Central Nervous System Vasculitis Research Articles

Primary Central Nervous System Vasculitis Mimicking Susac Syndrome and Multiple Sclerosis With Long-Term Remission and Spontaneous Resolution of Lesions: A Case Report

Primary Central Nervous System Vasculitis Mimicking Susac Syndrome and Multiple Sclerosis With Long-Term Remission and Spontaneous Resolution of Lesions: A Case Report

Primary central nervous system vasculitis with intracranial aneurysm

BackgroundUnruptured intracranial aneurysms (UIAs) are rarely reported in primary central nervous system vasculitis (PCNSV). In this study we described the clinical findings, response to therapy, and outcomes of UIA in a large cohort of PCNSV patients. MethodsWe retrospectively studied 216 consecutive patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 40-year period. UIAs were identified on cerebral angiography. The clinical, laboratory, radiologic and pathologic findings, management, and outcomes of patients with UIA were described and compared with those without UIA. Results12/216 (5.5 %) PCNSV patients had at least one UIA. Two patients underwent biopsies; one yielded negative results, while the other showed necrotizing vasculitis. Eleven patients had evidence of UIA on angiogram at diagnosis. One patient developed an aneurysm during the follow-up associated with a worsening of vasculitic radiological findings. The most common presenting symptom for PCNSV in the setting of UIA was headache (67 %), followed by persistent neurologic deficit or stroke (50 %). Most patients with UIA presented with multiple cerebral infarcts on MRI (67 %), one patient had subarachnoid hemorrhage, and one left parieto-occipital intracerebral hematoma, both unrelated to the aneurysm. Black blood imaging was performed in 4 patients and 2 showed segmental circumferential mural enhancement involving multiple vessels. Two patients had 2 UIAs, while the other 10 had 1. The most frequent UIA location was internal carotid artery (50 %), followed by anterior cerebral artery (21 %). Ten of the UIAs were < 5 mm in diameter, and 3 were 5–7 mm in diameter; the size was not available for one. All UIAs were unchanged in size and configuration during follow-up (median: 18.5 months; range 1–151 months) and no new aneurysms were detected. Compared to the 204 patients with PCNSV without a UIA, no significant clinical differences were observed, except for a reduced disability at last follow-up (p = 0.038). ConclusionsUIAs uncommonly occur in PCNSV.

Spinal Cord Biopsy Revealing Primary Central Nervous System Vasculitis (PCNSV) in a Patient with Suspected Non-compressive Dorsal Myelopathy.

Spinal Cord Biopsy Revealing Primary Central Nervous System Vasculitis (PCNSV) in a Patient with Suspected Non-compressive Dorsal Myelopathy.

Aseptic meningitis and Fabry disease.

Fabry disease is caused by enzymatic defects in alpha-galactosidase A that leads to the accumulation of glycosphingolipids throughout the body, resulting in a multisystemic disorder. The most common neurological manifestations are neuropathic pain, autonomic nervous system dysfunction and strokes, but some rarer neurological manifestations exist. Among these, aseptic meningitis is a possible complication. Our objectives were to measure the prevalence of this complication in a cohort of patients with Fabry disease, and to describe its clinical features. We conducted a retrospective review of Fabry disease patients followed at our tertiary referral center between 1995 and September 2023 with at least one episode of meningitis, and performed a systematic review to identify similar published cases. Four patients out of 107 (3.7%) had at least one episode of aseptic meningitis. Our systematic review identified 25 other observations. The median age of these 29 patients was 29.0 years, the median cerebrospinal fluid leukocyte count was 24 cells/mm3 with a predominance of lymphocytes in 64.7% of cases. In 82.8% of the patients, the diagnosis of Fabry disease was unknown before the meningitis. Large artery stenosis was present in 17.2% of patients and 57.1% of patients had a recent stroke concomitant with the meningitis. Several differential diagnoses were evoked, such as multiple sclerosis or central nervous system vasculitis. Our study suggests that Fabry disease should be considered as a cause of aseptic meningitis. The pathophysiological mechanisms underlying meningeal inflammation remain largely unknown but may reflect the dysregulation of pro-inflammatory signaling pathways.

