Acute management of cerebral venous thrombosis: Indications, technique, and outcome of endovascular treatment in two high-volume centers.

INTRODUCTION: After several uncontrolled studies and one randomized clinical trial, there is still uncertainty regarding the role of endovascular treatment (EVT) in cerebral venous thrombosis (CVT). This study aims to describe and assess different acute management strategies in the treatment of CVT. METHODS: We performed a retrospective analysis of an international two-center registry of CVT patients admitted since 2019. Good outcome was defined as a return to baseline modified Rankin scale at three months. We described and compared EVT versus no-EVT patients. RESULTS: We included 61 patients. Only one did not receive systemic anticoagulation. EVT was performed in 13/61 (20%) of the cases, with a median time from diagnosis to puncture of 4.5 h (1.25-28.5). EVT patients had a higher median baseline NIHSS [6 (IQR 2-17) vs 0 (0-2.7), p = 0.002)] and a higher incidence of intracerebral hemorrhage (53.8% vs 20.3%, p = 0.03). Recanalization was achieved in 10/13 (77%) patients. Thrombectomy was performed in every case with angioplasty in 7 out of 12 patients and stenting in 3 cases. No postprocedural complication was reported. An improvement of the median NIHSS from baseline to discharge [6 (2-17) vs 1(0-3.75); p < 0.001] was observed in EVT group. A total of 31/60 patients (50.8%) had good outcomes. Adjusting to NIHSS and ICH, EVT had a non-significant increase in the odds of a good outcome [aOR 1.42 (95%CI 0.73-2.8, p = 0.307)]. CONCLUSIONS: EVT in combination with anticoagulation was safe in acute treatment of CVT as suggested by NIHSS improvement. Selected patients may benefit from this treatment.

Effectiveness of Intra-Arterial Chemotherapy for the Treatment of Intraocular Retinoblastoma: Relevance of a Multidisciplinary Setting.

Purpose: We aim to report about effectiveness and safety in the context of our centers' setting in the management of retinoblastoma with intra-arterial chemotherapy (IAC) in a 5-year retrospective analysis of the Portuguese population. Patients and Methods: Retrospective analysis of consecutive cases of retinoblastoma selected to initiate IAC between 2015 and 2020, at the Portuguese National Reference Center. All included patients underwent complete ophthalmological evaluation under anesthesia with fundus photography. Diagnosis and classification of retinoblastoma was made according to the International Classification of Intraocular Retinoblastoma (ICRB). The patients were further divided into two groups: Group I for primary IAC and Group II for secondary IAC. Tumor recurrence or relapses, systemic metastasis and deaths were documented. Main efficacy outcome included ocular salvage and recurrence-free survival rates estimated using the Kaplan-Meier method. Results: Twenty-eight eyes (19 eyes included in Group I and 9 eyes included in Group II) were eligible and a total of 130 IAC procedures were performed, with a median number of sessions of 4 (range 1-8) for each treated eye, during a median follow-up of 21 months (range 4-64). Of the included eyes, 22 (78.6%) were preserved. An overall survival of 100% was achieved. Considering the preserved eyes, the overall median decimal visual acuity achieved at the last visit was 0.15 (range 0.02-0.8). Three patients had permanent adverse events related to IAC (cataract, vitreous hemorrhage and choroidal ischemia). Considering the survival analysis of recurrence, the mean survival without recurrence was 84.2% for Group I and 66.7% for Group II, and the mean survival without enucleation was 78.6% (no events in Group II). Conclusion: IAC has been shown to be an effective and safe treatment for children with intraocular retinoblastoma. This study demonstrates that IAC is effective even in moderate sample sizes, when a multidisciplinary approach is available.

Safety and Outcome of Revascularization Treatment in Patients With Acute Ischemic Stroke and COVID-19: The Global COVID-19 Stroke Registry.

