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TNF-Blockade for Primary Stroke Prevention in Adenosine Deaminase 2 Deficiency: A Case Series
We present a family with multiple affected children to highlight the importance of TNF blockade not just as secondary stroke prevention but also as primary stroke prevention in genetically affected but clinically asymptomatic patients. Methods A proband with recurrent cryptogenic strokes was referred for evaluation at the NIH CC. The parents and 3 clinically asymptomatic siblings were also evaluated. Results The proband was diagnosed with DADA2 based on biochemical testing; her antiplatelet therapies were discontinued, and she was started on TNF blockade for secondary stroke prevention. Her 3 asymptomatic siblings were s...
Source: Neurology Neuroimmunology and Neuroinflammation - March 20, 2023 Category: Neurology Authors: Soldatos, A., Toro, C., Hoffmann, P., Romeo, T., Deuitch, N., Brofferio, A., Aksentijevich, I., Kastner, D. L., Ombrello, A. K. Tags: Childhood stroke, Stroke in young adults, Stroke prevention, Vasculitis, All Genetics Clinical/Scientific Note Source Type: research

CNS Antigen-Specific Neuroinflammation Attenuates Ischemic Stroke With Involvement of Polarized Myeloid Cells
Discussion CNS autoantigen-specific autoimmunity has a protective influence on primary tissue damage after experimental stroke, indicating a very early involvement of CNS antigen-specific, myeloid cell-associated anti-inflammatory immune mechanisms that mitigate ischemic injury in the acute EAE phase.
Source: Neurology Neuroimmunology and Neuroinflammation - June 8, 2022 Category: Neurology Authors: Guse, K., Hagemann, N., Thiele, L., Remlinger, J., Salmen, A., Hoepner, R., Keller, I., Meyer, P., Grandgirard, D., Leib, S. L., Vassella, E., Locatelli, G., Hermann, D. M., Chan, A. Tags: All Immunology, Multiple sclerosis, Infarction Research Article Source Type: research

Neurofilament Light Chain Levels in Multiple Sclerosis Correlate With Lesions Containing Foamy Macrophages and With Acute Axonal Damage
Discussion Our results demonstrated that active and mixed white matter MS lesions with foamy microglia show high acute axonal damage and correlate with elevated CSF NfL levels. Our data support the use of this biomarker to monitor inflammatory demyelinating lesion activity with axonal damage in MS.
Source: Neurology Neuroimmunology and Neuroinflammation - March 3, 2022 Category: Neurology Authors: van den Bosch, A., Fransen, N., Mason, M., Rozemuller, A. J., Teunissen, C., Smolders, J., Huitinga, I. Tags: Multiple sclerosis Research Article Source Type: research

Targeted RNA Sequencing of VZV-Infected Brain Vascular Adventitial Fibroblasts Indicates That Amyloid May Be Involved in VZV Vasculopathy
Discussion Gene expression profiles and pathway enrichment analysis of VZV-infected HBVAFs, as well as phenotypic studies, reveal features of pathologic vascular remodeling (e.g., increased cell migration and changes in the extracellular matrix) that can contribute to cerebrovascular disease. Furthermore, the discovery of amyloid-associated transcriptional pathways and intracellular amyloid deposition in HBVAFs raise the possibility that VZV vasculopathy is an amyloid disease. Amyloid deposition may contribute to cell death and loss of vascular wall integrity, as well as potentiate chronic inflammation in VZV vasculopathy,...
Source: Neurology Neuroimmunology and Neuroinflammation - November 10, 2021 Category: Neurology Authors: Bubak, A. N., Como, C. N., Hassell, J. E., Mescher, T., Frietze, S. E., Niemeyer, C. S., Cohrs, R. J., Nagel, M. A. Tags: Vasculitis, Viral infections, All Cerebrovascular disease/Stroke, All Cognitive Disorders/Dementia, Gene expression studies Article Source Type: research

Primary Angiitis of the CNS: A Systematic Review and Meta-analysis
Discussion PACNS is associated with disabling symptoms, frequent relapses, and significant mortality. Differences in symptoms and neuroimaging results and low overlap between biopsy and angiogram suggest that biopsy- and angiogram-confirmed cases represent different histopathologic types of PACNS. The optimal treatment is unknown.
Source: Neurology Neuroimmunology and Neuroinflammation - October 18, 2021 Category: Neurology Authors: Beuker, C., Strunk, D., Rawal, R., Schmidt-Pogoda, A., Werring, N., Milles, L., Ruck, T., Wiendl, H., Meuth, S., Minnerup, H., Minnerup, J. Tags: Vasculitis, All Cerebrovascular disease/Stroke Views [amp ] Reviews Source Type: research