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

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

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

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). 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. Two experienced readers independently reviewed the neuroimages. Clinical data were extracted from the electronic patient records. The findings were then applied to a cohort of patients with large vessel central nervous system (CNS) vasculitis. We included 21 patients with SV-PACNS from December 2021 to November 2023. Of these, 12 (57.14%) had cerebral cortical microhemorrhages with atrophy. The pattern with microhemorrhages was described in detail based on the gradient echo sequence, leading to the identification of what we have termed the "coral-like sign." The onset age of patients with coral-like sign (33.83 ± 9.93 years) appeared younger than that of patients without coral-like sign (42.11 ± 14.18 years) (P = 0.131). Furthermore, the cerebral lesions in patients with cortical microhemorrhagic SV-PACNS showed greater propensity toward bilateral lesions (P = 0.03). The coral-like sign was not observed in patients with large vessel CNS vasculitis. The key characteristics of the coral-like sign represent cerebral cortical diffuse microhemorrhages with atrophy, which may be an important MRI pattern of SV-PACNS. ANN NEUROL 2024;96:194-203.

Primary CNS vasculitis: insights into clinical, neuropathological, and neuroradiological characteristics.

Primary CNS vasculitis (PCNSV) is a rare inflammatory disorder that affects the blood vessels of the central nervous system (CNS). We aimed to analyze the neurological presentations, clinical follow-up, and long-term outcomes of patients with primary central nervous system vasculitis. We conducted a retrospective analysis of medical records to assess the neurological presentation, rate of remission, and functional status at the last follow-up in patients with primary central nervous system vasculitis seen in our center in the last 13 years (2010-2023). We identified five patients, whose median age at symptom onset was 31 years (range: 15-41 years), including four male individuals (80%) from Muslim Arab (n = 4) and Ashkenazi Jewish (n = 1) backgrounds. Symptoms persisted for a median of 36 weeks (range: 3 weeks to 4 years) before diagnosis, with one case exceeding 3 years. Follow-up lasted a median of 56 months (range: 20-161 months). Clinical symptoms varied, presenting unilateral weaknesses (n = 2), cognitive and gait abnormality (n = 1), headaches (n = 1), and epileptic seizures (n = 1). MRI scans revealed abnormalities in the basal ganglia, corona radiata, parietal, and frontal lobes, showing hemorrhage, vasogenic edema, restricted diffusion, and enhancement post-gadolinium. All patients reported progressive holocephalic headaches and cognitive changes with overall progressive symptoms. Initial neurological examinations revealed abnormalities in all patients and included one or more of the following: cognitive or visual impairment (n = 2), seizures (n = 1), and unilateral UMN signs (n = 2) at the initial neurological examination, all but one patient required walking aids including (cane 2, wheelchair, bedridden 1). Patients were stable (n = 2), deteriorated (n = 1), or improved (n = 2). Following treatment, two patients still required ambulatory aids, with one using a cane and the other using a wheelchair, while the remaining three did not require any ambulatory aids. The study on PCNSV highlights varied symptoms and diagnostic challenges, including delayed diagnosis and a spectrum of neurological issues from cognitive impairments to seizures. Brain biopsies showed lymphocytic infiltration, thrombi, and necrosis. Immunotherapy significantly improved clinical and radiological outcomes. Over 56 months of follow-up, outcomes varied from stability and deterioration to improvement.

