Hemorragia encefálica por angiopatía amiloidea / Amyloid angiopathy brain hemorrhage

Amyloid angiopathy (AA) is a selective deposition of amyloid in the walls of the brain vessels. It is a form of sporadic and localized amyloidosis, constituted by the Aβ4 protein, the same of Alzheimer's disease senile plaques. The most consistent clinical effect of AA is spontaneous brain hemorrhage (BH). It is the second most common cause of BH after arterial hypertension (HT). Other clinical manifestations are cognitive impairment and transient focal neurological episodes. AA BH is characteristically localized in the cerebral cortex and subcortical white matter (lobar hemorrhage), consistent with the preferential deposit of amyloid in the walls of leptomeningeal and intracortical small cerebral vessels. Other types of AA hemorrhagic complications are microbleeds (MB), cerebral convexity subarachnoid hemorrhage (cSAH) and superficial hemosiderosis (cSS). The diagnosis of AA BH is based on the Boston criteria. Using these criteria, several non-hemorrhagic biomarkers of AA have been identified that can be useful in its diagnosis. The principal AA BH risk factor is age, followed by cSS, MB, Apolipoprotein E gen ε2 and ε4 alleles, HT and the use of antithrombotics. This condition has a high recurrence rate that shares the same risk factors. There is no specific treatment for AA BH. It has a better prognosis than HT BH during the acute period, but worse on the long term, due to its high recurrence rate and cognitive impairment.

Hemorragia cerebral durante fase ativa de infecção por SARS-CoV-2 em paciente com angiopatia amiloide: relato de caso / Cerebral hemorrhage during the active phase of SARS-CoV-2 infection in a patient with amyloid angiopathy: case report

RESUMO As alterações neurológicas associadas à COVID-19 têm sido frequentemente descritas, principalmente nos casos de maior severidade, e estão relacionadas a causas multifatoriais, como a disfunção endotelial, a liberação de mediadores inflamatórios (cytokine storm), a disfunção endotelial e a hipoxemia. Relatamos o caso de uma paciente do sexo feminino, 88 anos, com quadro de hemorragia cerebral associada à angiopatia amiloide, no contexto de infecção por SARS-CoV-2.

ABSTRACT The neurological changes associated with COVID-19 have been frequently described, especially in cases of greater severity, and are related to multifactorial causes, such as endothelial dysfunction, inflammatory mediator release (cytokine storm), endothelial dysfunction and hypoxemia. We report the case of a female patient, 88 years old, with cerebral hemorrhage associated with amyloid angiopathy in the context of SARS-CoV-2 infection.

Cerebral amyloid angiopathy: a cross-sectional study in a single center in Northeastern Brazil / Angiopatia amiloide cerebral: um estudo transversal em um único centro no Nordeste brasileiro

ABSTRACT Background: Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder caused by progressive deposition of β-amyloid peptides in the walls of small and medium-sized cortical and leptomeningeal vessels. Until today, the prevalence of CAA is unknown in our region. Objective: This study aims to analyze the prevalence of this entity in a specific elderly population in a tertiary hospital in Northeastern Brazil. Methods: A cross-sectional, retrospective study with the enrollment of patients aged 65 or older followed in the neurological outpatient service of the Universidade Federal do Piauí, Brazil, who underwent brain magnetic resonance imaging (MRI) from July 2016 to June 2018. Results: One hundred and seventy-four patients were enrolled, of whom 100 were women (57.4%) and 74, men (42.6%), aged from 65 to 91 years old (median age 73.27). Nine patients were excluded from the study due to unavailability of MRI sequences needed for an appropriate analysis. Out of the 165 remaining patients, 12 (7.2%) had established the diagnosis of CAA, according to the modified Boston criteria. Conclusion: The prevalence of CAA in our study was like those of medical literature, with a progressive age-related increase.

