Endovascular treatment in Danon disease: a case report.

BACKGROUND: Danon disease is a lysosomal storage disorder with X-linked inheritance. The classic triad is severe hypertrophic cardiomyopathy, myopathy, and intellectual disability, with different phenotypes between both genders. Ischemic stroke is an uncommon complication, mostly cardioembolic, related to intraventricular thrombus or atrial fibrillation, among others. CASE REPORT: We report the case of a 14-year-old Caucasian male patient with Danon disease who suffered from an acute ischemic stroke due to occlusion in the M1 segment of the middle cerebral artery. He underwent mechanical thrombectomy, resulting in successful revascularization with satisfactory clinical outcome. We objectified the intraventricular thrombus in the absence of arrhythmic events. CONCLUSION: To our knowledge, we report the first case of ischemic stroke related to Danon disease treated with endovascular treatment.

Case report: Endovascular embolization of a cerebral pseudoaneurysm caused by SARS-CoV2 infection.

Background: Severe COVID-19 has been shown to produce convulsions, encephalitis, Guillain-Barré syndrome, or cerebrovascular disease. However, only 4 case reports described subarachnoid or brain hemorrhage caused by ruptured cerebral aneurysms or pseudoaneurysms in patients with COVID-19. Cerebral pseudoaneurysms represent <1% of all intracranial aneurysms and have been related to radiation therapy, vasculitis, rupture of true saccular aneurysms, arteriovenous malformations, and infections by bacteria and viruses, such as Epstein-Bar and Herpes virus. Case presentation: A 28-year-old Caucasian woman, with no medical history of interest and completely vaccinated against SARS-CoV-2, was admitted to Neurology due to progressive tetraparesis with areflexia, a cough, and a fever of 38°C. SARS-CoV2 PCR was positive while lumbar puncture, blood tests, and electromyogram showed criteria for Guillain-Barré syndrome. Despite the treatment, the patient developed dyspnea and tetraplegia requiring invasive mechanical ventilation. There was motor neurological improvement but a decreased level of consciousness was observed on day 13. A brain CT scan demonstrated an acute haematoma and cerebral arteriography showed a 4-mm pseudoaneurysm located in a branch of the left middle cerebral artery. Given the high risk of rebleeding, endovascular treatment was decided upon. Therefore, complete embolization of the pseudoaneurysm was carried out by using the synthetic glue N-butyl-cyanocrylate. Two days later, the patient was clinically and neurologically recovered and was discharged. Lastly, a new angiography showed no evidence of the pseudoaneurysm 3-weeks later. Conclusions: We report, for the first time, a patient suffering a severe immune reaction caused by SARS-CoV2 infection and developing a cerebral pseudoaneurysm treated with endovascular embolization without complications.

Meningoangiomatosis: a propósito de un caso. Revisión de hallazgos en neuroimagen / Meningoangiomatosis: about a case review of findings in neuroimaging

La meningoangiomatosis es una lesión intracraneal benigna y poco frecuente, que afecta fundamentalmente a las leptomeninges y a la corteza cerebral subyacente, siendo más frecuente en niños y adultos jóvenes. Aunque la mayoría de casos se presentan de forma aislada, se ha descrito su asociación con síndromes como la neurofibromatosis tipo 2, estos últimos más frecuentemente asintomáticos y con buena respuesta farmacológica; sin embargo, las presentaciones esporádicas presentan un amplio espectro clínico, abarcando desde cefaleas crónicas hasta crisis convulsivas refractarias, llegando incluso a asociarse a lesiones intracraneales como los meningiomas. En este artículo presentamos nuestra experiencia con una paciente joven que debutó con un episodio de crisis epiléptica focal motora con evolución tónico-clónica generalizada y buena respuesta al tratamiento antiepiléptico. Dada la alta inespecificidad asociada a esta patología, tanto clínica como radiológica, nuestro objetivo es sintetizar los hallazgos radiológicos que nos permiten el planteamiento diagnóstico de esta entidad en pacientes clínicamente compatibles.(AU)

Meningoangiomatosis is a rare and benign intracranial affectation, affecting mainly leptomeninges and the underlying cerebral cortex, being more frequent in children and young adults. Although most of the cases are presented in an isolated way, it has been described its association with syndromes such as neurofibromatosis type 2, these last ones more frequently asymptomatic and with good pharmacological response; however, the sporadic presentations present a wide clinical spectrum, ranging from chronic headaches to refractory convulsive crisis, even being associated to intracranial lesions such as meningiomas.In this article we present our experience with a young patient who debuted with an episode of focal motor epileptic seizure with generalized tonic-clonic evolution and good response to antiepileptic treatment. Given the high unspecificity associated with this pathology, both clinical and radiological, our aim is to synthesize the radiological findings that allow us the diagnostic approach of this entity in clinically compatible patients.(AU)

Fibrin-Platelet Clots in Acute Ischemic Stroke. Predictors and Clinical Significance in a Mechanical Thrombectomy Series.

