Diagnosis Of Primary Central Nervous System Vasculitis Research Articles

A rare case report of primary central nervous system vasculitis

Primary central nervous system (CNS) vasculitis is defined as an inflammation of cerebral vasculature without vasculitis of other organs. It represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. An average annual incidence rate of 2.4 cases per 1,000,000 persons per year has been reported all over the world. It affects medium and small-sized arteries of the brain parenchyma, spinal cord, and leptomeninges resulting in CNS dysfunction. Here, we report the case of a 19-year-old female brought with a history of headache, fever and vomiting, and altered sensorium, and on further clinical, biochemical and radiological evaluation, a final diagnosis of primary CNS vasculitis was made.

THU0591 CENTRAL NERVOUS SYSTEM VASCULITIS IN WHIPPLE DISEASE: A CASE REPORT

Background:Whipple disease (WD) is a rare systemic infection with possible involvement of the central nervous system (CNS). The neurological manifestations of the disease are various and can mimic any neurologic condition.Objectives:To describe the severe neurological complications occurred in a patient with WD misdiagnosed as a chronic inflammatory immune-mediated disorder.Methods:Case reportResults:A 46-year-old woman developed acute right-sided hemiparesis and dysarthria. She had a 10-year history of ill-defined rheumatic condition (defined as seronegative rheumatoid arthritis, spondyloarthritis, and adult onset Still’s disease) presenting with polyarthritis, episodic fever > 38°C and rash of unclear etiology poorly responsive to different immunotherapies including methotrexate, anti-IL6 and anti-IL1 inhibitors. Brain MRI demonstrated multiple anterior circulation infarctions and stenosis of the bilateral M1 segments of the middle cerebral artery on MR angiography. Black blood sequences revealed contrast enhancement of the vessel walls consistent with vasculitis (Figure 1). Cerebrospinal fluid (CSF) analysis was unrevealing, including PCR for viruses and bacteria. A working diagnosis of primary CNS vasculitis and progressive neurologic deterioration with abnormal behavior and altered mental status prompted the initiation of intravenous (IV) methylprednisolone followed by cyclophosphamide without significant improvement. Re-evaluation of the long-standing history of joint symptoms unresponsive to immunotherapy, along with recurrent fevers and chronic diarrhea raised the suspicion of unrecognized Whipple’s disease. PCR for Tropheryma Whippleii was positive in stool, urine, blood and CSF, and duodenal mucosal biopsies confirmed the diagnosis. A combination of ceftriaxone, doxycycline, and hydroxychloroquine was initiated. Three days later the patient developed periocular burning pain and cutaneous vesicles consistent with shingles. Varicella-zoster virus DNA was detected in CSF and IV acyclovir was started. At 3 months follow-up neurologic examination was unremarkable except for a slightly fatuous behavior.Conclusion:Recognition of rare manifestations of WD is important to avoid diagnostic delay and inappropriate, potentially harmful treatments.Disclosure of Interests:Alessandro Giollo: None declared, Cecilia Zivelonghi: None declared, Davide Cardellini: None declared, Gian Marco Schiavi: None declared, Gaetano Vattemi: None declared, Ombretta Viapiana: None declared, Maurizio Rossini Speakers bureau: AbbVie, Abiogen, Amgen, BMS, Eli-Lilly, Novartis, Pfizer, Sanofi, Sandoz and UCB

Primary Central Nervous System Vasculitis Mimicking Brain Tumor

Primary central nervous system vasculitis (PCNSV) is a rare disease with a nonspecific and expansive spectrum of clinical manifestations. A 40-year-old woman presented with acute onset of severe headache associated with nausea. Computed tomography of the brain showed 1 solitary hypodense parenchymal lesion over the left temporal lobe with an internal fluid level. A brain magnetic resonance imaging showed the presence of 1 isointense parenchymal lesion on T1-weighted and T2-weighed images, with surrounding brain edema. The patient accepted craniotomy for excision of lesion under the preoperative impression of brain tumor with hemorrhagic transformation. The pathologic report was extensive vasculitis with hemorrhagic infarction. The diagnosis of PCNSV was made after exclusion of secondary causes–related vasculitis and hereditary hypercoagulant status. This report highlights that PCNSV could appear as a hemorrhagic tumor-like lesion on neuroimaging. Considering PCNSV in the differential diagnosis is important in patients suspected with cerebral hemorrhage, cerebral thrombosis, or brain tumor.

FRI0450 Primary Central Nervous System Vasculitis: Treatment and Course

BackgroundBackground. Because of the relatively small numbers of patients evaluated in the studies, primary central nervous system vasculitis (PCNSV) response to treatment is still uncertain.ObjectivesTo determine the response to therapy...

Malignant perivascular melanoma mimicking primary central nervous system vasculitis