Targeted RNA Sequencing of Formalin-Fixed, Paraffin-Embedded Temporal Arteries From Giant Cell Arteritis Cases Reveals Viral Signatures
Discussion The detection of viral and immune activation pathways in GCA TAs supports a role for virus infection in GCA pathogenesis. In addition, the detection of viral pathways in control/VZV-positive TAs, along with vascular remodeling pathways, suggests that these samples may represent early infection with progression to clinical disease, depending on host and other environmental factors.
Source: Neurology Neuroimmunology and Neuroinflammation - September 7, 2021 Category: Neurology Authors: Bubak, A. N., Mescher, T., Mariani, M., Frietze, S. E., Hassell, J. E., Niemeyer, C. S., Como, C. N., Burnet, A. M., Subramanian, P. S., Cohrs, R. J., Mahalingam, R., Nagel, M. A. Tags: All Immunology, Vasculitis, Viral infections, All Cerebrovascular disease/Stroke, Gene expression studies Article Source Type: research

Vascular Involvement in Neurosarcoidosis: Early Experiences From Intracranial Vessel Wall Imaging
We report our preliminary experience in patients with NS who underwent high-resolution vessel wall imaging (VWI). Methods A total of 13 consecutive patients with NS underwent VWI. Images were analyzed by 2 neuroradiologists in consensus. The assessment included segment-wise evaluation of larger- and medium-sized vessels (internal carotid artery, M1-M3 middle cerebral artery; A1-A3 anterior cerebral artery; V4 segments of vertebral arteries; basilar artery; and P1-P3 posterior cerebral artery), lenticulostriate perforator vessels, and medullary and deep cerebral veins. Cortical veins were not assessed due to flow-related a...
Source: Neurology Neuroimmunology and Neuroinflammation - August 4, 2021 Category: Neurology Authors: Bathla, G., Abdel-Wahed, L., Agarwal, A., Cho, T. A., Gupta, S., Jones, K. A., Priya, S., Soni, N., Wasserman, B. A. Tags: MRI, DWI, Other cerebrovascular disease/ Stroke, Vasculitis Article Source Type: research

Imaging Features of Susac Syndrome on High-Resolution Intracranial Vessel Wall MRI
Source: Neurology Neuroimmunology and Neuroinflammation - April 16, 2021 Category: Neurology Authors: Yahyavi-Firouz-Abadi, N., Kiczek, M., Zeiler, S. R., Wasserman, B. A. Tags: Stroke in young adults, MRI, Vasculitis, All Demyelinating disease (CNS) Clinical/Scientific Notes Source Type: research

Systemic Inflammation Is Associated With Neurologic Involvement in Pediatric Inflammatory Multisystem Syndrome Associated With SARS-CoV-2
Conclusions Broad neurologic features were found in 12% patients with PIMS-TS. By 3-month follow-up, half of these surviving children had recovered fully without neurologic impairment. Significantly higher systemic inflammatory markers were identified in children with neurologic involvement and in those who had not recovered fully.
Source: Neurology Neuroimmunology and Neuroinflammation - April 13, 2021 Category: Neurology Authors: Sa, M., Mirza, L., Carter, M., Carlton Jones, L., Gowda, V., Handforth, J., Hedderly, T., Kenny, J., Lascelles, K., Lin, J.-P., Lumsden, D., McDougall, M., Miller, O., Rossor, T., Shivamurthy, V., Siddiqui, A., Singh, R., Tang, S., White, M., Byrne, S., L Tags: Childhood stroke, Encephalitis, Prognosis, All Neuropsychology/Behavior Article Source Type: research

Presentations and mechanisms of CNS disorders related to COVID-19
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the cause of the coronavirus disease 2019 (COVID-19) pandemic. In addition to severe respiratory symptoms, there are a growing number of reports showing a wide range of CNS complications in patients with COVID-19. Here, we review the literature on these complications, ranging from nonspecific symptoms to necrotizing encephalopathies, encephalitis, myelitis, encephalomyelitis, endotheliitis, and stroke. We postulate that there are several different mechanisms involved in COVID-19–associated CNS dysfunction, particularly activation of inflammatory and thro...
Source: Neurology Neuroimmunology and Neuroinflammation - December 11, 2020 Category: Neurology Authors: Bodro, M., Compta, Y., Sanchez-Valle, R. Tags: Other cerebrovascular disease/ Stroke, Encephalitis, COVID-19 Views [amp ] Reviews Source Type: research

