Case Report: A Case Report on an 18-Year-Old Female with Cerebral Vasculitis in Systemic Lupus Erythematosus (SLE).

Cerebral vasculitis is a rare but severe complication of Systemic Lupus Erythematosus (SLE), presenting significant challenges in management due to its potential for devastating neurological consequences and poor prognosis. We present a case of an 18-year-old female with known SLE who presented with seizures, declining cognitive function, and unresponsiveness. Neurological examination, laboratory investigations, and radiological imaging supported the diagnosis of cerebral vasculitis secondary to SLE. Despite aggressive immunosuppressive therapy, the patient's neurological status continued to deteriorate, leading to respiratory failure and multiorgan dysfunction. Ultimately, the patient succumbed to multiorgan failure attributed to severe CNS vasculitis and its complications. This case underscores the importance of early recognition and aggressive management of cerebral vasculitis in SLE while highlighting the need for further research into more effective therapeutic strategies to improve patient outcomes.

Cerebral vasculitis presenting as acute posterior multifocal placoid pigment epitheliopathy in a 16-year-old male.

Purpose: To report on a case of the successful treatment of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) in a pediatric patient with a prior diagnosis of cerebral vasculitis. Observations: A 16-year-old male with a prior diagnosis of cerebral vasculitis presented without ocular complaints. Visual acuity was 20/20, and color vision remained normal. Fundus examination revealed yellowish-white placoid lesions and retinal pigmented epithelial changes involving the posterior pole. A work-up including a rapid plasma reagin test, complete cell blood count, comprehensive metabolic panel, and urinalysis was within normal limits. A head computed tomography angiography without contrast and a brain magnetic resonance imaging scan were compatible with acute and past episodes of ischemia. Ancillary testing was compatible with an assessment APMPPE. Immunosuppressive and monoclonal antibody therapy resulted in the improvement and remission without residual neurologic deficits and with a BCVA of 20/20. Conclusionand Importance: This case suggests that a diagnosis of cerebral vasculitis should prompt physicians to consider an ophthalmic evaluation that includes a dilated fundus exam, regardless of the presence or absence of ocular symptoms. Ophthalmic findings may affect the diagnostic processes, particularly concerning infectious and non-infectious etiologies, or potentially neoplastic diseases.

Multiple Severe Intracranial Stenoses with Ischemic Stroke in Neuroborreliosis-associated Cerebral Vasculitis: Endovascular Treatment Strategies and Literature Review.

INTRODUCTION: Neuroborreliosis is the disseminated form of Lyme borreliosis and refers to the involvement of the central nervous system by Borrelia burgdorferi sensu lato spirochetes. Several reports suggest its emergence as a potential cause of cerebral vasculitis and stroke in children and young adults. The objective of this paper is to highlight endovascular treatment options within this context. METHODS: The medicinal and endovascular treatments of three patients-two adults and one child-with ischemic stroke resulting from neuroborreliosis-associated severe cerebral vasculitis were retrospectively assessed. Detailed descriptions of the clinical course, treatments, and follow-up data for each patient are provided. Additionally, a literature review focusing on endovascular treatment options within this topic was conducted. RESULTS: Both endovascular and medicinal treatments resulted in excellent clinical outcomes in all three patients, with no observed periprocedural complications. Significant clinical improvement was noted during mid-term follow-up. Follow-up angiographies confirmed stent patency. CONCLUSION: Endovascular interventions as a bailout strategy may enhance clinical outcomes in patients with vascular complications of neuroborreliosis, especially when medicinal therapy alone fails to achieve further improvement. In the setting of severe ischemic stroke with sub-occlusive large vessel stenosis or occlusion, the cause of which is often unknown, it should be considered to prioritize prompt endovascular treatment, even if neuroborreliosis is suspected on admission.

Case report: Non-EBV associated cerebral vasculitis and cerebral hemorrhage in X-linked lymphoproliferative disease.

X-linked lymphoproliferative disease (XLP) is a rare genetic disorder characterized by immune dysregulation. The three most common clinical phenotypes are EBV-associated infectious mononucleosis (FIM), abnormal gammaglobulinemia, and lymphoma. We present a rare case of XLP1 with neurovasculitis, which is non-EBV-related and involves multiple systems, a condition rarely seen in children. The patient initially presented with an unsteady gait, which progressively evolved into language and consciousness disorders. Additionally, CT scans revealed multiple nodules in the lungs. Subsequent genetic testing and brain tissue biopsy confirmed the diagnosis: XLP1-related cerebral vasculitis and cerebral hemorrhage. Tragically, during the diagnostic process, the child experienced a sudden cerebral hemorrhage and herniation, ultimately resulting in fatality. This case offers a comprehensive insight into XLP1-related cerebral vasculitis and cerebral hemorrhage, underscoring the significance of early diagnosis and prompt treatment, while also imparting valuable clinical experience and lessons to the medical community.

