Post-infectious central nervous system vasculitides in adults: an underdiagnosed and treatable disease : Part I. Overview.

Introduction: The differential diagnosis of Primary Central Nervous System Angiitis (PACNS) is complex and includes several inflammatory and non-inflammatory conditions. Among the first ones, post-infectious CNS vasculitides represent a relevant topic and they are often underdiagnosed.

Aims: The main aim of this review is to summarize the clinical and neuroimaging features of post-infectious vasculitides, highlighting the diagnostic clues and the need to carefully consider them in the differential diagnosis of PACNS.

Anti-neutrophil cytoplasmic antibody-associated central nervous system vasculitis mimicking brain tumor: A case report.

Here, we report a case of antineutrophil cytoplasmic antibody (ANCA)-associated central nervous system (CNS) vasculitis that mimicked a brain tumor. The patient presented with progressive right upper arm weakness. Brain magnetic resonance imaging (MRI) revealed large tumor-like lesions in the left frontal and parietal lobes, with patchy and irregular enhancement with gadolinium and edema.

Primary CNS Vasculitis - A Focussed Review on Treatment.

Primary central nervous system vasculitis (PCNSV) is a rare and complex disease that poses formidable diagnostic and therapeutic challenges. Since its initial recognition as a distinct clinical entity in the 1950s, there has been considerable advancement in our understanding of PCNSV histopathology, specific clinical subsets, and their response to treatment. However, PCNSV is one of the rarest vasculitides, and still remains a challenging diagnosis with many unanswered questions regarding optimal management.

Myelin oligodendrocyte glycoprotein antibody-associated disease with histopathologic features of primary CNS angiitis without demyelination: Case report and literature review.

Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease that affects both small- and medium-sized vessels of the CNS, while myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a novel antibody-mediated inflammatory demyelinating disorder that causes damage to the myelin in CNS. We report a case diagnosed as MOGAD due to a history of recurrent myelitis, brain lesions, and positive anti-MOG, but the brain biopsy showed vasculitis without demyelination.

Prognostic Factors and Outcome Measures After Rituximab Therapy in Central Nervous System Vasculitis: A Systematic Review.

Given the growing popularity of Rituximab (Rmab) treatment as a potential substitute for cyclophosphamide, we conducted this review to determine aspects related to Rmab therapy in central nervous system vasculitis (CNSV) patients, aiming to establish both the beneficial and detrimental consequences of Rmab while providing clinical guidance for managing patients' conditions. This systematic review was prepared following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The PubMed, Web of Science, and Scopus databases were utilized to investigate Rmab treatment in CNSV from January 2015 to May 2024.

Pearls & Oy-sters: Tumour-Like Mass Lesion Secondary to Primary CNS Vasculitis.

Primary CNS vasculitis (PCNSV) is uncommonly considered in the differential diagnosis of tumor-like lesions. This case report of tumefactive PCNSV highlights imaging features that should increase clinical suspicion for CNS vasculitis, potentially lending to earlier diagnosis and treatment. A 62-year-old man presented with a 1-month history of focal motor seizures and cortical sensory loss localizing to the right frontoparietal lobe.

Unexpected Strikes: Sjögren Syndrome Triggering Multiple Evolving Cerebral Strokes in a Young Patient.

Neurological complications are observed less frequently with primary Sjögren syndrome (SS). The central nervous system (CNS) has seldom been shown to exhibit symptoms of SS, making the diagnosis of SS with neurological involvement difficult. We present a rare case scenario in which a young 23-year-old male presenting with an acute history of fever, headache, vomiting, altered sensorium, and seizures was admitted and diagnosed as a sub-acute infarct in the right frontal-parietal-temporal lobes on a computed tomography (CT) scan.

Occult Amyloid-β-Related Angiitis: Neuroimaging Findings at 1.5T, 3T, and 7T MRI.

Cerebral amyloid angiopathy (CAA) is a progressive neurodegenerative small vessel disease that is associated with intracranial hemorrhage and cognitive impairment in the elderly. The clinical and radiographic presentations have many overlapping features with vascular cognitive impairment, hemorrhagic stroke, and Alzheimer disease (AD). Amyloid-β-related angiitis (ABRA) is a form of primary CNS vasculitis linked to CAA, with the development of spontaneous autoimmune inflammation against amyloid in the vessel wall with resultant vasculitis.

Primary Angiitis of the CNS: Differences in the Profile Between Subtypes and Outcomes From an Indian Cohort.

Background And Objectives: Primary angiitis of the CNS (PACNS) is a rare disease that has significant morbidity and mortality. Subtypes of PACNS can have different presentations that could be missed with certain diagnostic modalities, further increasing diagnostic complexity. We sought to distinguish the subtypes of PACNS and describe their outcomes in an Indian cohort.