RESUMO Introdução: A angiopatia amiloide cerebral (AAC) é uma desordem vascular causada pela deposição progressiva de peptídeos β-amiloides nas paredes de pequenos e médios vasos corticais e leptomeníngeos. Até a presente data, a epidemiologia da AAC é desconhecida em nossa região. Objetivos: Avaliar a prevalência da AAC em uma população específica de pacientes idosos de um hospital terciário no nordeste brasileiro. Métodos: Estudo transversal, retrospectivo, com seleção de pacientes com idade igual ou superior a 65 anos, acompanhados no serviço de Neurologia do Hospital Universitário da Universidade Federal do Piauí, Brasil, e que foram submetidos a exame de ressonância nuclear magnética entre julho de 2016 e junho de 2018. Resultados: Foram recrutados 174 pacientes, dos quais 100 eram mulheres (57,4%) e 74 homens (42,6%), com idades entre 65 e 91 anos (média de 73,27). Nove pacientes foram excluídos devido à indisponibilidade de sequências de ressonância magnética necessárias para uma análise apropriada. Dos 165 pacientes restantes, 12 (7,2%) foram diagnosticados com AAC de acordo com os critérios de Boston modificados. Conclusão: A prevalência da AAC em nosso estudo foi semelhante ao resultado encontrado na literatura médica, com um aumento progressivo relacionado à idade.

Hemorragia subaracnoídea no traumática con angiografía por tomografía computada inicial "negativa / Non-traumatic subarachnoid hemorrhage with "negative" initial computed tomography angiography

La hemorragia subaracnoidea (HSA) no traumática es un subtipo de ictus hemorrágico que representa aproximadamente el 5% de todos los accidentes vasculares encefálicos (AVE). El 85% de los casos de HSA espontánea (no traumática) son secundarios a un aneurisma intracraneano roto, el 10% a hemorragia perimesencefálica no aneurismática y el otro 5% a otras causas. Entre estas se incluyen malformaciones arterio-venosas, fístulas durales, vasculits, trombosis de vena cortical, síndrome de vasoconstricción reversible, angiopatía amiloidea y síndrome de encefalopatía posterior reversible. La aproximación inicial a una HSA no traumática requiere un estudio angiográfico no invasivo con tomografía computada para la toma de decisiones terapéuticas. Si no se detecta un aneurisma sacular intradural que explique el sangrado, las conductas a seguir dependerán del patrón de distribución de la sangre. En esta revisión sugerimos una aproximación basada en 1) revisar el estudio inicial tomando en cuenta los puntos ciegos para la detección de aneurismas, 2) analizar el patrón de distribución de la sangre y 3) analizar los hallazgos en imágenes de acuerdo a las posibles causas según patrón.

Non-traumatic subarachnoid hemorrhage represents approximately 5% of strokes. From these, 85% of nontraumatic subarachnoid hemorrhage are secondary to a ruptured aneurysm, 10% to nonaneurysmal perimesencephalic hemorrhage and the other 5% to other causes. These include but are not limited to arteriovenous malformations, dural fistulae, vasculitis, cortical vein thrombosis, reversible cerebral vasoconstriction syndrome, amyloid angiopathy and posterior reversible encephalopathy syndrome. Initial workup of nontraumatic subarachnoid hemorrhage requires a non-enhanced CT and CT angiography for decision making and management. If there is no aneurysm as a source of hemorrhage, subsequent imaging studies will depend on blood distribution pattern. In this review we suggest an approach: 1) review blind spots for aneurysm detection in the initial CT angiography, 2) analyze blood distribution pattern and 3) evaluate imaging findings and possible causes according to each pattern.

Interrelationship between implant and orthognathic surgery for the rehabilitation of edentulous cleft palate patients: a case report

A 43-year-old woman with a unilateral cleft lip and palate, presenting a totally edentulous maxilla and mandible with marked maxillomandibular discrepancy, attended the Prosthodontics section of the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo for treatment. She could not close her mouth and was dissatisfied with her complete dentures. Treatment planning comprised placement of six implants in the maxilla, four in the mandible followed by prostheses installation and orthognathic surgery. The mandibular full arch prosthesis guided the occlusion for orthognathic positioning of the maxilla. The maxillary complete prosthesis was designed to assist the orthognathic surgery with a provisional prosthesis (no metal framework), allowing reverse treatment planning. Maxillary and mandibular realignment was performed. Three months later, a relapse in the position of the maxilla was observed, which was offset with a new maxillary prosthesis. This isa complex interdisciplinary treatment and two-year follow-up is presented and discussed. It should be considered that this type of treatment could also be applied in non-cleft patients.