Pediatrik Bir Hastada İskemik İnmeye Neden Olan Primer Santral Sinir Sistemi Vasküliti: Nadir Bir Olgu Sunumu

Amaç: Çocukluk çağında inme edinsel beyin hasarına yol açabilen nörolojik bulgu ve semptomlarla karakterize nöropatolojik bir sendromdur. Etiyolojide sıklıkla kardiyoembolik, trombofilik, genetik hastalıklar ve nadiren serebral vaskülitler görülür. Serebral vaskülit terimi sistemik hastalıklara sekonder serebral tutulumu ve yalnızca santral sinir sitemini tutan primer vaskülitleri kapsamaktadır. Primer santral sinir sitemi vasküliti nadir görülmekle birlikte, tanısı sekonder nedenler ve sıklıkla karışan moya moya arteriopatisi, geçici serebral vazokonstrüksiyon sendromu, mikroskobik poliarterit gibi tanıların dışlanmasına dayanır.&#x0D; &#x0D; Olgu: 14 yaşında erkek hasta bayılması ve o sırada başlayan konuşma bozukluğu olması üzerine acil servise getirildi. Fizik muayenede Glasgow koma skalası skoru 15, motor kuvvetinin sağ alt ve üst ekstremitelerde 1/5, sol alt ve üst ekstremitelerde 5/5, fasiyal asimetri ve konuşmada artikülasyon bozukluğu olduğu görüldü. Sistemik muayenesinde travma bulgusu saptanmadı. Difüzyon ağırlıklı manyetik rezonans görüntülemesinde sol bazal ganglionlar, posterior frontal ve presantral giruslarda akut iskemik inme ile uyumlu difüzyon kısıtlılığı olduğu tespit edildi. Acil serviste enoksaparin (6000 ANTI-XA IU/0.6 ml subkutan 2x1) tedavisi başlanan hasta iskemik inme ön tanısı ile hastaneye yatırıldı. Hastanın bilgisayarlı beyin tomografisi, kontrastlı beyin anjiografi ve manyetik rezonans venografisinde patoloji saptanmadı. Ayrıca beyin manyetik rezonans anjiografi ve dijital subtraksiyon anjiyografide patoloji görülmedi. Olası diğer tanılar dışlandıktan sonra primer santral sinir sistemi vasküliti düşünülerek yüksek doz metilprednizolon tedavisi başlandı.&#x0D; &#x0D; Sonuç: Çocukluk çağında iskemik inme erişkinlere oranla daha az görülse de nörolojik disfonksiyon, tekrarlayan inme riski ve artmış mortalite ile ilişkilidir. Primer santral sinir sistemi vasküliti tanısı dikkatli bir ayırıcı tanı sürecine dayanan nadir bir pediatrik inme nedeni olduğu hatırlanmalıdır.

High Frequency of Autoantibodies in COVID-19 Patients with Central Nervous System Complications: a Multicenter Observational Study.

We present a panel of central nervous system (CNS) complications associated with coronavirus disease 2019 (COVID-19) and their clinical characteristics. We aim to investigate associations between neurological autoantibodies and COVID-19 patients with predominant CNS complications. In this retrospective multi-center study, we analyze neurologic complications associated with COVID-19 patients from Dec. 2022 to Feb. 2023 at four tertiary hospitals in China. CSF and/or serum in the enrolled patients were tested for autoantibodies using tissue-based assays (TBAs) and cell-based assays (CBAs). A total of 34 consecutive patients (median age was 40.5 years [range 15-83], 50% were female) were enrolled. CNS syndromes included encephalitis (n=15), encephalopathies (n=6), meningoencephalitis (n=3), ADEM (n=2), depression (n = 2), Alzheimer's disease (n=2), Parkinson disease (n=1), and central nervous system vasculitis (n=1). Twenty-eight specimens (of 44 tested; 11/27 [40.7%] CSF, 13/17 [76.5%] serums) were confirmed by TBAs to be autoantibodies positive. However, only a few autoantibodies (1 with MOG and 1 with NMDAR) were detected by CBAs assays. Twenty-four patients received immunotherapy. After a mean time of 7.26 months of follow-up, 75.8% (25/33) of patients had good outcome (mRS score ≤2). Although no significant difference was observed between the two groups, the proportion of positive CSF autoantibodies in the poor outcomes group was higher than that in the good outcomes group (57.1% vs 31.5%, P = 0.369). Autoantibodies were frequently observed in COVID-19-associated CNS complications. The identification of these autoantibody-positive COVID-19 cases is important as they respond favorably to immunotherapy.

