: Report of three cases and literature review

La trombosis venosa intracraneal es un tipo de accidente cerebrovascular poco frecuente cuyo diagnóstico muchas veces ha sido demorado debido a sus diversas causas y presentaciones. Sin embargo, hoy en día su diagnóstico está aumentando debido al mayor empleo de la resonancia magnética nuclear, la angioresonancia y a la mejor comprensión del cuadro clínico. Se presentan los casos de tres mujeres con cefalea y déficit focal de evolución subaguda. Los estudios de neuroimágenes fueron compatibles con trombosis venosa intracraneal. Las pacientes tuvieron diferentes factores de riesgo, fueron tratadas con heparina y presentaron buena respuesta al tratamiento...

Intracranial sinuses thrombosis is an infrequent cerebrovascular accident whose diagnosis is usually delayed due to diverse causes and varying clinical presentations. However, with the advent of magnetic resonance imaging, angioresonance and with better knowledge of the clinical picture its diagnosis has improved nowadays. We present here three women cases with headache and focal deficits of sub-acute onset. Neuroimaging studies were compatible with intracranial sinuses thrombosis. Patients had different risk factors; they were treated with heparin and responded well to treatment...

Trombosis de seno venoso secundaria a traumatismo encéfalo craneano: caso clínico / Cerebral vein thrombosis secondary to closed head injury: Report of one case

Intracranial sinus thrombosis (1ST) after closed head injury is an uncommon but potentially serious complication. It has no correlation with the severity of the injury. The symptoms and clinical course are highly variable. The most frequent but least specific symptom is severe headache. Cerebral lesions and neurologic signs develop in half of patients with IST. We report a 29 year-old male who had an IST after a severe closed head injury. The patient initially developed headache and had later 2 secondarily generalized seizures. The magnetic resonance imaging showed a superior sagittal sinus thrombosis. Anticoagulation with unfractionated heparin and intravenous phenytoin was started. At the moment of this report he is asymptomatic and continues with oral anticoagulants and phenytoin.

Accidente vascular encefálico. Presentación clínica y factores de riesgo / Clinical presentation and risk factors of cerebrovascular accident

Introducción: La alteración focal aguda de la función cerebral en el adulto permite diagnosticar un accidente vascular encefálico (AVE) e iniciar precozmente el rescate de tejido cerebral. En los niños, las diferencias clínicas de esta patología en relación al adulto generan atraso, subdiagnóstico e impiden el tratamiento en la etapa aguda. Objetivo: Describir la presentación clínica y factores de riesgo del AVE isquémico en 156 niños, comparar la presentación clínica según edad y tipo de AVE. Pacientes y Método: Se analiza un registro de AVE isquémico, confirmado con imágenes cerebrales, en población menor de 18 años, iniciado en Enero 2003. Resultados: La mediana de edad: 4,9 meses (33 por ciento recién nacidos, 46 por ciento menores 6 años y 21 por ciento mayor de 6 años), 64 por ciento fueron hombres. Los infartos arteriales correspondieron al 85 por ciento y el resto fueron trombosis de senos venosos. Predominaron los signos difusos (67 por ciento) en menores de 6 años (p = 0,001). Las convulsiones fueron observadas en el 61 por ciento de los niños menores de 6 años (p = 0,03), el 33 por ciento presentó signos focales. Los factores de riesgo más frecuentes fueron patologías sistémicas agudas y cardiopatías. En 14,7 por ciento no se encontraron factores de riesgo. Conclusiones: La búsqueda de signos focales agudos de AVE no tiene utilidad diagnóstica en el niño, a diferencia de los adultos, pues su ausencia no excluye patología vascular isquémica focal. La patología sistémica aguda fue el factor de riesgo más frecuente para AVE.

Introduction: Acute focal brain dysfunction in adults serves to diagnose and to provide early rescue of the brain tissue. In children, the clinical differences of this condition compared to adults results in delayed diagnosis and treatment. Objective: To describe the clinical presentation and risk factors of CVA in 156 children and related to age and type of CVA. Patients and Method: A CVA registry of newborn to 18 years old was started in January 2003 were analized. Diagnosis was confirmed by brain imaging. Results: The median of age was 4.9 month (33 percent was newborn, 46 percent < 6 years old, 21 percent > 6 years old); 64 percent were males. Arterial ischemic stroke represented 85 percent, the rest were cerebral sinovenous thrombosis. In the clinical manifestations the diffuse signs were more frequently seen (67 percent) in children < 6 years of age (p 0.001). Seizures were observed in 61 percent in children < 6 years old (p = 0.03), 33 percent presented focal signs. The most common risk factors were acute systemic diseases and heart disease. 14.7 percent of the patients did not present risk factors. Conclusions: Acute focus signals of CVA in children have no diagnostic value, unlike adults; however absence doesn't exclude focal ischemic vascular disease. The acute systemic disease was the most common risk factor for stroke.

Cerebral venous thrombosis and cerebral infarction: a rare disease with a rare cause

We present a case of cerebral venous thrombosis and cerebral infarction associated with diabetic ketoacidosis. The patient presented with vomiting, headache, lethargy and altered sensorium. CT venography provided the final diagnosis; therefore, anticoagulant was used, which resulted in the improvement of neurological outcome. Neurological deterioration during an episode of diabetic ketoacidosis is usually assumed to be caused by cerebral edema. Neuroimaging should always be performed in suspected cerebral edema associated with diabetic ketoacidosis in order to exclude other pathologies.

