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Objective Methods Results Interpretation Intravascular lymphoma is a rare subtype of B‐cell lymphoma characterized by a clonal proliferation restricted to the lumen of small vessels. Over 50% of patients exhibit central nervous system (CNS) involvement, but diagnosis is often delayed due to the lack of distinctive features. We aimed to identify key phenotypic features for early diagnosis of intravascular lymphoma with CNS involvement through an in‐depth cohort study.We built up a multicenter retrospective cohort of 17 patients recruited in collaboration with the French Expert Network for Oculo‐Cerebral Lymphomas (LOC network), and retrospectively analyzed data from medical records.In this cohort, 15 of 17 (88%) patients developed focal neurological episodes, often fluctuating and/or recurrent, with a sudden onset in 68% of episodes, suggesting a vascular origin. Rapid cognitive deterioration occurred in 15 of 17 (88%) patients, psychiatric manifestations in 8 of 17 (47%), and “B signs” in 14 of 17 (82%). Brain MRI showed polymorphic FLAIR hyperintensities in 14 of 16 (87%) patients, and DWI‐positive lesions in 13 of 16 (81%) of patients, which accumulated over time and had unusual characteristics for ischemic lesions (progressive growth, persistent DWI‐hyperintensity over 1 month, surrounded by a wider FLAIR hyperintensity). Early‐onset inflammatory syndrome, and elevated lactate dehydrogenase (LDH) levels were observed in over 90% of cases. Mild and inconsistent meningitis contrasted with a nearly‐constant hyperproteinorachia. An increased interleukin 10/6 ratio over 0,7 was found in 4 of 7 (57%) patients, and skin biopsy led to a pathological diagnosis in 3 of 6 (50%) patients.The results of this study highlight “red flags” that could help accelerate the diagnosis of intravascular lymphoma involving the CNS. ANN NEUROL 2024 [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Because delayed post-contrast three-dimensional fluid-attenuated inversion recovery imaging sequences enable the distinction between the utricle and the saccule, we raised the hypothesis that patients with vestibular atelectasis (VA) could show unilateral collapse of the utricle and the ampullas on imaging.Study Design: Retrospective case series.Methods: We retrospectively reviewed 200 patients who underwent 3 T magnetic resonance imaging (MRI) after intravenous administration of gadolinium. MRI scans were assessed for the presence of VA. The endolymphatic space was considered as collapsed when the utricle and at least two ampullas were not visible or were barely visible.Results: We reported four patients with VA on MRI responsible for atypical clinical presentations of acute vestibular deficit. All patients presented a specific involvement of the pars superior sensory captors (utricle, ampullas), preserving the pars inferior sensory captors (cochlea and saccule). This was confirmed both clinically and on MRI.Conclusions: Our study is the first to describe in vivo unilateral collapse of the pars superior on delayed postcontrast MRI in patients with a clinical unilateral vestibular loss.Level Of Evidence: 4 Laryngoscope, 129:1689-1695, 2019. [ABSTRACT FROM AUTHOR]

Background and Purpose: By studying the evolution of brain volume across the life span in male and female patients, we aimed to understand how sex, brain volume, and the epidermal growth factor repeat domain of the mutation, the 3 major determinants of disability in CADASIL, interact in driving disease evolution., Materials and Methods: We used validated methods to model the evolution of normalized brain volume with age in male and female patients using nonparametric regression in a large, monocentric cohort with prospectively collected clinical and high-resolution MR imaging data. We used k-means clustering to test for the presence of different clinical course profiles., Results: We included 229 patients (mean age, 53 [SD, 12] years; 130 women). Brain volume was larger in women (mean size, 1024 [SD, 62] cm 3 versus 979 [SD, 50] cm 3 ; P  < .001) and decreased regularly. In men, the relationship between brain volume and age unexpectedly suggested an increase in brain volume around midlife. Cluster analyses showed that this finding was related to the presence of a group of older male patients with milder symptoms and larger brain volumes, similar to findings of age-matched women. This group did not show specific epidermal growth factor repeat domain distribution., Conclusions: Our results demonstrate a detrimental effect of male sex on brain volume throughout life in CADASIL. We identified a subgroup of male patients whose brain volume and clinical outcomes were similar to those of age-matched women. They did not have a specific distribution of the epidermal growth factor repeat domain, suggesting that yet-unidentified predictors may interact with sex and brain volume in driving disease evolution., (© 2022 by American Journal of Neuroradiology.)