Compromiso cerebrovascular agudo en síndrome hemolítico urémico: descripción de dos casos pediátricos / Acute cerebrovascular events associated to hemolytic uremic syndrome: description of two pediatric cases

El Síndrome Hemolítico Urémico asociado a diarrea (SHU+D) es un desorden multisistémico en el cual el compromiso neurológico se asocia a empeoramiento del pronóstico. Una causa importante de daño neurológico permanente es el compromiso cerebrovascular. OBJETIVO: Reportar dos casos pediátricos de SHU+D con compromiso neurológico severo debido a patología cerebrovascular y revisar la literatura disponible. CASOS CLÍNICOS: Dos niños de 15 y 21 meses, previamente sanos, debutaron con convulsiones y compromiso de conciencia dentro de la primera semana de un SHU+D. Ambos presentaron hipertensión, falla renal aguda severa y déficit motor focal. Un niño mejoró significativamente su estado neurológico después de cinco sesiones de plasmaféresis. La resonancia magnética encefálica, mostró en el primer niño infartos bilaterales múltiples de vasos pequeños y lesiones de sustancia blanca. En el segundo paciente se identificaron extensos infartos bilaterales en territorios de ambas arterias cerebrales medias. Al año del evento agudo, ambos niños con déficit funcional marcado; el primer paciente evolucionó con retraso del desarrollo del lenguaje y hemiparesia espástica; el segundo persistió con cuadriparesia espástica, epilepsia con mal control de crisis y marcado deterioro funcional. CONCLUSIÓN: Aunque la mayoría de los niños con SHU+D y compromiso cerebral no presentan secuelas a largo plazo, la patología cerebrovascular en el período agudo puede causar daño permanente, por lo que, además del manejo de las alteraciones hidroelectrolíticas, hipertensión y falla renal, las terapias dirigidas a mecanismos fisiopatológicos específicos desencadenantes del compromiso vascular podrían mejorar el pronóstico.

Diarrhea-associated Hemolytic Uremic Syndrome (D+HUS) is a multisystem disorder in which neurological involvement (35 to 50%) is associated to adverse outcome. An important cause of a permanent neurological impairment is the cerebrovascular pathology. OBJECTIVE: To report two pediatric cases of D+HUS with severe neurological involvement due to cerebrovascular disease, and review available literature. CLINICAL CASES: Two previously healthy 15- and 21-month-old children debuted with seizures and impairment of consciousness within the first week of a D+HUS. Both presented hypertension, severe acute renal failure, and focal motor deficit. One child showed significant improvement in neurologic status after five sessions of plasmapheresis. Brain magnetic resonance showed in the first child multiple bilateral infarcts of small vessels and lesions of white matter. In the second patient, large bilateral infarcts on both middle cerebral arteries territories were identified. One year after the acute event, both children showed functional impairment; The first patient evolved with language delay and spastic hemiparesis; the second patient with spastic quadriparesis, epilepsy with poor seizure control and marked functional impairment. CONCLUSION: Although most of the children with D+HUS and brain involvement do not have long-term sequelae, cerebrovascular disease in the acute period causes permanent damage, and in addition to the management of electrolyte disturbances, hypertension, and renal failure, therapies directed at specific pathophysiological mechanisms that trigger vascular compromise may improve prognosis.

Multidetector computed tomography angiography in clinically suspected hyperacute ischemic stroke in the anterior circulation: an etiological workup in a cohort of Brazilian patients / Angiotomografia computadorizada multidetectores na avaliação da suspeita clínica de acidente vascular encefálico isquêmico hiperagudo da circulação anterior: determinação etiológica precoce em um grupo de pacientes brasileiros

Objective The potential of computed tomography angiography (CTA) was assessed for early determination of stroke subtypes in a Brazilian cohort of patients with stroke. Method From July 2011 to July 2013, we selected patients with suspected hyperacute stroke (< 6 hours). Intracranial and cervical arteries were scrutinized on CTA and their imaging features were correlated with concurrent subtype of stroke. Results Stroke was documented in 50/106 selected patients (47.2%) based on both clinical grounds and imaging follow-up (stroke group), with statistically significant arterial stenosis and vulnerable plaques on CTA. Intracranial large artery disease was demonstrated in 34% of patients in the stroke group. Partial territorial infarct prevailed (86%) while artery-to-artery embolization was the most common stroke mechanism (52%). Conclusion Multidetector CTA was useful for the etiologic work-up of hyperacute ischemic stroke and facilitated the knowledge about the topographic pattern of brain infarct in accordance with its causative mechanism. .

