primary angiitis of the central nervous system

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is most commonly characterized by focal or diffuse severe thunderclap headache with or without focal neurological deficits and associated transient focal vasoconstriction of the intracerebral arteries lasting up to three months. We present six patients diagnosed as RCVS, with three patients presenting with focal neurological deficits without headache and the remaining three with severe headache alone. Neuroimaging revealed focal subarachnoid bleed with or without segmental intracerebral vasospasm, which resolved over three months...

Primary angiitis of the central nervous system (PACNS) is a rare and severe disease confined to the central nervous system, i.e., the brain and spinal cord. The etiology, pathogenesis and immune mechanism of PACNS have not yet been completely elucidated. The diagnosis is challenging; it is based upon constellation of clinical picture, cerebrospinal fluid analysis, imaging methods or tissue biopsy as the gold standard. In differential diagnosis of PACNS, it is necessary to rule out infectious, malignant or systemic inflammatory diseases, as well as reversible cerebral vasoconstriction syndrome...

Primary Angiitis of the Central Nervous System (PACNS) is a rare disease and its diagnosis is a challenge for several reasons, including the lack of specificity of the main findings highlighted in the current diagnostic criteria. Among the neuroimaging pattern of PACNS, a tumefactive form (t-PACNS) is a rare subtype and its differential diagnosis mainly relies on neuroimaging. Tumor-like mass lesions in the brain are a heterogeneous category including tumors (in particular, primary brain tumors such as glial tumors and lymphoma), inflammatory (e...

Primary Angiitis of the Central Nervous System (PACNS) is a rare cerebrovascular disease involving the arteries of the leptomeninges, brain and spinal cord. Its diagnosis can be challenging, and the current diagnostic criteria show several limitations. Among the clinical and neuroimaging manifestations of PACNS, intracranial bleeding, particularly intracerebral hemorrhage (ICH), is poorly described in the available literature, and it is considered infrequent. This review aims to summarize the available data addressing this issue with a dedicated focus on the clinical, neuroradiological and neuropathological perspectives...

Primary angiitis of central nervous system (PACNS) is a rare idiopathic vasculitis that typically involves small arteries. An 18-year-old woman was operated on for resection of a ruptured aneurysm in a cerebral artery. Multiple aneurysms of cerebral arteries had been detected by neuroimaging examinations since the age of 12, and she had been administered drugs following a diagnosis of PACNS since the age of 15. The resected aneurysm was a ruptured saccular aneurysm occurring in a medium-sized artery. Histologically, necrotizing arteritis of the polyarteritis nodosa (PAN) type was noted in the aneurysmal wall...

Primary Angiitis of the Central Nervous System (PACNS) is an uncommon disease with kaleidoscopic clinical manifestations. Ischemic strokes are commoner than their hemorrhagic counterpart. Intracranial pseudoaneurysms are rarely reported in PACNS cohorts. We hereby describe the case of a 39-year-old female, who presented for evaluation of acute onset of left middle cerebral artery (MCA) ischemic stroke, with cerebral angiogram showing multifocal stenosis and irregularities in intracranial blood vessels with an aneurysm arising from the lenticulostriate branch of the left MCA M1 segment...

INTRODUCTION: Primary angiitis of the central nervous system (PACNS) is an uncommon inflammatory condition that exclusively affects blood vessels within the brain parenchyma, leptomeninges, and spinal cord. Due to its infrequency and the variability in its clinical presentation and imaging findings, diagnosing PACNS can be challenging. CASE DESCRIPTION: In this study, we present the case of a teenager who initially presented with headaches and epilepsy. Comprehensive laboratory tests yield normal results...

Primary angiitis of the central nervous system (PACNS) is a rare vasculitis in the central nervous system. Herein, we report a case of diagnosis and treatment of necrotic pattern PACNS, which was difficult to differentiate from a brain abscess. A 19-year-old male presented with blurred vision and a headache. Brain MRI revealed irregular rim-enhancing necrotic masses with central diffusion-high signal intensity in the corpus callosum and peripheral diffusion-high signal intensity in the left parietotemporal periventricular area...

A 79-year-old woman presented with difficulty walking and disturbance of consciousness. Magnetic resonance imaging revealed diffuse white matter lesions and abnormal signals along the surface of the brain and sulci. A brain biopsy revealed granulomatous vasculitis with eosinophil infiltration. There was no peripheral blood eosinophilia or evidence of angiitis in other organs, and primary angiitis of the central nervous system (PACNS) with pathological findings of eosinophilic granulomatosis with polyangiitis (EGPA) was diagnosed...