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. RESULTS: Fourteen patients were included (six females; average age 48 ± 19 years). Diagnoses included primary CNS vasculitis (PCNSV) in six patients and secondary CNS vasculitis (SCNSV) in eight, half of which were infection-related. Thirteen patients showed vessel wall enhancement, which was intense in eleven patients (84.6%) and concentric in twelve (92.3%), affecting the anterior circulation in nine patients (69.2%), posterior in two patients (15.4%), and both circulations in two patients (15.4%). The enhancement patterns were similar across different CNS vasculitis types. DWI changes corresponded with areas of vessel wall enhancement in 77% of patients. Conclusions: CNS vasculitis is often associated with intense, concentric vessel wall enhancement in VW-MRI, especially in the anterior circulation. The consistent presence of DWI alterations in affected territories suggests a possible link to microembolization or hypoperfusion. These imaging findings complement parenchymal brain MRI and MRA/DSA data, potentially increasing the possibility of a clinical diagnosis of CNS vasculitis.

Cardiac myxoma and cerebral vasculitis: Is there a link?

BACKGROUND: Cardiac myxomas present a diagnostic challenge due to their ability to mimic various cardiovascular and systemic conditions. Timely identification is crucial for implementing surgical intervention and averting life-threatening complications. CASE PRESENTATION: We reported the case of a 49-year-old male patient who presented sudden legs weakness and slurred speech and was admitted 10 h later in emergency department. Physical examination was significant for paraparesis and paraphasia. Cardiac and carotid auscultation was normal. CT brain revealed multiple acute ischemic strokes and MRA was suggestive of cerebral vasculitis. As pre-therapy assessment, the EKG revealed no electrical abnormalities and the chest X-ray showed signs of left atrial enlargement. Transthoracic and transesophageal echocardiography showed a left atrial mass attached to the interatrial septum, measuring 9*5*4 cm and extending into the left ventricular cavity during diastole, which suggested the diagnosis of left atrial myxoma. The patient was referred for open-heart surgery and histopathological examination confirmed the diagnosis of myxoma. The patient weaned off from cardiopulmonary bypass and the postoperative period was uneventful. CONCLUSION: We reported an interesting case with an unusual and misleading neurological presentation of a cardiac myxoma. The unpredictability of serious complications occurrence must awaken our medical flair, for an early diagnosis among a long list of differentials.

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. In the present paper, the histopathological features of primary angiitis of the CNS are described and highlighted to help pathologists avoid misdiagnosis of a treatable acquired disease.

Case report: Infectious cerebral vasculitis due to rickettsiosis.

Rickettsiosis is a widespread infection throughout the world and in Africa, it covers a wide range of infectious diseases caused by Rickettsia species. Rickettsial infections, with the exception of Q fever, typically present with fever, rash, and vasculitis. The central nervous system (CNS) can be affected by all rickettsial diseases and is an important target for several of them. Clinical manifestations are suggestive of rickettsial infection, but serology and skin biopsy provide confirmation. Although the presence of abnormal neuroimaging is rare, its presence is associated with a worse clinical prognosis. Computed tomography (CT) and magnetic resonance imaging (MRI) scans mainly show signs of vasculitis, which may be reversible if appropriate treatment is initiated early in the course of the disease. We present here a case of infectious cerebral vasculitis due to rickettsiosis with some MRI features.

Crack lung with toxic cerebral vasculitis: Case report.

Crack is the most potent form of cocaine. It directly affects lungs if inhaled and the damage may include barotrauma, acute pulmonary edema, alveolar hemorrhage, bronchiolitis obliterans with organizing pneumonia, or vasculitis. The diagnosis of cocaine-related lung damage is based on clinical symptoms and radiological findings. When young individuals develop respiratory symptoms, investigation into cocaine use is necessary. We report the case of a young man with a history of cocaine use who presented for respiratory and neurological symptoms revealing crack lung and toxic cerebral vasculitis.

Comparison of patients with biopsy positive and negative primary angiitis of the central nervous system.