Relationship between cortical microinfarcts and cognitive impairment in Alzheimer disease / Relação entre microinfartos corticais e comprometimento cognitivo em doença de Alzheimer

Cerebrovascular disease and AD pathology co-exist in most dementia cases, and microinfarcts (MIs), particularly if cortical and multiple, play an additive and independent role in AD cognitive impairment. The main cause of cortical MIs ischronic cerebral hypoperfusion but occlusive vascular diseases, embolism and blood-brain barrier disruptions, isolated orcombined, may also play a role. The precise mechanisms by which MIs cause cognitive impairment are not well known, butone plausible explanation is that they are widespread and accompanied by diffuse hypoperfusion, hypoxia, oxidative stress and inflammation, particularly in the watershed areas of the tertiary association cortex, and hence could damage cognitionnetworks and explain many of ADs cognitive and behavioral disturbances. Therefore, it is crucial to control vascular risk factors and avoid uncontrolled use of the antihypertensives, neuroleptics and other sedative drugs frequently prescribed to AD patients.

Doença cerebrovascular e patologia da doença de Alzheimer (DA) coexistem na maioria dos casos de demência, nos quais os microinfartos desempenham papel relevante, aditivo e independente. A principal causa de microinfartos corticais é a hipoperfusão cerebral crônica, porém doenças vasculares oclusivas, embolismo, lesões da barreira hematoencefálica, isolados ou combinados, podem também influir. O mecanismo preciso pelo qual microinfartos comprometem a cognição não são bem conhecidos, entretanto uma explicação plausível seria que eles são extensamente distribuídos e acompanhados de hipoperfusão difusa, hipóxia, estresse oxidativo e inflamação, principalmente nas zonas de fronteiras arteriais do córtex associativo terciário, e deste modo, eles poderiam lesar as redes neurais da cognição e explicar muitos dos transtornos cognitivos e comportamentais da DA. Por isso, é crucial prevenir os fatores de risco vascular e evitar o uso exagerado de anti-hipertensivos, neurolépticos e drogas sedativas frequentemente prescritas para pacientes com DA.

Non-inflammatory cerebral amyloid angiopathy as a cause of rapidly progressive dementia: a case study / Angiopatia amilóide cerebral não inflamatória como causa de demência rapidamente progressiva: estudo de caso

Abstract ? A 77 year-old men developed a subacute-onset, rapidly progressive cognitive decline. After 6 months of evolution, he scored 6 on the Mini-Mental State Examination and had left hemiparesis and hemineglect. The patient died 11 months after the onset of cognitive symptoms. Brain MRI showed microhemorrhages on gradient-echo sequence and confluent areas of white matter hyperintensities on T2-weighted images. Brain biopsy revealed amyloid-? peptide deposition in vessel walls, some of them surrounded by micro-bleeds. In this case report, we discuss the role of cerebral amyloid angiopathy (CAA) in cognitive decline, due to structural lesions associated with hemorrhages and infarcts, white matter lesions and co-morbidity of Alzheimer?s disease, as well as the most recently described amyloid angiopathy-related inflammation.

Resumo ? Um homem de setenta e sete anos desenvolveu quadro de início subagudo de demência rapidamente progressiva. Após seis meses de evolução, sua pontuação do Mini exame do estado mental foi de 6, e no exame neurológico apresentava heminegligência e hemiparesia esquerda. O paciente faleceu 11 meses após início dos sintomas. RM do encéfalo mostrou microhemorragias em sequência gradiente-echo e áreas confluentes de hiperintensidade de substância branca nas imagens pesadas em T2. Na biópsia cerebral observou-se deposição de peptídeo ?-amilóide em vasos sanguíneos, alguns rodeados por microhemorragias. Neste relato de caso, discutimos o papel da angiopatia amiloide cerebral no declínio cognitivo, devido a lesões estruturais associadas a hemorragias e infartos, lesões de substância branca e comorbidade com doença de Alzheimer, assim como a mais recentemente descrita inflamação relacionada a angiopatia amiloide.

Angiopatia amilóide cerebral simulando tumor cerebral: relato de caso / Cerebral amyloid angiopathy presenting as a brain tumor: case report

Descrevemos o raro caso de um paciente de 45 anos portador de lesão expansiva intracraniana por angiopatia amilóide cerebral com características clínicas e de imagem compatíveis com um glioma de baixo grau. A biópsia revelou angiopatia amilóide cerebral. Os achados clínicos, radiológicos e histopatológicos são discutidos e analisados juntamente à literatura disponível.