BACKGROUND AND OBJECTIVES: COVID-19-related inflammation, endothelial dysfunction, and coagulopathy may increase the bleeding risk and lower the efficacy of revascularization treatments in patients with acute ischemic stroke (AIS). We aimed to evaluate the safety and outcomes of revascularization treatments in patients with AIS and COVID-19. METHODS: This was a retrospective multicenter cohort study of consecutive patients with AIS receiving intravenous thrombolysis (IVT) and/or endovascular treatment (EVT) between March 2020 and June 2021 tested for severe acute respiratory syndrome coronavirus 2 infection. With a doubly robust model combining propensity score weighting and multivariate regression, we studied the association of COVID-19 with intracranial bleeding complications and clinical outcomes. Subgroup analyses were performed according to treatment groups (IVT-only and EVT). RESULTS: Of a total of 15,128 included patients from 105 centers, 853 (5.6%) were diagnosed with COVID-19; of those, 5,848 (38.7%) patients received IVT-only and 9,280 (61.3%) EVT (with or without IVT). Patients with COVID-19 had a higher rate of symptomatic intracerebral hemorrhage (SICH) (adjusted OR 1.53; 95% CI 1.16-2.01), symptomatic subarachnoid hemorrhage (SSAH) (OR 1.80; 95% CI 1.20-2.69), SICH and/or SSAH combined (OR 1.56; 95% CI 1.23-1.99), 24-hour mortality (OR 2.47; 95% CI 1.58-3.86), and 3-month mortality (OR 1.88; 95% CI 1.52-2.33). Patients with COVID-19 also had an unfavorable shift in the distribution of the modified Rankin score at 3 months (OR 1.42; 95% CI 1.26-1.60). DISCUSSION: Patients with AIS and COVID-19 showed higher rates of intracranial bleeding complications and worse clinical outcomes after revascularization treatments than contemporaneous non-COVID-19 patients receiving treatment. Current available data do not allow direct conclusions to be drawn on the effectiveness of revascularization treatments in patients with COVID-19 or to establish different treatment recommendations in this subgroup of patients with ischemic stroke. Our findings can be taken into consideration for treatment decisions, patient monitoring, and establishing prognosis. TRIAL REGISTRATION INFORMATION: The study was registered under ClinicalTrials.gov identifier NCT04895462.

Single-phase CT angiography predicts ASPECTS decay and may help determine when to repeat CT before thrombectomy.

OBJETIVES: Time is relative in large-vessel occlusion acute ischemic stroke (LVO-AIS). We aimed to evaluate the rate of inter-hospital ASPECTS decay in patients transferred from a primary (PSC) to a comprehensive stroke center (CSC); and to identify patients that should repeat computed tomography (CT) before thrombectomy. MATERIALS AND METHODS: This was a retrospective cohort study of consecutive anterior circulation LVO-AIS transferred patients. The rate of ASPECTS decay was defined as (PSC-ASPECTS - CSC-ASPECTS)/hours elapsed between scans. Single-phase CT angiography (CTA) at the PSC was used to classify the collateral score. We compared patients with futile versus useful CT scan re-evaluation. RESULTS: We included 663 patients, of whom 245 (37.0%) repeated CT at a CSC. The median rate of ASPECTS decay was 0.4/h (0.0-0.9). Patients excluded from thrombectomy after a CT scan repeat (n=64) had a median ASPECTS decay rate of 1.18/h (0.83-1.61). Patients with absent collateral circulation had a median rate of 1.51(0.65-2.19). The collateral score was an independent predictor of the ASPECTS decay rate (aß = -0.35; 95%CI -0.45 - -0.19, p<0.001). Age (aOR: 1.04 95% CI 1.02-1.07, p<0.001), NIHSS (aOR: 1.11 95% CI 1.06-1.15, p<0.001), PSC ASPECTS (aOR: 0.74 95% CI 0.60-0.91, p=0.006) and the CTA collateral score (aOR: 0.14 95% CI 0.08-0.22, p<0.001) were independent predictors of the usefulness of a CT scan repeat. CONCLUSIONS: The rate of ASPECTS decay can be predicted by the CTA collateral score, helping in the selection of patients that would benefit from repeating a CT assessment on arrival at the CSC.

Nationwide Access to Endovascular Treatment for Acute Ischemic Stroke in Portugal.