Assessment of central nervous system vasculitis in children based on high-resolution vascular wall imaging.

Central nervous system vasculitis (CNSV) is a rare disease. High-resolution vessel wall imaging (HR-VWI) enables the identification of inflammatory changes within the vessel wall. Few studies have applied HR-VWI to assess CNSV in children. This study delves into the utility of HR-VWI for diagnosing and treating CNSV in children, with the aim of enhancing clinical diagnosis and efficacy evaluation. Imaging data were acquired from children who underwent HR-VWI examinations. The study meticulously analysed clinical data and laboratory tests to discern the characteristics and distribution patterns of diverse vasculitis forms. In children, CNSV mainly involves medium vessels with grade 1 and 2 stenosis (grade 4 stenosis is rare), and the imaging features generally show centripetal and moderate enhancement, suggesting that this feature is specific for the diagnosis of CNSV. High-grade stenosis, concentric enhancement and strong enhancement of the vasculature indicate more severe disease activity. Remarkably, HR-VWI proved to be significantly more sensitive than magnetic resonance angiography in detecting CNSV. Among the 13 cases subjected to imaging review, 8 demonstrated a reduction or resolution of vessel wall inflammation. In contrast, five patients exhibited worsening inflammation in the vessel wall. HR-VWI demonstrated that changes in vessel wall inflammation were closely correlated with changes in brain parenchymal lesions and symptoms. This study underscores the diagnostic value of HR-VWI in CNSV assessment and treatment monitoring, offering a quantitative evaluation of CNSV in children.

DIAGNOSTIC CHALLENGE IN MIDDLE-AGED WOMAN WITH RECURRENT ISCHEMIC STROKES: A CASE OF PRIMARY CENTRAL NERVOUS SYSTEM VASCULITIS AND LITERATURE REVIEW

Primary vasculitides of the central nervous system are rare vascular disorders whose pathophysiology involves inflammatory infiltration exclusively in the vessels of the central nervous system. Its spectrum extends from primary angiitis of the central nervous system to cerebral amyloid angiopathy. This case portrays a 46-year-old woman with a history of multiple strokes. History of multiple comorbidities such as systemic arterial hypertension, prediabetes, and smoking. During follow-up after the second ischemic event, a patent foramen ovale was detected with positive microbubbles stand. Therefore, she was using oral anticoagulants. Transthoracic echocardiogram and magnetic resonance angiography without relevant changes. However, she resorted to the neurological deficit three more times. Cerebrospinal fluid (CSF) analysis was done twice; both were normal. Cerebral angiography was performed at the time of the last stroke and revealed suggestive signs of vasculitis. Discrimination of angiitis and reversible cerebral vasoconstriction syndrome was challenging and based on the demographic and clinical features of the patient. The characteristics of this case that corroborate the hypothesis of angiitis are the patient's age, which is in the median age range (40-60 years), chronic headache, and focal symptoms not temporally associated with the headache. Intravenous methylprednisolone pulse therapy (one gram/day) was performed for five days. Evolved with progressive improvement of the motor deficit. On hospital discharge, it was prescribed prednisone, and after ten days, Azathioprine 2.5mg/Kg/day was started. Diagnosis of primary central nervous system vasculitis requires high clinical suspicion due to the low specificity of the clinical features. This report reinforces the importance of investigating CNS vasculitis in young patients with a history of recurrent stroke.

Treatment of Primary Angiitis of the Central Nervous System.

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. Mortality rates of 8% to 9% are reported in the literature.