Objetivo Avaliar o potencial da angiotomografia computadorizada multidetectores (ATCM) na determinação etiológica precoce do acidente vascular encefálico (AVE) e correlacionar o mecanismo causal com o padrão de infarto. Método De Julho de 2011 a Julho de 2013, foram selecionados os pacientes com suspeita clínica de AVE hiperagudo. Os achados da ATCM dos vasos intracranianos e cervicais foram correlacionados com a etiologia final do evento. Resultados AVE foi confirmado em 50/106 pacientes (47,2%). Estes apresentaram alterações angiográficas estatisticamente mais relevantes. Aterosclerose dos grandes vasos intracranianos esteve presente em 34% destes pacientes. O padrão radiológico topográfico de infarto mais comum foi o infarto territorial parcial (86%). A embolização arterio-arterial foi o mecanismo mais prevalente (52%). Conclusão A utilização da ATCM traz benefícios na detecção etiológica precoce dos pacientes com suspeita de AVE hiperagudo, além de possibilitar o entendimento do padrão radiológico topográfico de acordo com o mecanismo causal do evento isquêmico. .

The 'silence' of silent brain infarctions may be related to chronic ischemic preconditioning and nonstrategic locations rather than to a small infarction size

OBJECTIVE: Silent brain infarctions are the silent cerebrovascular events that are distinguished from symptomatic lacunar infarctions by their 'silence'; the origin of these infarctions is still unclear. This study analyzed the characteristics of silent and symptomatic lacunar infarctions and sought to explore the mechanism of this 'silence'. METHODS: In total, 156 patients with only silent brain infarctions, 90 with only symptomatic lacunar infarctions, 160 with both silent and symptomatic lacunar infarctions, and 115 without any infarctions were recruited. Vascular risk factors, leukoaraiosis, and vascular assessment results were compared. The National Institutes of Health Stroke Scale scores were compared between patients with only symptomatic lacunar infarctions and patients with two types of infarctions. The locations of all of the infarctions were evaluated. The evolution of the two types of infarctions was retrospectively studied by comparing the infarcts on the magnetic resonance images of 63 patients obtained at different times. RESULTS: The main risk factors for silent brain infarctions were hypertension, age, and advanced leukoaraiosis; the main factors for symptomatic lacunar infarctions were hypertension, atrial fibrillation, and atherosclerosis of relevant arteries. The neurological deficits of patients with only symptomatic lacunar infarctions were more severe than those of patients with both types of infarctions. More silent brain infarctions were located in the corona radiata and basal ganglia; these locations were different from those of the symptomatic lacunar infarctions. The initial sizes of the symptomatic lacunar infarctions were larger than the silent brain infarctions, whereas the final sizes were almost equal between the two groups. CONCLUSIONS: Chronic ischemic preconditioning and nonstrategic locations may be the main reasons for the 'silence' of silent brain infarctions.

Infarto agudo talámico bilateral en paciente joven con foramen oval permeable / Acute bilateral thalamic infarcts in a young man with patent foramen ovale

Antecedentes: La presencia de foramen oval permeable se asocia a infartos cerebrales criptogénicos en pacientes jóvenes. Caso clínico: Hombre de 27 años en quien se inició de forma súbita síndrome confusional agudo, bradipsiquia y somnolencia. La imagen de resonancia magnética cerebral mostró infarto talámico bilateral simétrico por probable oclusión de arteria de Percheron tipo 2b. El ecocardiograma identificó foramen oval permeable. Conclusiones: El evento cerebrovascular es causa frecuente de discapacidad e incluso muerte en población joven. Debe hacerse énfasis en el estudio integral de esta patología.