Pericardite constritiva em paciente com passado de múltiplas trocas de valva mitral, complicada com insuficiência tricúspide grave e hemorragia cerebral por angiopatia amilóide / Constrictive pericarditis in the patient with a history of numerous mitral valve replacements, complicated by serious tricuspid regurgitation and cerebral hemorrhage for amyloid angiopathy

Paciente do sexo feminino, portadora de cardiopatia reumática, com passado de cirurgias de troca valvar mitral que desenvolveu insuficiência tricúspide grave e pericardite constritive, fez hemorragia cerebral grave por angiopatia amilóide, tendo evoluído para óbito

Hemorragias cerebrales y angiopatía amiloidea / Cerebral amyloid angiopathy in brains of patients dying of non traumatic cerebral hemorrhages

Background: Cerebral amyloid angiopathy is considered pathogenic in non traumatic cerebral lobar hemorrhages. Aim: To study the frequency of cerebral amyloid angiopathy in brains of patients dying of non traumatic cerebral hemorrhages. Material and methods: Thirty seven brains from patients, 25 men and aged 65ñ10 years old, with cerebral hemorrhages (14 lobar, 18 in basal ganglia and 5 in cerebellum or brainstem) were studied. As controls, the brains of 30 subjects, 14 men and aged 64ñ16 years old, dying of non neurological causes were studied. Deep and cortical vessels were stained with hematoxylin eosin, Gomori, Thioflavin T and Bodian. Definitive cerebral amyloid angiopathy was diagnosed when amyloid deposition was observed in the media of vessels. Results: Twenty six out of 32 patients dying of cerebral hemorrhage and 3 of 21 controls had chronic hypertension. Cerebral amyloid angiopathy was present in 19 of 37 brains of patients with cerebral hemorrhage and 13 of 30 control brains. In patients with hypertension, vascular changes independent of the location and volume of amyloid deposition, were observed. Such changes were dilatation, tortuousness, thickening of walls specially in muscular and adventitia and hyaline degeneration. Thirteen brains with hemorrhage had fibrinoid necrosis and 10 had microaneurysms. Conclusions: In this series of patients, cerebral amyloid deposition was unspecific and its role in the pathogenesis of cerebral hemorrhages was not confirmed. Hypertension was associated with vascular degenerative changes that can lead to cerebral hemorrhages

Angiopatía cerebral amiloide: informe de tres casos autopsia / Cerebral amyloid angiopathy. A report of three autopsy cases

Hay diferentes enfermedades que cursan con depósito de amiloidosis sistémica. Este material se deposita en los vasos pequeños corticales y de las leptomeninges y generalmente se manifiesta con demencia o hemorragia. Se informa un caso que cursó clínicamente con demencia, y dos que murieron como consecuencia de una hemorragia parenquimatosa extensa. Todos tenían angiopatía amiloide. Se revisan los conceptos sobre angiopatía amiloide, las enfermedades en las que se presenta y el posible mecanismo de la proteína beta, componente del amiloide de la mayor parte de las entidades con amiloidosis cerebral primaria

Hemorragia cerebral lobar clínica, etiologia, evolução. Revisão de 58 casos / Lobar cerebral hemorrhage. Clinical aspects, etiology and course. Review of 58 cases

Fifty eight patients with spontaneous lobar hemorrhage of a 264 patients series with intracerebral hemorrhage seen during a six years period are reviewed. Twenty nine had arterial hypertension, 9 had other etiologies, and in the remaining 20 cases no apparent etiology was detected. Headache was a prominent symptom and occurred in 56%. Seizures occurred in 12%. Coma was infrequent. Clinical findings on admission depended on the location of the hematoma. Lobar hemorrhage occurred most commonly in the parietal region. Mortality rate was 8.6%. Size of hematoma (larger than 4 cm) on CT and altered consciousness on admission correlated with poor outcome.

Hemorragia intracerebral secundaria a la evacuación de hematoma subdural crónico / Brain hemorrhage secondary to the evacuation of chronic subdural hematoma

Se investigan las posibles causas de complicaciones hemorrágicas intracerebrales secundarias a la evacuación de colecciones líquidas intracraneanas, tomando como modelo clínico el hematoma subdural crónico y se postula la preexistencia de una angiopatía cerebral de base para este fenómeno, especialmente referido a personas sobre 65 años de edad, hipertensos, con elementos clínicos de deterioro sicorgánico. Se comenta además la posible gravitación de la técnica quirúrgica empleada para el vaciamiento