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

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. A single-center retrospective chart review was conducted at the University of Utah, Department of Neurology, between February 2011 and October 2022. The median age of the 44 included patients at diagnosis was 54years; 25.0% were men. Compared to primary CNS vasculitis, secondary CNS vasculitis exhibits higher fever incidence (observed in infectious and connective tissue disorder [CTD]-associated vasculitis), low glucose levels (mostly in infectious vasculitis) and unique cerebrospinal fluid oligoclonal bands (observed in infectious and CTD-associated vasculitis). Patients with inflammatory cerebral amyloid angiopathy (CAA) were older and more commonly had microhemorrhage than primary angiitis of the CNS (PACNS). All patients with CTD-associated vasculitis had a known history of CTD at presentation. Brain biopsies were performed on 10 of 17 PACNS patients and 4 of 8 inflammatory CAA patients, confirming vasculitis in 7 and 4 patients, respectively. Intravenous methylprednisolone was the predominant induction therapy (63.6%), and cyclophosphamide was the most used adjunctive therapy. Cyclophosphamide, rituximab, azathioprine, and mycophenolate mofetil were utilized as maintenance therapy, often with concurrent prednisone. Patients with inflammatory CAA had a higher tendency for relapse rates than PACNS. This study highlights the variations in patients' characteristics, symptoms, and treatment for CNS vasculitis. Understanding these differences can lead to more efficient diagnostic and management strategies.

GLOBRYTE: A Phase III, Open-Label, Multicenter, Randomized Trial Evaluating Glofitamab Monotherapy in Patients with Relapsed or Refractory Mantle Cell Lymphoma

Background and Significance: There is currently no standard of care for patients (pts) with relapsed or refractory (R/R) mantle cell lymphoma (MCL). Although Bruton tyrosine kinase inhibitors (BTKi) have improved clinical outcomes in R/R MCL, there is a high unmet need for new treatment options for pts who do not respond to, or progress through, BTKi therapy. Chimeric antigen receptor (CAR) T-cell therapies, such as brexucabtagene autoleucel, have shown promising outcomes, but alternative systemic therapies are still needed in this pt population. Glofitamab is a CD20xCD3 T-cell-engaging bispecific antibody that redirects T cells to eliminate B cells. A Phase I/II trial of glofitamab monotherapy with step-up dosing (SUD) and obinutuzumab pretreatment (Gpt; 1000 or 2000mg) to mitigate the risk of cytokine release syndrome (CRS) showed high and durable complete response (CR) rates (73.0%) and manageable, mostly low-grade CRS in heavily pretreated pts with R/R MCL (n=37), most of whom had failed prior BTKi therapy (Phillips et al. ASH 2021, Phillips et al. ASH 2022). Study Design and Methods : The GLOBRYTE study (GO43878; EU CT: 2023-503206-37-00) is a Phase III, open-label, multicenter, randomized, controlled trial that will evaluate the efficacy, safety, pharmacokinetics (PK), and pharmacodynamics (biomarkers) of glofitamab monotherapy in pts with R/R MCL, in comparison with an investigator's choice of rituximab + bendamustine (BR) or rituximab + lenalidomide (R-Len). Pts with histologically confirmed R/R MCL who have received ≥1 prior line of systemic therapy (including a BTKi) and have an Eastern Cooperative Oncology Group performance status of 0-2 are eligible for enrollment. Exclusion criteria include leukemic, non-nodal MCL; prior CAR T-cell therapy or CD20xCD3 bispecific antibodies; primary or secondary central nervous system (CNS) lymphoma or history of CNS lymphoma; and current or prior CNS disease (including stroke or transient ischemic attack in the past 2 years and residual neurologic deficits, epilepsy, or CNS vasculitis). Eligible pts will be randomly assigned (1:1) to receive glofitamab or investigator's choice of BR or R-Len stratified by line of therapy (1 vs ≥2) and response to last therapy (relapsed vs refractory). Pts will receive intravenous (IV) Gpt (2000mg) on Day (D) 1 of Cycle (C) 1, 7 days prior to the first glofitamab dose (Gpt may be split over 2 days as 1000mg doses if needed: the second 1000mg dose must be administered ≥1 day before initial glofitamab treatment). Pts will receive glofitamab for a fixed duration of 12 cycles, unless progressive disease (PD) or unacceptable toxicity occurs earlier. Glofitamab IV SUD will be given on C1D8 (2.5mg), C1D15 (10mg), and then the target dose (30mg) on D1 of C2-12 (21-day cycles). Pts randomized to the investigator's choice will receive 375mg/m 2 IV rituximab on D1 in combination with either 90mg/m 2 IV bendamustine on D1-2 of each cycle (BR for six cycles) or 20mg/day oral lenalidomide on D1-21 of each cycle (R-Len until PD); the cycle length for BR or R-Len is 28 days. Crossover to glofitamab from the investigator's choice is permitted in pts with radiologic confirmation of PD ( Figure). The primary endpoint is progression-free survival (PFS) determined by independent review committee (IRC). Treatment comparisons will be made with a 2-sided, level 0.05 stratified log-rank test. Secondary endpoints include overall survival (OS); IRC- and investigator-assessed CR rate, objective response rate, duration of response, and duration of CR; PFS (investigator-assessed); safety; health-related quality of life including time to deterioration in physical functioning or fatigue evaluated by the EORTC QLQ-C30; glofitamab PK; and immune response to glofitamab (including presence of anti-drug antibodies). Response will be determined according to Lugano criteria (Cheson et al. J Clin Oncol 2014). Stratified hazard ratios with 95% confidence intervals (95% CI) will be calculated for PFS and OS, and odds ratios (95% CI) will be calculated for the difference in CR rate and overall response rate between treatment arms. Predictive and prognostic biomarkers (to characterize high-risk subgroups and minimal residual disease kinetics) will also be explored. An estimated 80 sites globally will enroll approximately 182 pts with R/R MCL (glofitamab n=91; BR or R-Len n=91 total) starting in Oct-Nov 2023 (APAC, US, and EU regions).