INTRODUCTION: Since the publication of endovascular treatment trials and European Stroke Guidelines, Portugal has re-organized stroke healthcare. The nine centers performing endovascular treatment are not equally distributed within the country, which may lead to differential access to endovascular treatment. Our main aim was to perform a descriptive analysis of the main treatment metrics regarding endovascular treatment in mainland Portugal and its administrative districts. MATERIAL AND METHODS: A retrospective national multicentric cohort study was conducted, including all ischemic stroke patients treated with endovascular treatment in mainland Portugal over two years (July 2015 to June 2017). All endovascular treatment centers contributed to an anonymized database. Demographic, stroke-related and procedure-related variables were collected. Crude endovascular treatment rates were calculated per 100 000 inhabitants for mainland Portugal, and each district and endovascular treatment standardized ratios (indirect age-sex standardization) were also calculated. Patient time metrics were computed as the median time between stroke onset, first-door, and puncture. RESULTS: A total of 1625 endovascular treatment procedures were registered. The endovascular treatment rate was 8.27/100 000 inhabitants/year. We found regional heterogeneity in endovascular treatment rates (1.58 to 16.53/100 000/year), with higher rates in districts closer to endovascular treatment centers. When analyzed by district, the median time from stroke onset to puncture ranged from 212 to 432 minutes, reflecting regional heterogeneity. DISCUSSION: Overall endovascular treatment rates and procedural times in Portugal are comparable to other international registries. We found geographic heterogeneity, with lower endovascular treatment rates and longer onset-to-puncture time in southern and inner regions. CONCLUSION: The overall national rate of EVT in the first two years after the organization of EVT-capable centers is one of the highest among European countries, however, significant regional disparities were documented. Moreover, stroke-onset-to-first-door times and in-hospital procedural times in the EVT centers were comparable to those reported in the randomized controlled trials performed in high-volume tertiary hospitals.

Introdução: A aprovação do tratamento endovascular para o acidente vascular cerebral isquémico obrigou à reorganização dos cuidados de saúde em Portugal. Os nove centros que realizam tratamento endovascular não estão distribuídos equitativamente pelo território, o que poderá causar acesso diferencial a tratamento. O principal objetivo deste estudo é realizar uma análise descritiva da frequência e métricas temporais do tratamento endovascular em Portugal continental e seus distritos. Material e Métodos: Estudo de coorte nacional multicêntrico, incluindo todos os doentes com acidente vascular cerebral isquémico submetidos a tratamento endovascular em Portugal continental durante um período de dois anos (julho 2015 a junho 2017). Foram colhidos dados demográficos, relacionados com o acidente vascular cerebral e variáveis do procedimento. Taxas de tratamento endovascular brutas e ajustadas (ajuste indireto a idade e sexo) foram calculadas por 100 000 habitantes/ano para Portugal continental e cada distrito. Métricas de procedimento como tempo entre instalação, primeira porta e punção foram também analisadas. Resultados: Foram registados 1625 tratamentos endovasculares, indicando uma taxa bruta nacional de tratamento endovascular de 8,27/100 000 habitantes/ano. As taxas de tratamento endovascular entre distritos variaram entre 1,58 e 16,53/100 000/ano, com taxas mais elevadas nos distritos próximos a hospitais com tratamento endovascular. O tempo entre sintomas e punção femural entre distritos variou entre 212 e 432 minutos. Discussão: A análise nacional a taxas de tratamento endovascular e tempos de atuação é comparável a outros registos internacionais. Verificaram-se heterogeneidades geográficas, com taxas de tratamento endovascular menores e maior tempo para tratamento nos distritos do sul e interior. Conclusão: Portugal continental apresenta uma taxa nacional de tratamento endovascular elevada, apresentando, contudo, assimetrias regionais no acesso. As métricas temporais foram comparáveis com as observadas nos ensaios clínicos piloto.

Collateral pial circulation relates to the degree of brain edema on CT 24 hours after ischemic stroke.