PURPOSE: Primary angiitis of the adult central nervous system (PACNS) is an increasingly recognized but limited disease. Using previous case reports, we sought to summarize the clinical symptoms, imaging manifestations, treatment, and prognosis of patients with biopsy-confirmed PACNS to guide clinical diagnosis and management. METHODS: We searched the Web of Science database for studies published from January 2000 to April 2023, with the language set to English and the document type limited to [Article or Review or Letter or Editorial Material]...

Primary angiitis of the central nervous system (PACNS) is a rare and potentially severe form of vasculitis that is limited to the brain, spinal cord, and meninges. Despite extensive research, the etiology and underlying immunologic mechanisms of PACNS remain largely unknown. PACNS presents with a variety of clinical, radiological, and pathologic features, but it is generally characterized by inflammation and destruction of the walls of blood vessels in the CNS, which can lead to tissue ischemia and/or hemorrhage...

Primary central nervous system vasculitis (PCNSV) is a vasculitis limited to the brain and spinal cord. Induction therapy often consists of steroids and cyclophosphamide. Maintenance therapy includes a prednisone taper and may be combined with medications such as azathioprine or mycophenolate mofetil. Relapse is common in PCNSV and an increased dose of steroids is often given, sometimes with a change in therapy. Medications such as rituximab and mycophenolate mofetil may be good alternatives in those who do not respond to initial treatment or who have relapse of disease...

The European Stroke Organisation (ESO) guideline on Primary Angiitis of the Central Nervous System (PACNS), developed according to ESO standard operating procedures (SOP) and Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology, was elaborated to assist clinicians in the diagnostic and treatment pathway of patients with PACNS in their decision making. A working group involving vascular neurologists, neuroradiologists, rheumatologists, a neuropathologist and a methodologist identified 17 relevant clinical questions; these were addressed according to the patient/population, intervention, comparison and outcomes (PICO) framework and systematic literature reviews were performed...

A 53-year-old woman was admitted to our hospital because of increasingly frequent transient speech disturbance and left upper limb weakness. Brain magnetic resonance imaging and angiography revealed multiple intracranial stenoses, including the proximal right middle cerebral artery (MCA), without evidence of infarction. The diagnosis of primary angiitis of the central nervous system was established based on circumferential vascular wall thickening with contrast enhancement observed in the right MCA. Following the administration of dual antiplatelet therapy and intravenous methylprednisolone pulse therapy, the patient experienced complete cessation of symptoms, and the stenosis gradually improved without infarction...

OBJECTIVE: There is limited evidence on when to obtain a central nervous system (CNS) biopsy in suspected primary angiitis of the central nervous system (PACNS). Our objective was to identify which clinical and radiological characteristics were associated with a positive biopsy in PACNS. METHODS: From the multicenter retrospective Cohort of Patients with Primary Vasculitis of the CNS (COVAC), we included adults with PACNS based on a positive CNS biopsy or otherwise unexplained intracranial stenoses with additional findings supportive of vasculitis...

Vasculitis of the central nervous system can be a localized process, such as primary angiitis of the central nervous system (PACNS), or systemic vasculitis, such as ANCA-associated vasculitis (AAV). Since both conditions share neurological manifestations, the following review will discuss the neurological aspects of both. This review aims to provide a comprehensive comparison of the pathogenesis, clinical manifestation and assessment, diagnostic workup, and treatment protocol for both PACNS and AAV with central nervous system involvement...

Childhood primary angiitis of the central nervous system (cPACNS) is a vasculitis of unknown etiology that is confined to the central nervous system (CNS) and can lead to repeated cerebral infarctions if left untreated. Several cases of cPACNS after COVID-19 have been reported. Herein, we present a case of post-vaccination cPACNS. A 9-year-old healthy boy presented with persistent headache and fever after receiving the second COVID-19 vaccine (BNT162b2/Pfizer-BioNtech) dose. Brain magnetic resonance angiography (MRA) performed on the sixth day of symptom onset after vaccination revealed stenosis of the left middle cerebral artery; the patient was referred to our department on the 12th day of symptom onset...

Primary angiitis of the central nervous system (PACNS) is an autoimmune disease with myriad presentations in the form of headache, focal neurological deficits, seizures and rapid cognitive decline. Predilection or isolated and recurrent involvement of one hemisphere is rarely described in literature. This leads to low clinical suspicion and often delayed diagnosis of PACNS, if clinical and radiological involvement is focal. We describe the case of a young man presenting with new-onset focal seizures with imaging suggestive of focal involvement and the brain biopsy clinched an early diagnosis...