Primary central nervous system vasculitis: Description based on Chapel Hill Conference Consensus 2012 and a case report

Primary central nervous system vasculitis: Description based on Chapel Hill Conference Consensus 2012 and a case report

Moyamoya disease mimicking primary central nervous system vasculitis: A case report

Rationale: Both Moyamoya disease (MMD) and primary central nervous system vasculitis (PCNSV) cause cerebrovascular stenosis resulting in similar ischemic neurological events, although therapeutic approaches are distinct from each other. High-resonance vascular wall imaging (HRVWI) with contrast-enhanced magnetic resonance imaging (MRI) is believed to be useful to differentiate PCNSV from MMD by detecting enhancement of cerebral vascular walls. However, recent studies have reported that the vascular walls of major arteries and superficial cerebral arteries are also enhanced by HRVWI in some MMD cases. Patient concerns: A 40-year-old Chinese woman admitted to our hospital with transient weakness of the right hand for a week. A cerebral infarction of the left frontal lobe was detected by diffusion-weighted brain MRI. Magnetic resonance angiography demonstrated severe stenosis of the left middle cerebral artery (MCA) and extensive vascular narrowing. Magnetic resonance angiography incidentally taken 3 years before admission showed only a mild stenosis at the proximal portion of right MCA with intact left internal carotid artery (ICA) and MCA. Moreover, on HRVWI with contrast-enhanced MRI after admission, the wall of ICA was strongly enhanced, and the superficial cerebral arteries were also highly enhanced, which initially suggested PCNSV. Despite these findings, reticular blood vessels were confirmed by digital subtraction angiography, and p.R4810K polymorphism in the ring finger protein 213 gene was detected. Diagnosis: Based on these result, the patient was diagnosed with MMD. Interventions: Bypass surgery was performed. Outcomes: Ischemic events have never been experienced since surgery. Lessons: Our MMD case demonstrated marked asymmetrical progression of cerebral artery stenosis, and enhanced vascular walls of ICA and superficial cerebral arteries by HRVWI, which made differential diagnosis from PCNSV difficult. Genetic analysis of SNP in the ring finger protein 213 gene should be performed to rule out MMD to avoid misdiagnosis of PCNSV particularly in Asian population.

