JC Virus-Related Retinopathy.

Importance: Reactivation of the JC polyomavirus in individuals with impaired immunity is most commonly associated with progressive multifocal leukoencephalopathy. We report a case of polyomavirus-related retinopathy, representing a potentially novel manifestation of JC polyomavirus infection.

Objective: To describe a patient with common variable immunodeficiency who exhibited isolated unilateral retinopathy related to JC polyomavirus infection of the retinal ganglion cell layer.

Balloon angioplasty alone in carotid artery dissections: an overlooked therapeutic choice in acute ischemic stroke.

Background & Purpose: Carotid dissection is a major cause of ischemic stroke in young patients and its endovascular management is complex and lacks consensus. Our aim was to carry out the first comparative evaluation of balloon angioplasty alone versus stenting in the endovascular treatment of acute carotid artery dissection presenting with acute stroke.

Materials And Methods: From the national ETIS (Endovascular Treatment of Ischemic Stroke) registry, between January 2015 and 2023, we included patients with acute ischemic stroke secondary to internal carotid artery dissection who underwent endovascular treatment.

Structural Alterations Associated With Cardiovascular Autonomic Failure in Multiple System Atrophy.

Background: Early severe autonomic failure (AF) in multiple system atrophy (MSA) is a risk factor for poor survival. Postmortem studies suggested that AF is related to the degeneration of preganglionic autonomic neurons of the brainstem and spinal cord.

Objectives: Characterize cerebral alterations on brain imaging associated with cardiovascular AF.

An insight from the default mode network in patients with amnesia following left thalamic infarction involving the mediodorsal nucleus and mammillothalamic tract.

The role of the medial part of the thalamus, and in particular the mediodorsal nucleus (MD) and the mammillothalamic tract (MTT), in memory has long been studied, but their contribution remains unclear. While the main functional hypothesis regarding the MTT focuses on memory, some authors postulate that the MD plays a supervisory executive role (indirectly affecting memory retrieval) due to its dense structural connectivity with the prefrontal cortex (PFC). Recently, it has been proposed that the MD, MTT and PFC form part of the DMN the default mode network (DMN).

Cerebrotendinous xanthomatosis: a literature review and case study.

Cerebrotendinous xanthomatosis (CTX) is a rare but treatable inherited neurometabolic disorder that can lead to severe sequelae if left untreated. Chenodeoxycholic acid is a safe and effective treatment for CTX. Early diagnosis is essential to improve patient outcomes.

How to distinguish spinocerebellar ataxia 27B from late onset cerebellar ataxia: insights from a case-control study.

Background: Spinocerebellar ataxia 27B is the most common genetic late onset cerebellar ataxia (LOCA). However, it commonly overlaps with other genetic LOCA as with the cerebellar form of multiple system atrophy (MSA-C).

Objectives: To pinpoint which clinical signs and symptoms best discriminate between FGF14 + from FGF14 - patients at symptoms' onset.

Blood-brain barrier profile pretreatment is associated with hemorrhagic transformation after endovascular reperfusion.

Background: While advances in endovascular thrombectomy (EVT) have led to high reperfusion rates, most patients treated with EVT do not avoid disability. Post-reperfusion hemorrhagic transformation (HT) is a potential target for improving outcomes. This study examined pretreatment blood-brain barrier (BBB) disruption in tissue that would subsequently become part of the final infarct to evaluate its role in post-EVT HT.

Prevalence and characteristics of vascular cognitive impairment in a European cohort of adult patients with Moyamoya angiopathy.

Introduction: Moyamoya angiopathy (MMA) is associated with a high risk of stroke, but it is also increasingly recognized as leading to cognitive impairment. The aim of this study was to determine the prevalence, nature, and severity of vascular cognitive impairment no dementia (VCIND) in adults with MMA and to identify clinical and imaging factors associated with VCIND.

Methods: We conducted a retrospective study of consecutive adult patients with MMA followed in two tertiary hospitals (Toulouse and Paris Lariboisiere).

Exploring the impact of the interthalamic adhesion on human cognition: insights from healthy subjects and thalamic stroke patients.