Primary central nervous system vasculitis (PCNSV) is an idiopathic disorder resulting in multifocal inflammation of small arteries and veins of the cortex and leptomeninges. Typically there is no evidence of vasculitis outside the central nervous system [7]. It is a rare disorder, but, nevertheless, responsible for 3–5 % of cerebrovascular accidents in patients aged \50 years [5]. The pathogenesis is poorly understood and the underlying cause is still unknown [2]. Diagnostic criteria include (1) acquired neurological deficits remaining unexplained after complete evaluation; (2) cerebral angiography with alternating segments of stenosis with normal or dilated intervening segments, and arterial occlusions; (3) no evidence of systemic vasculitis or any other condition that could mimic the angiographic findings [4]. The clinical signs and symptoms are nonspecific (e.g., nausea, headache, focal-neurological deficits, neuropsychological disturbances), reflecting the diffuse and often patchy nature of the pathologic process contributing to a high number of misleading diagnoses [7]. While magnetic resonance imaging of the brain is abnormal in more than 90 % of patients, the findings are unspecific. Multiple small or large ischemic lesions with partial hemorrhagic transformation are frequently found. Cerebrospinal fluid (CSF) analysis is usually abnormal with a modest elevation of total protein and lymphocytes. Cerebral angiography may show abnormal vessels, but has a low sensitivity and specificity [2]. Brain biopsy remains the gold standard for the diagnosis of PCNSV. After exclusion of other causes of multifocal infarctions or pleocytosis in CSF (e.g., endocarditis) brain biopsy is mandatory to confirm vasculitis before initiating a potentially toxic therapy [1]. Here we are presenting a patient with a diffuse perivascular melanoma, mimicking PCNSV. We report on a 45-year-old male who suffered from a dull holocephalic headache for three months, loss of weight (more than 10 kg over the last six months), intermittent nausea and vomiting, and progressive exhaustion during the last weeks. He also reported transient paresthesia in the right arm, gait disturbances, and blurred vision. The patient denied any fever and night sweats. His past medical history showed a naevus excised 15 years ago, which was later identified as a malignant melanoma. Frequent follow-up examinations were unremarkable. His temperature was 37.2 C; other vital signs, the general examination and routine blood studies including the erythrosedimentation rate (ESR) and acute phase M. Schneider (&) P. P. Urban Abteilung fur Neurologie, Asklepios Klinik Barmbek, Rubenkamp 220, 22291 Hamburg, Germany e-mail: mart.schneider@asklepios.com

Primary central nervous system vasculitis presenting with intracranial hemorrhage

To describe a subset of cases in a large retrospectively identified cohort of patients with primary central nervous system vasculitis (PCNSV) who present with intracranial hemorrhage. The study consisted of a cohort of 131 consecutive patients with PCNSV who were seen at the Mayo Clinic over a 25-year period from 1983 to 2007. The diagnosis of PCNSV was based on findings of brain or spinal cord biopsy, cerebral angiography, or both. Intracranial hemorrhage at presentation was defined as the presence of intracerebral or subarachnoid hemorrhage on computed tomography or magnetic resonance imaging (MRI) of the brain within 3 months of the date of PCNSV diagnosis. The clinical, laboratory, radiologic, and pathologic findings, therapy, and outcomes in patients presenting with intracranial hemorrhage were compared with those without intracranial hemorrhage. Sixteen patients (12.2%) had evidence of intracranial hemorrhage at or near the time of diagnosis. Twelve patients had intracerebral hemorrhage, and 4 had subarachnoid hemorrhage. Twelve patients were diagnosed by findings on angiography and 4 by findings on CNS biopsy. Compared with the 115 patients without intracranial hemorrhage, the 16 patients presenting with intracranial hemorrhage were more frequently women, less frequently had altered cognition, a persistent neurologic deficit, or stroke at presentation, less frequently had MRI evidence of cerebral infarctions, and less frequently needed therapy at last followup. A necrotizing histopathologic pattern of vasculitis was observed in 3 of the 4 patients with positive biopsy findings (75%). Our findings suggest that intracranial hemorrhage may not be an infrequent occurrence in early PCNSV. Necrotizing vasculitis may be a predominant histopathologic pattern.

Primary central nervous system vasculitis mimicking brain tumour: case report and literature review

Primary central nervous system vasculitis (PCNSV) is a rare inflammatory disease causing significant morbidity and mortality. We present a detailed history and clinical course of a patient with PCNSV along with a literature review. A 50-year-old Chinese female presented with a 6-month history of mild to moderate headache and sudden onset of visual loss. Early computed tomography of the brain showed multifocal infarctions. Over 3 weeks after admission, her clinical presentations changed considerably and her condition deteriorated. She became confused then fell in to semicoma, and developed left-sided paralysis and quadriparesis. At about 7 months after her illness, a magnetic resonance imaging of the brain revealed a tumour-like mass lesion within the right parietal lobe resulting in compressed posterior horn of the right lateral ventricle. In the end, a brain biopsy identified extensive vessel inflammation, which established the diagnosis of PCNSV. Steroid and immunosuppressive therapy dramatically improved the patient's condition. Our report highlights the importance of considering PCNSV as a differential diagnosis in patients who are suspected with encephalitis, multiple sclerosis, cerebral haemorrhage, cerebral thrombus, leukoencephalopathy, or brain tumour, etc. while their clinical presentations are exceptionally so changeable. This case also gives emphasis to the value of a brain biopsy in consolidating the diagnosis, and the efficacy of steroid and immunosuppressive therapy in PCNSV.

Central nervous system vasculitis in children

Central nervous system (CNS) vasculitis is an increasingly recognized, often devastating inflammatory brain disease of children and adults. In primary or isolated CNS vasculitis/angiitis of childhood (cPACNS), the vascular inflammation is limited to the brain and spinal cord. Secondary CNS vasculitis occurs in a variety of conditions including infections, collagen vascular diseases, systemic vasculidities, and malignancies. Mimics of CNS vasculitis in children include dissections and noninflammatory vasculopathies. Diagnosis of primary CNS vasculitis in both adults and children is based on the Calabrese criteria. This review summarizes recent data on CNS vasculitis in children; reviews the clinical spectrum at presentation and the role of laboratory tests, neuroimaging, and brain biopsy; and discusses treatment strategies, outcome data, and overlapping conditions of cPACNS.