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. Baseline findings were compared between patients with a positive and negative biopsy using logistic regression models. RESULTS: 200 patients with PACNS were included, among which a biopsy was obtained in 100 (50%) and was positive in 61 (31%). Patients with a positive biopsy were more frequently female (OR 2.90, 95% CI 1.25-7.10, p = 0.01) and more often presented with seizures (OR 8.31, 95% CI 2.77-33.04, p < 0.001) or cognitive impairment (OR 2.58, 95% CI 1.11-6.10, p = 0.03). On imaging, biopsy positive patients more often had non-ischemic parenchymal or leptomeningeal gadolinium enhancement (OR 52.80, 95% CI 15.72-233.06, p < 0.001) or ≥ 1 cerebral microbleed (OR 8.08, 95% CI 3.03-25.13, p < 0.001), and less often had ≥ 1 acute brain infarct (OR 0.02, 95% CI 0.004-0.08, p < 0.001). In the multivariable model, non-ischemic parenchymal or leptomeningeal gadolinium enhancement (aOR 8.27, 95% CI 1.78-38.46), p < 0.01) and absence of ≥ 1 acute brain infarct (aOR 0.13, 95% CI 0.03-0.65, p = 0.01) were significantly associated with a positive biopsy. CONCLUSIONS: Baseline clinical and radiological characteristics differed between biopsy positive and negative PACNS. These results may help physicians individualize the decision to obtain a CNS biopsy in suspected PACNS.

Cerebral vasculitis as a complication of pneumococcal meningitis: A cohort study.

OBJECTIVE: Cerebral vasculitis (CV) is a severe complication of pneumococcal meningitis (PM); whether dexamethasone use can reduce its occurrence remains to be determined. METHODS: This is a retrospective observational bicentric study analyzing all adults with proven PM hospitalized between January 2002 and December 2020 in two tertiary hospitals. Extrapolating from a standardized definition of primary angiitis of the central nervous system, we defined CV as worsened neurological symptoms associated with compatible imaging. All images were analyzed by a radiologist, and two neurologists reviewed all inconclusive cases of suspected CV for adjudication. Factors associated with CV were analyzed, including dexamethasone use. A subgroup analysis was limited to patients with a lumbar puncture at PM diagnosis. RESULTS: Among 168 patients with PM, 49 (29.2%) had CV, occurring after a median of 8 days (IQR 5-13) of PM diagnosis. In multivariate analysis (N = 151), initial CRP was associated with CV (OR 1.28 per 50-unit increase, p = 0.003), which was marginally linked with delayed hospital admission more than 48 hours after first symptoms (OR 2.39, p = 0.06) and prior NSAID intake (OR 2.94, p = 0.05). Dexamethasone administration did not impact CV occurrence. In 133 patients having undergone lumbar puncture, CSF protein level > 4.4 g/L (OR 4.50, p = 0.006) was associated with CV. CONCLUSIONS: In our cohort, CV was a frequent and severe complication of PM, often occurring in association with unduly delayed medical care, high CRP at admission, and high levels of protein in CSF.

Progressive Multifocal Leukoencephalopathy Mimicking a Cerebral Vasculitis Flare.

We report a case of a 68-year-old woman with a background of primary cerebral vasculitis, which was diagnosed two years ago. She appeared to have had a recurrence of her symptoms with new onset history of expressive dysphasia, right-sided upper limb weakness, and right-sided facial weakness during a rheumatology clinic visit. The patient was on maintenance azathioprine for her cerebral vasculitis at the time of presentation. She had received a total of 2 g of rituximab through intravenous infusion, with a two-week interval between doses. Additionally, she had undergone intravenous cyclophosphamide treatment (15 mg/kg) following the standard vasculitis regimen for induction remission therapy, which was administered at the time of her diagnosis two years prior. Initial imaging on non-contrast computed tomography head after admission to the emergency department did not show any acute neurological findings. Further imaging studies revealed changes in the right parietotemporal white matter T2 hyperintensity with similar changes on the left frontal and left parietal lobes suggestive of progressive multifocal leukoencephalopathy (PML). A magnetic resonance imaging (MRI) of the brain conducted three months prior was found to be unremarkable. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) testing confirmed the presence of polyoma John Cunningham (JC) virus deoxyribonucleic acid (DNA). This case highlights that PML should be an important differential to consider in any immunocompromised patient who presents with new stroke-like features.

Unraveling the Puzzle: A Case Report Questioning the Causal Relationship Between Subarachnoid Hemorrhage and Microscopic Polyangiitis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are small-to-medium-vessel vasculitis, which includes granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. MPA predominantly affects the kidneys and lungs. Subarachnoid hemorrhage (SAH), a life-threatening condition, rarely occurs with AAV. In this case, we present a 67-year-old female who presented with a sudden-onset headache after a recent diagnosis of ANCA-associated renal vasculitis. Kidney biopsy revealed pauci-immune glomerulonephritis, and serum was positive for ANCA along with myeloperoxidase antibody. A computed tomography scan of the head revealed both SAH and intraparenchymal hemorrhage. The patient was managed medically for SAH and intraparenchymal hemorrhage. ANCA vasculitis was treated with steroids and rituximab, and the patient showed improvement.

