Calabrese and cns vasculitis

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. The MRI protocol includes T1-weighted magnetization-prepared rapid gradient echo imaging, T2 star weighted imaging, and susceptibility-weighted imaging...

INTRODUCTION: Primary central nervous system vasculitis (PCNSV) is a rare disease affecting medium- and small-calibre blood vessels of the central nervous system. OBJECTIVE: The aim of this study was to analyse clinical findings and diagnostic aspects, with special attention to histopathological findings, as well as the treatments used and treatment response in patients diagnosed with PCNSV at our hospital. PATIENTS AND METHODS: We conducted a retrospective descriptive analysis of patients with a diagnosis of PCNSV at discharge from our centre and meeting the 1988 Calabrese criteria...

OBJECTIVES: To determine the diagnostic accuracy for high-resolution vessel wall image (HR-VWI) and brain biopsy according to angiographical classification in patients with primary central nervous system vasculitis (PCNSV). METHODS: We extracted the patients with PCNSV who underwent the complete brain MRI protocol and cerebral vascular image from Cleveland Clinic prospective CNS vasculopathy Bioregistry. The large-medium vessel variant (LMVV) was defined as patients with cerebral vasculature indicating vasculitis in proximal or middle arterial segments, whereas vessel involvements in smaller distal branches or normal angiography were considered as the small vessel variant (SVV)...

To observe the clinical and angiographic effectiveness of mycophenolate mofetil (MMF) as induction and maintenance immunosuppressive therapy in primary central nervous system vasculitis (PCNSV). In this open-label prospective study done at a tertiary care neurology centre, adult patients with PCNSV, diagnosed by Calabrese's criteria, were recruited from 2017 to 2021 and treated with glucocorticoids, MMF and standard of care. Patients were followed-up and clinical and angiographic changes were recorded. Total 26 patients were recruited with median age 39 years (34-49) with a slight female predilection (61...

OBJECTIVES: High-resolution vessel wall imaging (HR-VWI) often demonstrates strong and concentric vessel wall enhancement (VWE) in patients with central nervous system vasculitis (CNS-V). However, little is known about follow-up VWE characteristics and monitoring the response to treatments. The aim of this study was to investigate serial VWE patterns and its clinical practice through the management of CNS-V. METHODS: We extracted 9 patients with diagnosed of CNS-V who underwent serial HR-VWI (baseline, 1st follow-up, and 2nd follow-up) from Cleveland Clinic CNS vasculopathy registry...

Familial Mediterranean fever (FMF) is a genetic autoinflammatory disease with autosomal recessive transmission, characterized by periodic fever attacks with self-limited serositis. Secondary amyloidosis due to amyloid A renal deposition represents the most fearsome complication in up to 8.6% of patients. Amyloidosis A typically reveals a nephrotic syndrome with a rapid progression to end-stage kidney disease still. It may also involve the cardiovascular system, the gastrointestinal tract and the central nervous system...

OBJECTIVES: Neurosarcoidosis (NS) and primary angiitis of central nervous system (PACNS) are inflammatory diseases affecting central nervous system, with overlapping clinical and pathological characteristics. Distinguishing these diseases is important given distinct therapeutic implications. In this study, we aimed to compare demographic, CSF and MRI characteristics between these two conditions. METHODS: All the clinical, CSF and laboratory characteristics at the time of presentation were retrieved from electronic medical records...

PURPOSE OF REVIEW: The main purpose of this review is to present advances in diagnostics of central nervous system vasculitis (CNS-V). RECENT FINDINGS: Progress in molecular technologies and neuroimaging have added formidably to our knowledge of CNS-V. Next-generation sequencing has the promise to enhance our ability to diagnose, interrogate, and track infectious diseases, making this test attractive and capable of avoiding brain biopsy in cases where CNS infections are suspected...

OBJECTIVE: Primary CNS Vasculitis (PCNSV) is a rare disease that is often challenging to diagnose. Cerebral angiography and biopsy have been utilized in the diagnostic workup for several decades but limited literature reports on the concordance of findings of angiography and biopsy. The primary objective of this work was to examine how cerebral angiography corresponded with biopsy findings in patients with suspected PCNSV. PATIENTS AND METHODS: A total of 128 patients who underwent workup for PCNSV between years 2005-2016 were identified by query of existing neurological surgery and angiography databases at University Hospitals Cleveland Medical Center (UHCMC) and the Cleveland Clinic Foundation (CCF)...