Introduction: The histological composition of the clot influences its mechanical properties, affects the efficacy of endovascular treatment (EVT), and could determine the clinical outcome of patients with acute ischemic stroke (AIS). Insights into clot composition may guide therapeutic decision-making prior to EVT and facilitate revascularization therapies. Material and Methods: Consecutive patients with AIS recorded in a prospective single-center reperfusion registry from December 2015 to December 2019 and treated with EVT were included. Baseline, laboratory [including post-procedural C-reactive protein (CRP)], radiological, and angiographic variables were analyzed. We aimed to study the relationship between histological composition of the clot with basal neuroimaging, laboratory markers, and recanalization technique. The secondary outcome was to analyze the correlation between clot composition and functional outcome at 3 months assessed by the modified Rankin scale (mRS). Results: From the study period, 360 AIS patients treated with EVT were included, of whom 189 (53%) fulfilled the inclusion criteria. One hundred (53%) cases of fibrin-predominant clot (FPC) were recorded. Full recanalization in FPC cases was achieved with higher probability when stent retrievers (SR) were selected as the first-line device (68.2%, p = 0.039). Patients with FPC had higher levels of CRP (p = 0.02), lower frequency of the hyperdense middle cerebral artery (HMCA) in baseline imaging (p = 0.039), and higher rates of mortality (p = 0.012). The multivariate analysis showed that the absence of HMCA (OR = 0.420; 95% CI 0.197-0.898; p = 0.025) and higher levels of CRP (OR = 1.01; 95% CI 1.003-1.019; p = 0.008) were predictors of FPC. Leukocytes and platelet counts were not associated with clot histology. Conclusions: The absence of HMCA and higher levels of CRP were markers of FPC. In patients with FPC, complete recanalization was most likely to be achieved when a SR was selected as first line of treatment. Mortality was higher in patients within this histologic group.

Comaneci device-assisted embolization of wide-necked carotid aneurysms with an unfavorable ratio.

BACKGROUND: Endovascular treatment is the technique of choice for most intracranial aneurysms. However, the treatment of morphologically complex wide-necked aneurysms with an unfavorable anatomy is still a therapeutic challenge. The purpose of the study is to describe the initial experience with the Comaneci embolization assist device for the treatment of wide-necked aneurysms with an unfavorable ratio for direct embolization. METHODS: We report a retrospective single-center analysis taken from a prospective database of consecutive aneurysms of the anterior circulation treated using the Comaneci device in the period from March 2017 to March 2019. RESULTS: Eighteen aneurysms were collected from 16 patients (9 women and 7 men) treated using the Comaneci device. The mean age was 48.4 years (range 36-81). Twelve patients had SAH, three were incidental aneurysms and one had compressive symptoms. A complete asymptomatic occlusion rate of 88.8% was obtained. The major complication rate was 5.55%. CONCLUSION: The Comaneci embolization assist device is a safe, effective option for endovascular treatment of complex aneurysms with an unfavorable ratio.

Endovascular management of an unsual case of spontaneous Retroperitoneal Haemorrhage due to Fibromuscular Dysplasia.

BACKGROUND: Fibromuscular dysplasia (FMD) is an uncommon vascular disease that results in stenosis, dissection or aneurysmal degeneration. However, it can sometimes manifest atypically, as we show in this case. CASE PRESENTATION: A 24-year old patient with no relevant medical history with severe left hypochondrium pain. The physical examination showed blood pressure levels of 160/90 mmHg. An abdominopelvic CT evidenced left retroperitoneal haematoma associated with active bleeding and left renal artery stenosis. Given these findings, it was decided to perform an endovascular treatment. Significant stenosis was seen during the arteriography in both renal arteries, suggesting fibromuscular dysplasia and development of a collateral neovascular network responsible for the retroperitoneal haematoma. It was embolised in association with angioplasty of the left renal artery. The patient had a favourable outcome; however, high blood pressure levels persisted. A new bilateral renal angioplasty was performed, which returned blood pressure values to normal. The patient was discharged without needing antihypertensives. CONCLUSIONS: FMD is a rare disease that can show multiple clinical presentations and need individualized treatment options. Endovascular techniques are in the first therapeutic line regarding fibromuscular dysplasia.

Cerebrovascular disease in patients with COVID-19: neuroimaging, histological and clinical description.