A case of neonatal onset multisystem inflammatory disease supporting a role of interleukin-1{beta} in moyamoya syndrome
Moyamoya syndrome (MMS) is a chronic progressive cerebrovascular condition characterized by bilateral stenosis/occlusion of the terminal internal carotid artery (ICA) often extending to neighboring cerebral arteries with prominent collateral circulation and increased risk of hemorrhage. Symptoms include stroke, TIA, and headache.1 MMS's etiology is unknown. However, experimental evidence from arterial smooth muscle cells (SMC) derived from patients with MMS has suggested that interleukin-1 beta (IL-1β)-dependent prostaglandin overproduction plays a crucial role in its pathogenesis.2 Neonatal onset multisystem inflamma...
Source: Neurology Neuroimmunology and Neuroinflammation - October 27, 2020 Category: Neurology Authors: Wohlrab, F., Bauknecht, C., Meisel, C., Dreier, J. P. Tags: Stroke in young adults, All Immunology, All Pediatric, Other neurocutaneous disorders Clinical/Scientific Notes Source Type: research

CNS inflammatory vasculopathy with antimyelin oligodendrocyte glycoprotein antibodies in COVID-19
A 44-year-old right-handed woman reported a gradual onset of right hand incoordination seven days after the onset of minor respiratory symptoms and pruritus due to COVID-19 infection. Over 48 hours, the patient developed word-finding difficulties and progression in right arm weakness leading to presentation to the emergency department as a suspected stroke.
Source: Neurology Neuroimmunology and Neuroinflammation - June 9, 2020 Category: Neurology Authors: Pinto, A. A., Carroll, L. S., Nar, V., Varatharaj, A., Galea, I. Tags: MRI, Other cerebrovascular disease/ Stroke, Encephalitis, COVID-19 Clinical/Scientific Notes Source Type: research

The rare case of a 20-year-old male with rapidly progressive primary angiitis of the CNS with a good outcome
We describe the treatment and outcome of a patient with rapidly progressive PACNS who returned to his premorbid baseline with an mRS of 0.
Source: Neurology Neuroimmunology and Neuroinflammation - June 8, 2020 Category: Neurology Authors: Graham, E., Shoemaker, T., Stefoski, D., Kontzialis, M., Naumaan, A., Garg, R. K. Tags: Stroke in young adults, Other cerebrovascular disease/ Stroke, Autoimmune diseases, Vasculitis, Critical care Clinical/Scientific Notes Source Type: research

A case of cerebral vasculitis due to neurobartonellosis
We report a case of a 60-year-old right-handed woman with hypertension, hyperlipidemia, and hypothyroidism who presented with a three-week history of recurrent thunderclap headaches accompanied by photophobia, phonophobia, nausea, and vomiting. She reported one brief episode of slurred speech, expressive aphasia, right facial droop, and right hemiparesis suggestive of a TIA. Family history was remarkable for primary angiitis of the CNS (PACNS) in the mother. Neurologic examination was unremarkable. CT of the head was negative; CT angiography (CTA) of the head and neck suggested fibromuscular dysplasia in bilateral cervical...
Source: Neurology Neuroimmunology and Neuroinflammation - June 3, 2020 Category: Neurology Authors: Poursheykhi, M., Mithani, F., Garg, T., Cajavilca, C., Jaijakul, S., Fung, S., Klucznik, R., Gadhia, R. Tags: All Headache, Vasculitis, All Cerebrovascular disease/Stroke Clinical/Scientific Notes Source Type: research

Rituximab, MS, and pregnancy
Conclusion We observed no increase in adverse pregnancy outcomes compared with expected national incidence rates and remarkably little disease activity in RTX-treated women with MS, particularly when compared with periconceptional natalizumab-treated cohorts. However, larger studies are needed to fully assess the safety of RTX use before pregnancy, especially risks associated with prolonged B-cell depletion and hypogammaglobulinemia. Until these data are available, we recommend restricting RTX use before pregnancy to women who require highly effective MS treatments. Classification of evidence This study provides Class IV ...
Source: Neurology Neuroimmunology and Neuroinflammation - April 30, 2020 Category: Neurology Authors: Smith, J. B., Hellwig, K., Fink, K., Lyell, D. J., Piehl, F., Langer-Gould, A. Tags: Multiple sclerosis Article Source Type: research

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