OBJECTIVE: To identify molecular correlates of primary angiitis of the CNS (PACNS) through proteomic analysis of CSF from a biopsy-proven patient cohort. METHODS: Using mass spectrometry, we quantitatively compared the CSF proteome of patients with biopsy-proven PACNS (n = 8) to CSF from individuals with noninflammatory conditions (n = 11). Significantly enriched molecular pathways were identified with a gene ontology workflow, and high confidence hits within enriched pathways (fold change >1...

OBJECTIVES: Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the brain and spinal cord, which often presents with severe cognitive and functional deficits. Despite progress in diagnosis, little is still known about long-term outcomes. Our aim was to evaluate long-term functional capabilities, quality of life, and depression, and to determine the effect of treatment duration on patient outcomes. METHODS: We identified patients by ICD-9 codes for cerebral angiitis, and included them if they met two of the three following criteria: inflammatory cerebrospinal fluid (CSF), cerebral angiogram typical of vasculitis, or findings of vasculitis on pathologic examination of brain tissue...

PURPOSE OF REVIEW: The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis. RECENT FINDINGS: Challenges in diagnosis of CNS vasculitis still exist due to the broad differential diagnosis and generally nonspecific initial clinical manifestations. Differentiation between primary angiitis of the CNS (PACNS) and secondary causes is important in guiding management. Recent longitudinal cohort studies have improved our understanding of PACNS...

Importance: Identifying infectious causes of subacute or chronic meningitis can be challenging. Enhanced, unbiased diagnostic approaches are needed. Objective: To present a case series of patients with diagnostically challenging subacute or chronic meningitis using metagenomic next-generation sequencing (mNGS) of cerebrospinal fluid (CSF) supported by a statistical framework generated from mNGS of control samples from the environment and from patients who were noninfectious...

BACKGROUND AND PURPOSE: High-resolution MR imaging is an emerging tool for evaluating intracranial artery disease. It has an advantage of defining vessel wall characteristics of intracranial vascular diseases. We investigated high-resolution MR imaging arterial wall characteristics of CNS vasculitis and reversible cerebral vasoconstriction syndrome to determine wall pattern changes during a follow-up period. MATERIALS AND METHODS: We retrospectively reviewed 3T-high-resolution MR imaging vessel wall studies performed on 26 patients with a confirmed diagnosis of CNS vasculitis and reversible cerebral vasoconstriction syndrome during a follow-up period...

Central nervous system vasculitis is one of the foremost diagnostic challenges in rheumatology. It results in inflammation and destruction of the vasculature within the CNS. When vasculitis is confined to brain, meninges or spinal cord, it is referred to as primary angiitis of the CNS. Secondary CNS vasculitis occurs in the setting of a systemic vasculitis, auto-inflammatory or infectious disease. Prompt and accurate diagnosis of CNS vasculitis is essential to prevent irreversible brain damage, and to secure precise treatment decisions...

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Despite the multiple areas of progress that have been made in our understanding of primary angiitis of the central nervous system (PACNS ), many challenges exist when facing this diagnosis. This review will address the clinical presentation, diagnosis and differential diagnosis of PACNS, with emphasis on the recognized advancement in the field. Appreciation of different mimics especially the recognition of reversible cerebral vasoconstriction syndromes (RCVS) as a major mimic will be addressed. Finally, approach to treatment will be summarized...

Meaningful progress in our understanding and clinical approach to primary angiitis of the CNS (PACNS) has been made in the past three decades. Increased recognition of PACNS and general advances in diagnosis of neurological disorders have led to an aggressive diagnostic approach and a proliferation of case reports providing enriched clinical and pathological descriptions. We have witnessed major advances not only in the diagnosis of PACNS but in the recognition of its mimics. Epidemiological, clinical, neuroradiagnostic, and laboratory findings have enhanced our diagnostic accuracy and recognition of PACNS mimics, however, many challenges to our understanding and management of the disease in children and adults remain...

PURPOSE OF REVIEW: In the past decade, primary and secondary central nervous system (CNS) vasculitides have been more commonly diagnosed and recognized than previously. With the increasing awareness of these disorders, it is crucial for the treating physician to differentiate between causes of CNS vasculitis and to recognize their marked clinical and pathophysiological heterogeneity. This review focuses on the major forms of primary CNS vasculitis, as well as secondary CNS vasculitis with emphasis on their clinical findings, diagnoses, and treatment...

In the diagnosis of primary central nervous system (CNS) vasculitis, it is crucial to rule out clinical, angiographic, and pathological mimics. We report a case of arteriovenous malformation (AVM) mimicking primary CNS vasculitis. A young male presented with intracerebral haemorrhage and no other clinical, laboratory, or angiographic features suggesting vasculitis. Cerebral biopsy showed perivascular inflammation and slight infiltration of the muscular layer of cerebral vessels by chronic inflammatory cells close to the haemorrhagic areas...