Since the appearance of the first case of coronavirus disease 2019 (COVID-19) a pandemic has emerged affecting millions of individuals worldwide. Although the main clinical manifestations are respiratory, an increase in neurological conditions, specifically acute cerebrovascular disease, has been detected. We present cerebrovascular disease case incidence in hospitalized patients with SARS-CoV-2 infection. Patients were confirmed by microbiological/serological testing, or on chest CT semiology. Available data on co-morbidity, laboratory parameters, treatment administered, neuroimaging, neuropathological studies and clinical evolution during hospitalization, measured by the modified Rankin scale, were analysed. A bivariate study was also designed to identify differences between ischaemic and haemorrhagic subtypes. A statistical model of binary logistic regression and sensitivity analysis was designed to study the influence of independent variables over prognosis. In our centre, there were 1683 admissions of patients with COVID-19 over 50 days, of which 23 (1.4%) developed cerebrovascular disease. Within this group of patients, cerebral and chest CT scans were performed in all cases, and MRI in six (26.1%). Histological samples were obtained in 6/23 cases (two brain biopsies, and four arterial thrombi). Seventeen patients were classified as cerebral ischaemia (73.9%, with two arterial dissections), five as intracerebral haemorrhage (21.7%), and one leukoencephalopathy of posterior reversible encephalopathy type. Haemorrhagic patients had higher ferritin levels at the time of stroke (1554.3 versus 519.2, P = 0.004). Ischaemic strokes were unexpectedly frequent in the vertebrobasilar territory (6/17, 35.3%). In the haemorrhagic group, a characteristic radiological pattern was identified showing subarachnoid haemorrhage, parieto-occipital leukoencephalopathy, microbleeds and single or multiple focal haematomas. Brain biopsies performed showed signs of thrombotic microangiopathy and endothelial injury, with no evidence of vasculitis or necrotizing encephalitis. The functional prognosis during the hospital period was unfavourable in 73.9% (17/23 modified Rankin scale 4-6), and age was the main predictive variable (odds ratio = 1.5; 95% confidence interval 1.012-2.225; P = 0.043). Our series shows cerebrovascular disease incidence of 1.4% in patients with COVID-19 with high morbidity and mortality. We describe pathological and radiological data consistent with thrombotic microangiopathy caused by endotheliopathy with a haemorrhagic predisposition.

Simultaneous Angioplasty and Mechanical Thrombectomy in Tandem Carotid Occlusions. Incidence of Reocclusions and Prognostic Predictors.

OBJECTIVES: The best revascularization technique for tandem carotid occlusions is not clearly defined. The primary objective of this study is to describe our technical and clinical results, analyzing the main predictors of functional independence. The secondary objective is the analysis of stent reocclusion rate. METHODS: A single-center series of 250 mechanical thrombectomies in the anterior circulation was studied. A subsequent analysis of 40 carotid occlusions was performed. The demographics, etiology, angiographic results, antithrombotic drugs, and 3-month follow-up were registered. A bivariate analysis was performed to establish the association of the study variables with major clinical complications (death, symptomatic hemorrhagic transformation and early recurrence) and the functional prognosis. In addition, the relationship between the preprocedure antiaggregation regimen and the reocclusions was studied, as well as its clinical impact. Independent predictive factors were studied using a multivariate logistic regression model. RESULTS: Complete recanalization was achieved in 30 cases (75%). Simultaneous stent placement was decided in 32 cases (80%). Functional independence was reached in 19 cases (47.5%), and 3 (7.5%) died at 3 months. Seven major complications were reported (17.5%). In patients with satisfactory revascularization where a carotid stent was used, 9 reocclusions (28.1%) were detected during the follow-up, 2 of them symptomatic. The only factor related independently with functional independence was the administration of single antiaggregation (odds ratio = .31; 95% confidence interval .002-.595; P = .021). CONCLUSIONS: Urgent endovascular treatment of tandem carotid occlusions has shown to be effective and safe in our series. The administration of single antiaggregation is a predictor of functional independence. In patients treated with carotid stent, the reocclusion rate is high, but generally asymptomatic.

Histopathological and Bacteriological Analysis of Thrombus Material Extracted During Mechanical Thrombectomy in Acute Stroke Patients.

PURPOSE: Management of stroke secondary to septic emboli (SE) remains challenging, due to both the lack of specific recommendations and the gravity of the underlying pathology.The aim of this study is to describe the presence of SE in a series of mechanical thrombectomies (MT), analyzing technical complexity and outcomes with respect to the patients by means of histological analysis and microbiological study of the clot. METHODS: All the retrieved clots were studied under an established protocol, including histopathological and bacteriological study with hematoxylin-eosin, Gram and Gomori trichrome staining.Technical complexity in SE with respect to the series was evaluated by analyzing time of the procedures, number of passes and use of intracranial definitive stents. RESULTS: Over a 24-month period, bacteria were detected in the retrieved clot of four out of 65 patients (incidence 6.2%). Two cases were eventually diagnosed with infective endocarditis, while the remaining two were diagnosed with urinary tract infection and respiratory septicemia, respectively. Three of the four patients (75%) required an intracranial definitive stent in order to achieve successful recanalization.These procedures were significantly longer (137.7 vs. 59.8 min, p < 0.001), needed a higher number of passes (5.8 vs. 2.2, p < 0.001), and delivery of an intracranial stent more frequently (75% vs. 1.6%, p = 0.008), with respect to the rest of the series. CONCLUSIONS: In our series, systematic histopathological and bacteriological study of the MT samples allowed a higher proportion of SE diagnosis in comparison with previous reports.