Background Cerebral edema is frequent in patients with acute ischemic stroke (AIS) who undergo reperfusion therapy and is associated with high mortality. The impact of collateral pial circulation (CPC) status on the development of edema has not yet been determined. Methods We studied consecutive patients with AIS and documented M1-middle cerebral artery (MCA) and/or distal internal carotid artery (ICA) occlusion who underwent reperfusion treatment. Edema was graded on the 24-hour non-contrast computed tomography (NCCT) scan. CPC was evaluated at the acute phase (≤6 hours) by transcranial color-coded Doppler, angiography and/or CT angiography. We performed an ordinal regression model for the effect of CPC on cerebral edema, adjusting for age, baseline National Institutes of Health Stroke Scale, Alberta Stroke Program Early Computed Tomography Score (ASPECTS) on admission, NCCT, parenchymal hemorrhagic transformation at 24 hours and complete recanalization at six hours. Results Among the 108 patients included, 49.1% were male and mean age was 74.2 ± 11.6 years. Multivariable analysis showed a significant association between cerebral edema and CPC status (OR 0.22, 95% CI 0.08-0.59, p = 0.003), initial ASPECTS (OR 0.72, 95% CI 0.57-0.92, p = 0.007) and parenchymal hemorrhagic transformation (OR 23.67, 95% CI 4.56-122.8, p < 0.001). Conclusions Poor CPC is independently associated with greater cerebral edema 24 hours after AIS in patients who undergo reperfusion treatment.

Brain atrophy and physical disability in primary progressive multiple sclerosis: A volumetric study.

UNLABELLED: Grey matter atrophy has been shown in primary progressive multiple sclerosis (PPMS), but its association with physical incapacity is unclear. We submitted 19 patients with PPMS to a neurological evaluation and brain magnetic resonance imaging (MRI) with volumetric analysis using FreeSurfer. We found no relation between the Expanded Disability Status Scale or disease duration and the grey matter or white matter structures analysed. Lesion load was negatively correlated with cortical and subcortical grey matter volumes, but not with total white matter volume. We concluded that physical disability in PPMS is not directly related to brain atrophy and that focal inflammatory white matter lesions may contribute to progressive neuronal degeneration. INTRODUCTION: Primary progressive multiple sclerosis (PPMS) is characterized by chronic progression since onset, with predominant involvement of the spinal cord and prominent neurodegeneration. Grey matter atrophy has been shown in patients with PPMS, but its association with clinical incapacity is uncertain. We investigated the relationship between regional brain atrophy and physical disability in patients with PPMS. METHODS: Patients with an established diagnosis of PPMS underwent a neurological evaluation followed by brain MRI at 1.5 T. Volumetric analysis was performed with FreeSurfer software, and evaluated the neocortex, total white matter, total subcortical grey matter, putamen, caudate, globus pallidus, thalamus, hippocampus, brainstem, corpus callosum and pre-central gyrus volumes. Clinical data obtained included physical disability as measured by the Expanded Disability Status Scale (EDSS). RESULTS: Nineteen patients were included, 14 female (73.7%), mean age of 55.7 (SD 7.6) and mean disease duration of 13.0 years (SD 8.8). Median EDSS score was 6.0 (3.5-8.0). The average T1 lesion load (4.9 cm³, SD 3.4) and T2 load (10.5 cm³, SD 9.9) did not relate to disease duration. There was no significant correlation between EDSS score or disease duration and the cortical grey matter, deep grey matter or white matter structures analysed. Lesion load was negatively correlated with cortical and subcortical grey matter volumes (p < 0.05), but not with total white matter volume. CONCLUSIONS: Physical disability in PPMS is not directly related to brain volume loss. Grey matter atrophy correlates with lesion load in patients with PPMS, indicating that focal inflammatory white matter lesions may contribute to progressive neuronal degeneration.

Monitoring balloon test occlusion of the internal carotid artery with transcranial Doppler. A case report and literature review.