Predictive Value of Clinical, CSF and Vessel Wall MRI Variables in Diagnosing Primary Angiitis of the CNS.

Background And Objectives: Without brain biopsy, there are limited diagnostic predictors to differentiate primary angiitis of the CNS (PACNS) from intracranial atherosclerotic disease (ICAD). We examined the utility of clinical, CSF, and quantitative vessel wall magnetic resonance imaging (VWMRI) variables in predicting PACNS from ICAD.

Methods: In this cross-sectional design, observational study, we reviewed electronic medical records to identify patients (18 years and older) who presented to our medical center between January 2015 and December 2021 for ischemic stroke due to intracranial vasculopathy.

Neurovascular Adverse Effects of Sars-Cov-2 Vaccination.

The global deployment of SARS-CoV-2 vaccines has been pivotal in curbing the COVID-19 pandemic, reducing morbidity and mortality associated with the virus. While most of these vaccines have demonstrated high efficacy and overall safety, emerging reports have highlighted potential neurovascular adverse effects, albeit uncommon, associated with these vaccinations. This review aims to assess and summarize the current knowledge on the neurovascular complications arising post-SARS-CoV-2 vaccination.

Primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (CNS) is an uncommon inflammatory disorder, with highly variable clinical presentation. It needs to be differentiated from several mimickers, such as CNS involvement in systemic vasculitides, connective tissue disorders, infectious disease, and leukodystrophy as well as neoplastic diseases. The diagnosis requires a combination of clinical and laboratory investigations, multimodal imaging, and histopathological examination, which should be available for confirmation.

The Contribution of Vessel Wall Magnetic Resonance Imaging to the Diagnosis of Primary and Secondary Central Nervous System Vasculitis.

Background: To describe high-resolution brain vessel wall MRI (VW-MRI) patterns and morphological brain findings in central nervous system (CNS) vasculitis patients.

Methods: Fourteen patients with confirmed CNS Vasculitis from two tertiary centers underwent VW-MRI using a 3T scanner. The images were reviewed by two neuroradiologists to assess vessel wall enhancement characteristics and locations.

Radiologic Lag and Brain MRI Lesion Dynamics During Attacks in MOG Antibody-Associated Disease.

Background And Objectives: Knowledge of the evolution of CNS demyelinating lesions within attacks could assist diagnosis. We evaluated intra-attack lesion dynamics in patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) vs multiple sclerosis (MS) and aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorder (AQP4+NMOSD).

Methods: This retrospective observational multicenter study included consecutive patients from Mayo Clinic (USA) and Great Ormond Street Hospital for Children (UK).

Cerebral large vessels vasculitis following Guillain-Barré syndrome as first clinical manifestations of primary Sjogren's syndrome: A case based - Review.

Background: Primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy in which extraglandular signs of pSS are determinant for the prognosis. Involvement of both peripheral and central nervous system (CNS) are known to be among the sites of high systemic activity in pSS.

Case Presentation: We, herein, report a case of a 57-year-old female patient with pSS presenting with typical (GBS), shortly followed by acute headaches accompanied by cortical blindness.

Comprehensive Therapeutic Approaches to Tuberculous Meningitis: Pharmacokinetics, Combined Dosing, and Advanced Intrathecal Therapies.

Tuberculous meningitis (TBM) presents a critical neurologic emergency characterized by high mortality and morbidity rates, necessitating immediate therapeutic intervention, often ahead of definitive microbiological and molecular diagnoses. The primary hurdle in effective TBM treatment is the blood-brain barrier (BBB), which significantly restricts the delivery of anti-tuberculous medications to the central nervous system (CNS), leading to subtherapeutic drug levels and poor treatment outcomes. The standard regimen for initial TBM treatment frequently falls short, followed by adverse side effects, vasculitis, and hydrocephalus, driving the condition toward a refractory state.

Vasculitis in the Central Nervous System: Etiology, Characteristics, and Outcomes in a Large Single-Center Cohort.

Background And Purpose: For the management of central nervous system (CNS) vasculitis, it is crucial to differentiate between primary and secondary CNS vasculitis and to understand the respective etiologies. We assessed the etiology, characteristics, and outcomes of patients with CNS vasculitis.

Methods: A single-center retrospective chart review was conducted at the University of Utah, Department of Neurology, between February 2011 and October 2022.

Cortical Microhemorrhage Presentation of Small Vessel Primary Angiitis of the Central Nervous System.

Objective: Primary angiitis of the central nervous system (PACNS) is a rare vasculitis restricted to the brain, spinal cord, and leptomeninges. This study aimed to describe the imaging characteristics of patients with small vessel PACNS (SV-PACNS) using 7 T magnetic resonance imaging (MRI).

Methods: This ongoing prospective observational cohort study included patients who met the Calabrese and Mallek criteria and underwent 7 T MRI scan.