Central nervous system vasculitis

La vasculitis primaria del Sistema Nervioso Central (VPSNC) se refiere a un grupo de enfermedades que resultan de la inflamación y destrucción de los vasos sanguíneos de la médula espinal, encéfalo y meninges, tanto en el sector venoso como arterial, esto puede conducir a la oclusión o formación de aneurismas, con las consiguientes alteraciones isquémico-hemorrágicas.1 La presentación es heterogénea y poco sistematizable. El diagnóstico se establece con un cuadro clínico compatible, una angiografía o biopsia del parénquima encefálico y/o meninges que evidencien vasculitis. Presentamos el caso de un paciente portador de retrovirus con probable VPSNC con clínica compatible, hallazgos imagenológicos sugestivos, con escasa alteración de LCR y EEG.2

Intravenous immunoglobulin and rituximab in a treatment‐refractory COVID‐19‐associated central nervous system vasculitis: A case report and literature review

AbstractCentral nervous system (CNS) vasculitis is an uncommon inflammatory disease that affects the brain and spinal cord. It might present with headache, focal neurological deficits, seizures and encephalopathy. CNS vasculitis might be triggered by systemic vasculitides, connective tissue diseases, malignancies and infectious agents, including severe acute respiratory syndrome coronavirus 2. Diagnosis requires a combination of clinical presentation, serological and cerebral spinal fluid analysis, radiological findings (magnetic resonance imaging and conventional angiography), and brain biopsy. In the lack of randomized prospective studies, the treatment approach is based on retrospective cohort studies, anecdotal reports and therapeutic approaches derived from other vasculitides. In this report, we present a case of a 55‐year‐old man who presented with hemiparesthesia 39 days after coronavirus disease 2019 (COVID‐19) infection. During the disease course, the patient developed right‐sided hemiparesis and was referred to our center. Conventional angiogram suggested CNS vasculitis. After an extensive work‐up, systemic vasculitis was excluded. Despite having been treated with pulse steroid and cyclophosphamide, the patient experienced relapses. As we administered intravenous immunoglobulin and rituximab, remission was achieved both in clinical and radiological aspects. In this study, we present the first case of treatment‐refractory CNS vasculitis associated with COVID‐19, which was treated successfully with intravenous immunoglobulin and rituximab. Second, we report a review of COVID‐19‐related CNS vasculitis articles and applied treatment strategies published in English. To elucidate the mechanism of COVID‐19‐related CNS vasculitis, further research is needed.

Primary Central Nervous System Vasculitis Following Alemtuzumab Treatment for Multiple Sclerosis: A Case Report and Literature Review.

Cerebral vasculitides are often devastating conditions that require immediate diagnosis and treatment. We report a pathologically proven clinical case of primary central nervous system vasculitis in a 50-year-old man with a diagnosis of relapsing-remitting multiple sclerosis after alemtuzumab therapy, which required additional immunosuppression to control this life-threatening condition. In patients presenting subacute neurological deterioration after alemtuzumab therapy, primary central nervous system vasculitis should be considered as a differential diagnosis among other autoimmune conditions.

Selective immune suppression using interleukin-6 receptor inhibitors for management of immune-related adverse events

BackgroundManagement of immune-related adverse events (irAEs) is important as they cause treatment interruption or discontinuation, more often seen with combination immune checkpoint inhibitor (ICI) therapy. Here, we retrospectively evaluated the...