Lyme neuroborreliosis: Progressive cerebral vasculitis responsive to cyclophosphamide. A case report and review of the literature.

OBJECTIVES: Less than 1 % of patients with Lyme Neuroborreliosis (LNB) present with a cerebrovascular event. Ischaemic strokes occur more commonly than parenchymal or subarachnoid haemorrhages. If cerebral vasculitis due to LNB is suspected, antibiotic treatment should be started immediately, which will normally lead to remission. Very rarely progression and recurrent strokes are observed despite sufficient antibiotic therapy, even if steroids are added. Currently there are no guidelines on the adequate treatment of cerebral vasculitis due to LNB which is not responsive to antibiotics and steroids, but in very few reported cases cyclophosphamide led to disease stabilisation. We reviewed the literature regarding cyclophosphamide treatment in these patients and want to share our experience of cyclophosphamide therapy in progressive cerebral vasculitis due to LNB. RESULTS: We report a 71-year-old female patient with cerebral vasculitis and multiple strokes as a complication of LNB. Progression could only be halted by additional immunosuppressive treatment using cyclophosphamide. However, at that point the patient had already suffered severe ischaemic brain damage. Similarly, in existing case reports cyclophosphamide had been administered only at a time when patients already showed serious neurological deficits. CONCLUSION: Cerebral vasculitis in patients with LNB is very rare and normally responds to antibiotic treatment. A minority of patients show disease progression despite antibiotics and steroids. Our case report strengthens the recommendation that in those patients - even if signs of progressive vasculitis are only detectable on imaging and not clinically - cyclophosphamide should be considered without delay to prevent further cerebrovascular events.

Severe Juvenile-Onset Systemic Lupus Erythematosus: A Case Series-Based Review and Update.

Juvenile-onset systemic lupus erythematosus (jSLE) is a multisystemic disease diagnosed in young patients based on the clinical criteria of the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). The importance of this condition lies in its greater aggressiveness compared with lupus diagnosed during adulthood (aSLE). Management, which is based on supportive care and immunosuppressive drugs, aims to reduce the overall disease activity and to prevent exacerbation. Sometimes the onset is accompanied by life-threatening clinical conditions. In this paper, we introduce three recent cases of jSLE that required admission to the Pediatric Intensive Care Unit (PICU) of a Spanish pediatric hospital. This manuscript aims to review some of the main complications associated with jSLE, such as diffuse alveolar hemorrhage, cerebral vasculitis, or an antiphospholipid syndrome; these are life-threatening conditions but they have a chance of favorable prognosis if treated early and aggressively.

Cerebral Vasculitis Revealing Systemic Sarcoidosis: A Case Report and Review of the Literature.

Vasculitis in neurosarcoidosis is rare, with only a few cases reported in the literature. We report the clinical observation of a 51-year-old patient with no previous medical history, who was admitted to the emergency department due to sudden onset confusion, fever, sweating, weakness, and headaches. The first brain scan was normal, but a further biological exam with a lumbar puncture revealed lymphocytic meningitis. A complementary cerebral MRI revealed abnormalities in the white matter signal, suggestive of multiple sclerosis, with petechial hemorrhagic foci associated with leptomeningeal involvement and cerebral vasculitis. Thoraco-abdomino-pelvic computed tomography revealed hilar and mediastinal lymphadenopathy, as well as lymph nodes in the lower cervical region. A biopsy of the lymph nodes confirmed the presence of non-caseating granulomatous inflammation consistent with sarcoidosis. High-dose corticosteroid therapy was initiated with good clinical outcomes. Cerebral vasculitis in neurosarcoidosis is rare but can lead to neurological complications requiring long-term multidisciplinary management.

Reversible cerebral vasoconstriction syndrome: literature review.

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a neurovascular condition characterized by a severe sudden-onset headache that may be associated with focal neurological deficits. On imaging, the suggestive finding corresponds to multifocal vasoconstriction of the cerebral arteries, with a spontaneous resolution of approximately 12 weeks. The identification of precipitating factors and diagnosis must be carried out early, so that adequate management is established and the patient has a good prognosis, given the risk of secondary complications and residual neurological deficits. This study consists of a literature review based on the analysis of articles published between 2017 and 2022 in PubMed, SciELO, and ScienceDirect on RCVS, intending to understand the clinical and radiological characteristics, diagnosis, treatment, and prognosis of patients with RCVS. The pathophysiology, drug management, and prognosis still lack solid evidence; therefore, further studies on RCVS are needed to expand medical knowledge and avoid underdiagnosis and inadequate treatment of this important condition.

A 53-year-old woman with a rapidly progressive, non-enhancing left frontotemporal lesion.

Fifty-three-year-old woman presented with chronic, episodic headache.