Search

Background and Purpose: Ischemic stroke has been reported in various conditions associated with eosinophilia. FIP1L1-PDGFRA fusion ([Fip1-like 1-platelet-derived growth factor receptor alpha]; F/P ) leads to the proliferation of the eosinophilic lineage and thus to a clonal hypereosinophilic syndrome that is highly responsive to imatinib. Methods: We previously reported on a nationwide retrospective study of 151 patients with F/P -associated clonal hypereosinophilic syndrome. Patients from this cohort with a clinical history of ischemic stroke (as well as 2 additional cases) were further analyzed to better define their clinical picture and outcomes. Results: Sixteen male patients (median age, 51 [43–59] years) with low-to-intermediate cardiovascular risk were included. Median National Institutes of Health Stroke Scale was 4 (range, 1–6). Most cerebral imaging disclosed multiple bilateral infarctions of watershed distribution (69%). Despite frequent cardiac involvement (50%), cardiac thrombus was evidenced in a single patient and, according to the TOAST classification (Trial of ORG 10172 in Acute Stroke Treatment), 62.5% of strokes were presumably of undetermined etiology. Among the 15 patients treated with imatinib, and after a median follow-up of 4.5 years, stroke recurred in only 3 patients (consisting of either cardio embolic or hemorrhagic events, unrelated to the first episode). Conclusions: F/P + clonal hypereosinophilic syndrome is a diagnosis to consider in patients with unexplained ischemic stroke and hypereosinophilia (especially in the setting of multiple cortical borderzone distribution) and warrants prompt initiation of imatinib.

Background and Purpose: Stroke is reported as a consequence of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in several reports. However, data are sparse regarding the details of these patients in a multinational and large scale. Methods: We conducted a multinational observational study on features of consecutive acute ischemic stroke, intracranial hemorrhage, and cerebral venous or sinus thrombosis among SARS-CoV-2–infected patients. We further investigated the risk of large vessel occlusion, stroke severity as measured by the National Institutes of Health Stroke Scale, and stroke subtype as measured by the TOAST (Trial of ORG 10172 in Acute Stroke Treatment) criteria among patients with acute ischemic stroke. In addition, we explored the neuroimaging findings, features of patients who were asymptomatic for SARS-CoV-2 infection at stroke onset, and the impact of geographic regions and countries’ health expenditure on outcomes. Results: Among the 136 tertiary centers of 32 countries who participated in this study, 71 centers from 17 countries had at least 1 eligible stroke patient. Of 432 patients included, 323 (74.8%) had acute ischemic stroke, 91 (21.1%) intracranial hemorrhage, and 18 (4.2%) cerebral venous or sinus thrombosis. A total of 183 (42.4%) patients were women, 104 (24.1%) patients were P =0.02) and higher rate of mechanical thrombectomy (12.4% versus 2%; P Conclusions: We observed a considerably higher rate of large vessel occlusions, a much lower rate of small vessel occlusion and lacunar infarction, and a considerable number of young stroke when compared with the population studies before the pandemic. The rate of mechanical thrombectomy was significantly lower in countries with lower health expenditures.

Background and Purpose— Cortical microinfarcts and secondary cortical degeneration have been demonstrated in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a severe monogenic cerebral small vessel disease. The aim of this study was to determine whether focal macroscopic cortical lesions can be detected using a specific in vivo magnetic resonance imaging approach. Methods— Three-dimensional T1 magnetic resonance imaging scans were obtained in 28 nondemented nondisabled CADASIL patients and 29 age- and sex-matched controls. The cortical mantle of patients and controls were extracted using Brainvisa by an experienced user and then evaluated during a dedicated reading session by a second reader after removing the white matter to stay blind to the clinical status. Thereafter, confirmed focal macroscopic cortical lesions were characterized using all available imaging data, including 7-T magnetic resonance imaging in some patients. Results— Three focal macroscopic cortical lesions were confirmed in 3 of 28 patients (11%) but none in controls. All lesions were observed in the close vicinity of severe signal changes in the underlying white matter. Conclusions— Focal macroscopic cortical lesions can be detected using specific magnetic resonance imaging approaches in CADASIL patients long before the end stage of the disorder. The underlying mechanisms and precise clinical consequences of these cortical changes still need to be determined.

Il s’agit d’une patiente âgee de 71 ans ayant presente un syndrome cerebelleux statique et cinetique severe associe a une crise epileptique generalisee unique et des troubles visuels transitoires. L’imagerie par resonance magnetique a mis en evidence un hypersignal FLAIR isole du cervelet.