Angiographic balloon test occlusion (BTO) allows preoperative risk evaluation of patients undergoing permanent therapeutic occlusion of the internal carotid artery (ICA). The sensitivity of the BTO can be increased using different complementary techniques. Transcranial Doppler (TCD) stands out as a non-invasive, bedside method providing real-time monitoring of cerebral haemodynamics, therefore accurately identifying patients at risk of stroke. A case of a 30-year-old woman with a giant intracavernous aneurysm of the left ICA presenting with subacute left VI nerve palsy is described. A pre-operative TCD- and EEG-monitored BTO of the left ICA was performed. The 16.7% drop found in the middle cerebral artery's peak systolic velocity (PSVMCA) predicts clinical and haemodynamic tolerance to the permanent loss of that vessel. This case illustrates the potential of TCD monitoring during temporary BTO of the ICA. It highlights its ability to provide a complete preclinical evaluation of collateralization and autoregulatory adaptation to unilateral ICA occlusion. TCD may also decrease the time of occlusion required for the BTO.

Traumatic middle cerebral artery aneurysm secondary to a gunshot wound.

Traumatic intracranial aneurysms are rare lesions, accounting for less than 1% of all intracranial aneurysms. Formation of these lesions after a penetrating missile wound is very unusual, and diagnosis can be difficult due to the presence of associated lesions. In this article, we report a case of a woman who developed a middle cerebral artery aneurysm after a gunshot wound, and discuss potential pitfalls found during diagnostic work-up.

[Bilateral hyperintensity of the pulvinar and dorsomedial nucleus of the thalamus in sporadic Creutzfeldt-Jakob disease]. / Hiperintensidade bilateral do pulvinar e núcleo dorso-medial do tálamo na forma esporádica da doença de Creutzfeldt-Jakob.

INTRODUCTION: Creutzfedt-Jakob Disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Early diagnosis and the determination of its form are epidemiologically important, with strong impact on public health. Bilateral pulvinar hyperintensity, either alone (pulvinar sign) or in association with the dorsomedial nucleus of the thalamus (double hockey stick sign) on T2, FLAIR and diffusion weighted imaging (DWI), is a criterion for the probable diagnosis of the variant CJD (vCJD). Bilateral hyperintensity of the caudate, putamina and cortex is the usual pattern found in the sporadic CJD (sCJD). OBJECTIVE: Analysis of the imaging aspects on a sCJD patient showing T2 hyperintensity of the pulvinar and dorsomedial thalamic nucleus, in order to assess the magnetic resonance imaging (MRI) accuracy in the discrimination between vCJD and sCJD, when this lesion pattern is present. METHODS: We performed a MRI on a 62-year-old female with definitive diagnosis of sCJD made by anatomopathologic study of the brain tissue. Qualitative analysis of MRI, including DWI, T2 and FLAIR sequences, as well as lesional patterns found. RESULTS: Brain MRI showed hyperintensity of the caudate, putamina, pulvinar and dorsomedial nucleus of the thalamus, in DWI, T2 and FLAIR sequences; hypersignal of the caudate and putamina was greater than the signal intensity of the thalami. Hyperintensity of the hippocampus and frontal, temporal and parietal cortex were more obvious in FLAIR and DWI. COMMENT: Hyperintensity of the pulvinar and dorsomedial nucleus of the thalamus on sCJD may complicate the differential diagnosis with vCJD. True pulvinar sign and double hockey stick sign, consistent with vCJD, must only be considered if the hyperintensity is greater than signal intensity of the caudate and putamina.

Multiple dural arteriovenous fistulas presenting as rapidly progressive dementia.

INTRODUCTION: Dural arteriovenous fistulas (DAVFs) are important causes of neurological dysfunction and are many times misdiagnosed. Particularly in older populations, DAVFs may present with a selective cognitive dysfunction. CASE REPORT: The authors describe a 70-year-old woman presenting with a rapidly progressive dementia, very similar in presentation to prion disease. Neuroimaging showed multifocal DAVFs associated with venous thrombosis and white matter changes, suggesting that impaired cerebral circulation due to venous hypertensive encephalopathy caused the patient's dementia. Prompt treatment of some of the abnormal shunts with endovascular embolization resulted in a clinically relevant improvement. Subsequent clinical improvement was achieved with anticoagulation, although no cause or predisposing factor was documented that could have led to the development of the venous thrombosis. CONCLUSIONS: Neurologists should maintain a high degree of suspicion to avoid missing the diagnosis of DAVFs that are potentially treatable lesions.