BACKGROUND: Patent foramen ovale (PFO) has been associated with cryptogenic stroke in young patients. CLINICAL CASE: A 27-year-old man presented with acute confusional syndrome, altered language, bradypsychia and somnolence. Brain MRI showed symmetrical bilateral thalamic infarctions probably due to occlusion of Percheron's artery type 2b. Echocardiography showed patent foramen ovale. CONCLUSIONS: Cerebrovascular disease is a frequent cause of disability and even death in young patients, and thus its medical approach should be emphasized.

Case report Brain infarction in a child with severe diabetic ketocidosis

The most common cause for neurological deterioration in patients with DKA is cerebral edema. In a large retrospective study, estimated risk of cerebral edema was 6.8 per 1000 cases of DKA and was associated with a mortality rate of 24%. In addition to cerebral edema, other causes of neurological deterioration was the result of thrombosis and infection or hemorrhage

[Arterial involvement in Behcet's disease: a series of 7 cases]

Arterial involvement is rarely described in Behcet disease BD and it is associated with poor prognosis. We report our experience with a rare and interesting subset of Behcet disease patients with arterial involvement: thrombosis and aneurysm formation types of angio-BD. From 1994 to 2008, seven cases of arterial BD with 3 aneurysm formation and 4 thrombosis were found amongst BD patients in the department of internal medicine of Habib Thameur Hospital. Combination of venous and arterial manifestations occurred in 4 patients. All patients were male and the mean age when arterial involvement manifested was diagnosed was about 39.3. The artery most often affected is the aorta followed by the pulmonary arteries. Brain infraction occurred in about 4 cases. Pericarditis and neurological symptoms were more frequently noted in patients with arterial BD than others without vasculo-Behcet disease. All patients underwent corticosteroids and immunosuppressive therapy. Two patients were successfully operated for aneurysms. All patients were alive after a mean of 7.8 years fo11owup, Arterial involvement is more frequently noted in vasculo-Behcet than venous thrombosis and it's associated with a high morbidity and mortality rate

Curso de Atualização em Emergências Médicas: aula 7: Acidente vascular encefálico / Updating Medical Emergencies Course: class 7: Encephalic vascular accident

O acidente vascular encefálico (AVE) é uma doença grave e constitui a terceira causa de morte e a primeira de seqüelas incapacitantes, em todo o mundo. Pode ser de tois tipos: isquêmico ou hemorrágico. A AVE isquêmico é mais comum (responsável por cerca de 80 por cento dos casos) e pode decorrer de trombose ou embolia das artérias cerebrais. O presente artigo destaca o quadro clínico, a conduta para diagnóstico e as medidas terapêuticas a serem adotadas, de acordo com a forma de apresentação do AVE. O autor enfatiza a importância da prevenção, a partir do reconhecimento e correção dos fatores de risco, a exemplo da hipertensão arterial

Bilateral cerebellar infarction: a rare complication of scorpion sting.

INTRODUCTION: Complications following scorpion sting are common in India and can be fatal. Stroke following scorpion sting is a rare complication and can occur by various mechanisms such as hypertension, hypotension, DIC, myocarditis and venom-induced vasculitis. We present a rare case of extensive cerebellar infarction following scorpion sting, which has rarely been reported in medical literature. OBJECTIVES: To study the clinical profile of two patients presenting with an acute onset of cerebellar symptoms following a scorpion sting. To evaluate the possible causes of the stroke and to study the relation of their symptoms to the scorpion sting. METHODS: Two young women presented with a history of acute onset of dysarthria, ataxia and incoordination following scorpion sting. They did not have any known risk factors for stroke. They had cerebellar type of dysarthria and cerebellar signs on both sides along with incoordination. A CT-scan of the brain showed bilateral extensive cerebellar infarctions. They were investigated for other causes of stroke without any positive results. With treatment the patients made a gradual but complete recovery. CONCLUSION: Since there was no evidence of hypertension, hypotension, myocarditis or disseminated intravascular coagulation, we can conclude that the patients had suffered a thrombotic stroke caused by the vasculotoxic action of the scorpion venom.