Background Recommended treatments for spontaneous intracranial hypotension (SIH) range from bed rest only to neurosurgery. However, the serious form of SIH is poorly defined. A better description of patient characteristics and their outcome may help define therapeutic options. Methods We reviewed 24 cases of patients with SIH and separated them into 2 groups according to whether or not they presented with signs of severity at admission: disturbance of consciousness, subdural hematomas (SDHs), and cerebral venous thrombosis. Results Nine patients (37%) were classified as having a serious form of SIH: six (25%) presented SDHs; three (12%) disturbance of consciousness; and one (4%) cerebral venous thrombosis. Bed rest and epidural blood patches (EBPs) were sufficient to treat all patients in the nonserious form group and 4 patients in the serious form group. Two patients (8%) had to undergo cerebrospinal fluid leak repair, and 3 others (12%) evacuation of SDHs. Outcome was good in both groups, with only one (4%) death due to extensive SDHs. Times to diagnosis in the serious form group (63 vs. 35 days, P = 0.052) and to recovery (9 months vs. 5 months, P = 0.088) tended to be higher without reaching difference. Conclusions The presence of SDHs, disturbance of consciousness, and a trend toward a longer time to diagnosis and recovery seem to define the serious form of SIH. These patients may require exploration and surgical repair of cerebrospinal fluid leak, only after failure of conservative measures—bed rest and time—and EBP, with good outcome.

Objective:To assess whether 18F-florbetapir, a PET amyloid tracer, could bind vascular amyloid in cerebral amyloid angiopathy (CAA) by comparing cortical florbetapir retention during the acute phase between patients with CAA-related lobar intracerebral hemorrhage (ICH) and patients with hypertension-related deep ICH.Methods:Patients with acute CAA-related lobar ICH were prospectively enrolled and compared with patients with deep ICH. 18F-florbetapir PET, brain MRI, and APOE genotype were obtained for all participants. Cortical florbetapir standard uptake value ratio (SUVr) was calculated with the whole cerebellum used as a reference. Patients with CAA and those with deep ICH were compared for mean cortical florbetapir SUVr values.Results:Fifteen patients with acute lobar ICH fulfilling the modified Boston criteria for probable CAA (mean age = 67 ± 12 years) and 18 patients with acute deep ICH (mean age = 63 ± 11 years) were enrolled. Mean global cortical florbetapir SUVr was significantly higher among patients with CAA-related ICH than among patients with deep ICH (1.27 ± 0.12 vs 1.12 ± 0.12, p = 0.001). Cortical florbetapir SUVr differentiated patients with CAA-ICH from those with deep ICH (area under the curve = 0.811; 95% confidence interval [CI] 0.642–0.980) with a sensitivity of 0.733 (95% CI 0.475–0.893) and a specificity of 0.833 (95% CI 0.598–0.948).Conclusions:Cortical florbetapir uptake is increased in patients with CAA-related ICH relative to those with deep ICH. Although 18F-florbetapir PET can label vascular β-amyloid and might serve as an outcome marker in future clinical trials, its diagnostic value in acute CAA-related ICH seems limited in clinical practice.

Introduction Une atteinte corticale, invisible a l’œil nu, a ete demontree par des techniques de traitement d’image dans les demences vasculaires sous-corticales liees aux maladies des petits vaisseaux cerebraux (MPVC). Objectifs Determiner s’il existe des lesions corticales macroscopiques, visibles en IRM conventionnelle, dans CADASIL, une forme pure de MPVC affectant des sujets jeunes. Patients et methodes Les rubans corticaux de 28 patients CADASIL et 29 controles apparies ont ete etudies systematiquement a la recherche de lesions focales a partir d’IRM 3DT1. Des techniques de traitement d’images validees avaient ete prealablement utilisees par un expert independant pour masquer la substance blanche afin de permettre une lecture en aveugle du statut du sujet. Cette approche etait indispensable en l’absence de donnees sur la prevalence de telles lesions en population generale. Resultats Des lesions macroscopiques corticales focales ont ete identifiees chez 3/28 patients (11 %) et aucune chez les controles. Ces lesions corticales correspondaient a un amincissement du ruban cortical pouvant aller jusqu’a une rupture complete impliquant le cortex sur toute sa hauteur. Ces lesions etaient situees en regard de lesions severes de la substance blanche sous jacente. Des donnees d’IRM a 7 Tesla ont montre un hyposignal lineaire sous certaines lesions suggerant des processus de degenerescence secondaire. Discussion Le recours a une lecture en aveugle a permis de s’assurer du caractere specifique de ces lesions apparaissant donc liees a CADASIL, chez des patients indemnes de facteur de risque cardiovasculaire. Si certains elements suggerent des phenomenes de degenerescence secondaire, l’atteinte du cortex sur toute sa hauteur implique d’autres mecanismes tels qu’une vulnerabilite locale du ruban cortical. Conclusion La mise en evidence de lesions corticales visibles en IRM conventionnelle dans un modele pur de MPVC souligne le role du cortex cerebral dans des affections longtemps considerees « sous-corticales ». Informations complementaires Financements particuliers : AP–HP et CEA (annee d’interface), reseau Leducq, ERANET Neuron.