The interthalamic adhesion (IA) is a structure that connects the median borders of both thalami. Its anatomical variants and functions remain poorly studied. The main objective of this study was to explore the role of the IA on cognition.

MRI hypoperfusion as a determinant of cognitive impairment in adults with Moyamoya angiopathy.

Introduction: In Moyamoya angiopathy (MMA), mechanisms underlying cognitive impairment remain debated. We aimed to assess the association of cognitive impairment with the degree and the topography of cerebral hypoperfusion in MMA.

Methods: A retrospective analysis of neuropsychological and perfusion MRI data from adults with MMA was performed.

Cerebrovascular and cardiovascular autonomic regulation in sickle cell patients with white matter lesions.

Background And Purpose: White matter lesions (WMLs) are frequent in sickle cell disease (SCD), with a prevalence described to be as high as 53% by age 30. Cerebrovascular regulation and cardiovascular autonomic regulation, more specifically the sympatho-vagal balance, can be altered in SCD. In this study the association between WMLs, cerebrovascular regulation and sympatho-vagal balance was assessed in SCD patients.

Immunohistologic Features of Cerebral Venous Thrombosis Due to Vaccine-Induced Immune Thrombotic Thrombocytopenia.

Objectives: Vaccine-induced immune thrombotic thrombocytopenia (VITT), a recently described entity characterized by thrombosis at unusual locations such as cerebral venous sinus and splanchnic vein, has been rarely described after adenoviral-encoded COVID-19 vaccines. In this study, we report the immunohistological correlates in 3 fatal cases of cerebral venous thrombosis related to VITT analyzed at an academic medical center.

Methods: Detailed neuropathologic studies were performed in 3 cases of cerebral venous thrombosis related to VITT after adenoviral COVID-19 vaccination.

The Use of Intracranial Vessel Wall Imaging in Clinical Practice.

Three-dimensional vessel wall MR imaging has gained popularity in the diagnosis and management of patients with cerebrovascular disease in clinical practice. Vessel wall MR imaging is an imaging technique that delivers a fundamentally different viewpoint by emphasizing on the pathology of the vessel wall as opposed to traditional descriptions that focus on the vessel lumen. It shows a crucial power in detecting vessel wall changes in patients with diseases including, but not limited to, central nervous system vasculitis, moyamoya disease, aneurysms, dissections, and intracranial atherosclerotic disease.

Specific profiles of new-onset vs. non-inaugural status epilepticus: From diagnosis to 1-year outcome.

While new-onset status epilepticus (NOSE) is a harbinger of chronic epilepsy, prospective medical data are sparse in terms of specifying whether the evolution of status epilepticus (SE) and seizure expression in NOSE resembles what occurs in patients who have already been diagnosed with epilepsy [non-inaugural SE (NISE)] in all aspects apart from its inaugural nature. The aim of this study was to compare the clinical, MRI, and EEG features that could distinguish NOSE from NISE. We conducted a prospective monocentric study in which all patients ≥18 years admitted for SE over a 6-month period were included.

CNS tumor with EP300::BCOR fusion: discussing its prevalence in adult population.

The Central Nervous System (CNS) tumor with BCOR internal tandem duplication (ITD) has recently been added as a novel embryonal histomolecular tumor type to the 2021 World Health Organization (WHO) Classification of CNS Tumors. In addition, other CNS tumors harboring a BCOR/BCORL1 fusion, which are defined by a distinct DNA-methylation profile, have been recently identified in the literature but clinical, radiological and histopathological data remain scarce. Herein, we present two adult cases of CNS tumors with EP300::BCOR fusion.

Diagnostic imaging strategies of acute intracerebral hemorrhage in European academic hospitals-a decision-making analysis.

Purpose: To evaluate and compare which factors are relevant to the diagnostic decision-making and imaging workup of intracerebral hemorrhages in large, specialized European centers.

Methods: Expert neuroradiologists from ten large, specialized centers (where endovascular stroke treatment is routinely performed) in nine European countries were selected in cooperation with the European Society of Neuroradiology (ESNR). The experts were asked to describe how and when they would investigate specific causes in a patient who presented with an acute, atraumatic, intracerebral hemorrhage for two given locations: (1) basal ganglia, thalamus, pons or cerebellum; (2) lobar hemorrhage.