Trombosis venosa cerebral / Cerebral venous thrombosis

La trombosis venosa cerebral (TvC) es una rara enfermedad que se incluye dentro del grupo de trombosis en sitios infrecuentes. El correcto estudio y manejo terapéutico de la misma es de capital importancia por la alta morbimortalidad que conlleva de no diagnosticarse y tratarse correctamente. El desarrollo en el conocimiento de los factores de riesgo como las trombofilias entre otros, ha sido de gran importancia diagnóstica. Aún faltan estudios randomizados y controlados de gran número de pacientes para definir la duración óptima del tratamiento según cada caso particular. Por el momento las recomendaciones terapéuticas en este tipo de trombosis se basan en estudios observacionales y opinión de expertos, los cuales recomiendan anticoagulación a largo plazo para pacientes con trombosis inexplicadas, trombosis recurrentes, o asociadas a trombofilias de alto riesgo o combinadas. El objetivo de esta publicación es presentar una serie de casos clínicos, revisar el tema de TvC dado la importancia por las posibles complicaciones evolutivas; conocer los factores de riesgo y discutir las posibilidades terapéuticas.

Clinical profile of cerebral venous sinus thrombosis and the role of imaging in its diagnosis in patients with presumed idiopathic intracranial hypertension.

Retrospective descriptive study reporting the rate of occurrence of cerebral venous sinus thrombosis (CVST), highlighting the role of magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) in patients with presumed idiopathic intracranial hypertension (IIH). Study was conducted in the department of neuro-ophthalmology at a tertiary eye care center in South India. Data from 331 patients diagnosed with IIH from June 2005 to September 2007 was included. Inclusion criteria were: Elevated opening cerebrospinal fluid (CSF) pressure of more than 200 mm of water on lumbar puncture, normal CSF biochemistry and microbiology, and normal neuroimaging as depicted by computed tomography(CT) scan. Exclusion criteria were: Space-occupying lesions, hydrocephalus, meningitis, intracranial pressure within normal range, abnormal CSF biochemistry and microbiology. The remaining patients were evaluated with MRI and MRV. CVST was present in 11.4% of patients who were presumed to have IIH (35/308). MRI alone identified 24 cases (68%) of CVST, while MRI used in combination with MRV revealed an additional 11 cases (32%). Risk factors associated with CVST were identified in nine out of 35 patients (26%). CVST may be misdiagnosed as IIH if prompt neuroimaging by MRI and MRV is not undertaken. Risk factors of CVST may not be apparent in all the cases and these patients are liable to be missed if CT scan alone is used for neuroimaging, hence MRI, combined with MRV should be undertaken to rule out CVST.

Trombosis venosa intracraneal: signos imaginológicos y errores frecuentes / Intracranial venous thrombosis: imaging signs and common errors

Although intracranial venous thrombosis is a relatively rare disease, it constitutes an entity that must be timely and accurately diagnosed in emergency services, given the need for prompt treatment to avoid serious complications, including neurological deficits or even death. There are several imaging signs provided by both CT and magnetic resonance imaging scans that allow this early diagnosis. On the other hand, some differential diagnoses need to be performed to prevent the error of mistaking intracranial venous thrombosis for any other entity.

Si bien la trombosis venosa intracraneal es una patología relativamente poco frecuente, es una entidad que debe ser diagnosticada a tiempo y con certeza en los servicios de urgencia, dada la necesidad de pronto tratamiento para evitar complicaciones graves que incluyen déficit neurológicos e incluso muerte. Existen varios signos imaginológicos que nos permiten hacer este diagnóstico, tanto en tomografía computarizada como en resonancia magnética. Por otra parte, es necesario considerar algunos diagnósticos diferenciales para no cometer errores, confundiendo esta patología con otras entidades.

Malignant cutaneous melanoma associated with cerebral venous sinus thrombosis

Cerebral venous sinus thrombosis [CVST] is an infrequent disease [representing around 1% of all strokes] manifested by clotting of blood in cerebral venous or dural sinuses as well as cortical veins. Herein, we describe an unusual case of malignant melanoma presenting with CVST in whom the diagnosis of CVST was suspected on the basis of clinical findings along with brain CT scan, and finally was confirmed by brain MRI and magnetic resonance venography. Intravenous heparin treatment was started immediately; but unfortunately the patient died one week after hospitalization. We suggest that the hypercoagulable state provoked by malignant melanoma generated the CVST as brain MRI did not indicate any evidence of brain metastasis; therefore, direct invasion of the sinus by the tumor is less likely; on the other hand, we should not omit the possible role of microscopic sinus metastasis

[Cerebral venous sinus thrombosis following sildenafil administration; a case report]

Sildenafil is a selective inhibitor of phosphodiesterase type 5 [PDE5] which currently used for the treatment of erection dysfunction [ED]. Medicine approved by the FDA for treatment of pulmonary hypertension. Although there is no evidence of hypercoagulation states being associated with the use of PDE5 inhibitors, several animal studies suggest that PDE5 inhibitors may cause arterial and venous thrombotic or embolic events. The patient was a 37 year old man who had suffered sever headache and tonic- clonic generalized seizure. In neurological examination, he had decreased level of consciousness and he had no other positive finding in examination. The patient has been constantly taken 100 mg of Sildenafil once a day without medical supervision over the last month. Brain computerized tomography [CT] showed a small hemorrhage in left frontal lobe with suspicious to cerebral venous sinus thrombosis [CVST] lumbar puncture was performed. Increased cerebrospinal fluid pressure suggested the probability of CVST. Brain MRI result confirmed superior sagittal sinus thrombosis. Cerebral venous sinus thromboses need to be considered a potential side effect of Sildenafil administration