Infarto cerebelar: análise de 151 pacientes / Cerebellar infarction: analysis of 151 patients

Este estudo apresenta o tratamento de 151 pacientes com infarto cerebelar, sendo 98 homeNs (65 por cento) e 53 mulheres (35 por cento), com média de idade de 62,4 anos. Hidrocefalia obstrutiva foi diagnosticada em 7,9 por cento dos pacientes associada com um infarto cerebelar extenso e em todos os 11 pacientes operados (7,2 por cento). Quatro pacientes foram submetidos a derivação ventricular externa com 3 óbitos (75 por cento) e 7 foram submetidos a craniectomia descompressiva suboccipital com 2 óbitos (28,5 por cento). A mortalidade no grupo clínico foi de 15 pacientes (10,7 por cento). Vertigem, vômito, sinal de Romberg e dismetria foram os sinais e sintomas de envolvimento cerebelar mais frequentemente observados. Infarto cerebelar devido a embolismo provocado por cirurgia cardiovascular ocorreu em 57 pacientes (37,7 por cento).Infarto cerebelar como fato isolado ocorreu em 59 pacientes (39 por cento) e infartos cerebelares associados a infartos de outras regiões ocorreram em 92 pacientes (61 por cento). A ressonância magnética foi o melhor método para o diagnóstico das lesões, embora a tomografia pôde mostrar infarto cerebelar em 68 pacientes (78 por cento).

Aspectos neurológicos del púrpura trombótico trombocitopénico: Presentación de un caso / Thrombotic thrombocytopenic purpura with neurological involvement: Report of one case

Neurological manifestations, secondary to perfusion problems, vasogenic edema or small infarcts, are common in thrombotic purpura. Moreover, they are the first symptoms of the disease in 50% of patients. We report a 50 year-old woman who presented with focal intermittent neurological signs with aphasia and right hemiparesis, who then developed progressive impairment of consciousness with stupor and generalized tonic-clonic seizures. Despite the severe neurological impairment, first neuroimaging studies were normal. A second magnetic resonance imaging showed small cortical infarcts, that were visible only with the technique of diffusion weighted imaging. The standard electroencephalograms showed focal left temporal slowing and low voltage first and then diffuse slowing accordind to the clinical condition of the patients. She was treated with plasmapheresis and had a partial neurological recovery at the fifth day, but died at the twelfth day of therapy .

Migrainous infarct: an update

Migraine and stroke have been linked in a complex bi-directional association, though there are quite a few pathophysiological pieces to be found to complete the puzzle. International Classification of Headache Disorders-11 [ICHD-II] has defined a specific term 'migrainous infarct' that can be the crucial link between the two disorders. The authors review the latest developments related to migrainous infarct and present a compilation of case reports from 1988 to 2006

Neuropathological complications of infective endocarditis : study of autopsy material.

78 autopsy proven cases of infective endocarditis (IE) seen during 1983 to 1995 were retrospectively reviewed. The brain was available for examination in 44 cases. In the remaining cases, brain was not examined because examination of it was not requested due to lack of neurological findings. Brain lesions were observed in 35 out of 44 cases of IE. Assuming remaining 34 cases to be without brain lesions, the brain involvement in IE would be 44.87% (35 out of 78 cases). Mean age of all cases of IE and those with brain lesions was similar i.e. 26.5+/-16.6 years and 26.6+/-13.06 years respectively. Largest number of cases with neuropathological lesions were associated with normal valve IE (48.57%). Mitral valve was most commonly involved in cases with CNS complications (57.14%) (p<0.05). The various types of brain lesions were infarction (68.57%), haemorrhage (57.14%), cerebral micro-abscess (31.42%) and focal meningitis (14.28%). More than one type of lesion was observed in 19 cases, indicating complicated nature of brain lesions in fatal cases of IE. Left sided middle cerebral artery (MCA) territory was the commonest site of infarction and haemorrhage. Staphylococcus aureus appeared to be the most common organism in fatal cases of IE. Normal valve IE with or without CNS complications constitutes a significant group in India and is different from the west as far as the predisposing conditions are concerned.