[Cerebral venous thrombosis: retrospective analysis of 49 cases]. / Trombose venosa cerebral: análise retrospectiva de 49 casos.

INTRODUCTION: Cerebral Venous Thrombosis (CVT) is a rare and potentially life-threatening disease, accounting for about 0.5% of stroke cases. However, it is believed to be an underdiagnosed condition. Early diagnosis requires a high degree of suspicion and appropriate use of imaging modalities. OBJECTIVES: Imagiological and clinical characterization of CVT cases diagnosed at our hospital from 2004 to 2007. METHODS: This study was a retrospective, cross-sectional analysis from 2004 to 2007, using our institution database. We reviewed hospital discharge data to assess the incidence of CVT. The study population consisted of 49 patients. Retrospective review of the clinical data and imaging studies of these patients was then performed. RESULTS: Of the 49 patients with confirmed CVT, 38 were female. Patient age varied between 16 and 75 years, with an average of 42.6 years. Thrombotic risk factors were found in 43 patients; the most frequent was dyslipidemia (n = 22) followed by oral contraceptive use (n = 18). Initial head Computerized Tomography (CT) was normal in six cases. Diagnosis was made by Magnetic Resonance (MR) in 38 cases, Cerebral CT-Venography in 10 cases and Digital Subtraction Angiography in one case. Average time from onset of symptoms to diagnosis was nine days; this was not significantly different when comparing the group diagnosed by MR with the group diagnosed by CT-Venography. Right transverse sinus was the most frequent location of thrombosis (n = 36). Only in four cases thrombosis did not involve the lateral sinuses. CONCLUSIONS: Lateral sinus thrombosis is a frequent variety of CVT, accounting for 91.8% of our cases. A negative Head CT scan does not exclude the presence of cerebral venous thrombosis; therefore appropriate imaging study should be performed whenever there's a high degree of clinical suspicion. Cerebral CT-Venography seems to be a good alternative to MR for the diagnosis of CVT.

Specific retinotopically based magnocellular impairment in a patient with medial visual dorsal stream damage.

We report here retinotopically based magnocellular deficits in a patient with a unilateral parieto-occipital lesion. We applied convergent methodologies to study his dorsal stream processing, using psychophysics as well as structural and functional imaging. Using standard perimetry we found deficits involving the periphery of the left inferior quadrant abutting the horizontal meridian, suggesting damage of dorsal retinotopic representations beyond V1. Retinotopic damage was much more extensive when probed with frequency-doubling based contrast sensitivity measurements, which isolate processing within the magnocellular pathway: sensitivity losses now encroached on the visual central representation and did not respect the horizontal meridian, suggesting further damage to dorsal stream retinotopic areas that contain full hemi-field representations, such as human V3A or V6. Functional imaging revealed normal responses of human MT+ to motion contrast. Taken together, these findings are consistent with a recent proposal of two distinct magnocellular dorsal stream pathways: a latero-dorsal pathway passing to MT+ and concerned with the processing of coherent motion, and a medio-dorsal pathway that routes information from V3A to the human homologue of V6. Anatomical evidence was consistent with sparing of the latero-dorsal pathway in our patient, and was corroborated by his normal performance in speed, direction discrimination and motion coherence tasks with 2D and 3D objects. His pattern of dysfunction suggests damage only to the medio-dorsal pathway, an inference that is consistent with structural imaging data, which revealed a lesion encompassing the right parieto-occipital sulcus.

Blue rubber-bleb nevus syndrome: report of a familial case with a dural arteriovenous fistula.

We report a case of a 45-year-old woman known to have a familial form of blue rubber-bleb nevus syndrome (BRBNS). The patient developed severe occipital headaches and bilateral retroauricular bruits. Cerebral angiography showed a large dural arteriovenous fistula in the torcular region. Central nervous system involvement in BRBNS is not often reported, and most cases of BRBNS are sporadic.