scholarly journals Anti-MOG encephalitis mimicking small vessel CNS vasculitis

2019 ◽  
Vol 6 (2) ◽  
pp. e538 ◽  
Author(s):  
Kristina Patterson ◽  
Estibaliz Iglesias ◽  
Maclean Nasrallah ◽  
Verónica González-Álvarez ◽  
Mariona Suñol ◽  
...  
Keyword(s):  
Brain Biopsy ◽  
Lymphocytic Infiltration ◽  
Small Vessel ◽  
Response To Treatment ◽  
Cns Vasculitis ◽  
Small Vessels ◽  
Retrospective Assessment ◽  
Relapsing Remitting ◽  
A Cell ◽  
Mog Antibodies

ObjectiveTo report 2 patients with anti–myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis who were initially misdiagnosed with small vessel primary CNS vasculitis.MethodsReview of symptoms, MRI and neuropathologic features, and response to treatment. MOG antibodies were determined in serum and CSF using a cell-based assay.ResultsSymptoms included fever, headache, and progressive mental status changes and focal neurologic deficits. CSF studies revealed lymphocytic pleocytosis, and both patients had abnormal brain MRIs. Brain biopsy samples showed prominent lymphocytic infiltration of the wall of small vessels; these findings initially suggested small vessel CNS vasculitis, and both patients were treated accordingly. Although 1 patient had a relapsing-remitting course not responsive to cyclophosphamide, the other one (also treated with cyclophosphamide) did not relapse. Retrospective assessment of serum and CSF demonstrated MOG antibodies in both cases, and review of biopsy specimens showed absence of fibrinoid necrosis (a pathologic requirement for small vessel CNS vasculitis).ConclusionsAnti–MOG-associated encephalitis can be mistaken for small vessel CNS vasculitis. This is important because the diagnosis of anti–MOG-associated encephalitis does not require brain biopsy and can be established with a serologic test.

scholarly journals Pearls & Oy-sters: The critical role of histopathology in diagnosing cancer-associated necrotizing CNS vasculitis

Neurology ◽  
2018 ◽  
Vol 90 (17) ◽  
pp. 808-811
Author(s):  
Joshua Sheehan ◽  
Jessica Tate ◽  
Ryan Mott ◽  
Carol Geer ◽  
Rachel Wolfe ◽  
...  
Keyword(s):  
Ductal Carcinoma ◽  
Critical Role ◽  
Growth Factor Receptor ◽  
Brain Biopsy ◽  
Brain Lesions ◽  
Response To Treatment ◽  
Newly Diagnosed ◽  
Necrotizing Vasculitis ◽  
Primary Malignancy ◽  
Cns Vasculitis

ObjectiveTo highlight the importance of a broad differential and histopathologic confirmation in patients with newly diagnosed cancer with brain lesions atypical for CNS metastasis.MethodsWe report 2 cases of biopsy-proven CNS vasculitis in patients undergoing treatment for a newly diagnosed nonmetastatic cancer. Comprehensive medical record review was performed to identify the clinical presentation, representative neuroimaging, histopathologic features, and response to treatment.ResultsPatient 1 presented 1 month into induction therapy of malignant vaginal squamous cell carcinoma (stage 3, T2N1M0) with acute episodic left-sided hemiparesis due to seizure activity progressing to severe encephalopathy. Imaging revealed a right frontoparietal lesion while systemic workup was unrevealing. Biopsy demonstrated necrotizing vasculitis. Patient 2 presented 6 months after diagnosis of right breast invasive ductal carcinoma (stage IIa, T2N0M0, estrogen receptor–positive, progesterone receptor–positive, human epidermal growth factor receptor–2 positive) with subacute bifrontal headaches with associated phonophobia. Imaging showed hyperintense lesions involving the right temporoparietal region and systemic workup was unrevealing. Brain biopsy showed a necrotizing vasculitis. Patient 1 was treated with methyprednisolone and plasmapheresis and patient 2 was treated with prednisone. Both patients showed complete resolution of symptoms shortly after treatment and improvement on imaging.ConclusionsThese cases highlight the importance of comprehensive evaluation of new brain lesions in patients with nonmetastatic solid tumors. Characteristics of new brain lesions in patients with cancer that should raise suspicion of diagnoses other than brain metastasis include (1) primary malignancy without regional or distant metastasis, (2) imaging without discrete mass-like enhancement, and (3) cortically based location of lesions not at the gray–white matter junction.

scholarly journals Decreased Frequency of Circulating Myelin Oligodendrocyte Glycoprotein B Lymphocytes in Patients with Relapsing-Remitting Multiple Sclerosis

2015 ◽  
Vol 2015 ◽  
pp. 1-12 ◽  
Author(s):  
Annie Elong Ngono ◽  
Maud Lepetit ◽  
Markus Reindl ◽  
Alexandra Garcia ◽  
Flora Guillot ◽  
...  
Keyword(s):  
B Cells ◽  
B Lymphocytes ◽  
Healthy Individuals ◽  
Relapsing Remitting ◽  
Antibody Titers ◽  
A Cell ◽  
Mog Antibodies ◽  
Specific Alteration ◽  
Relapsing Remitting Multiple Sclerosis

Although there is no evidence for a role of anti-MOG antibodies in adult MS, no information on B lymphocytes with MOG-committed BCR is available. We report here on the frequency of anti-MOG B cells forming rosettes with polystyrene beads (BBR) covalently bound to the extracellular domain of rhMOG in 38 relapsing-remitting patients (RRMS) and 50 healthy individuals (HI). We show a substantial proportion of circulating anti-MOG-BBR in both RRMS and HI. Strikingly, MOG-specific B cells frequencies were lower in MS than in HI. Anti-MOG antibodies measured by a cell-based assay were not different between MS patients and controls, suggesting a specific alteration of anti-MOG B cells in MS. Although anti-MOG-BBR were higher in CNS fluid than in blood, no difference was observed between MS and controls. Lower frequency of MOG-BBR in MS was not explained by an increased apoptosis, but a trend for lower proliferative capacity was noted. Despite an efficient B cell transmigration across brain derived endothelial cells, total and anti-MOG B cells transmigration was similar between MS and HI. The striking alteration in MOG-specific B cells, independent of anti-MOG antibody titers, challenges our view on the role of MOG-specific B cells in MS.

Progressive Leukodystrophy-Like Demyelinating Syndromes with MOG-Antibodies in Children: A Rare Under-Recognized Phenotype

2021 ◽  
Author(s):  
Elise Yazbeck ◽  
Hélène Maurey ◽  
Carole Leroy ◽  
Philippe Horellou ◽  
Silvia Napuri ◽  
...  
Keyword(s):  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Active Immunotherapy ◽  
Oligoclonal Bands ◽  
Second Child ◽  
Fluid Analysis ◽  
Appropriate Treatment ◽  
Neurological Prognosis ◽  
Mog Antibodies

AbstractAcquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.

scholarly journals Arterial hypertension and unusual ascending aortic dilatation in a neonate with acute kidney injury: mechanistic computer modelling

2018 ◽  
Author(s):  
Sanjay R Kharche
Keyword(s):  
Acute Kidney Injury ◽  
Kidney Injury ◽  
Ascending Aorta ◽  
Small Vessel ◽  
Aortic Dilatation ◽  
Small Vessels ◽  
Left Ventricle Hypertrophy ◽  
Vessel Resistance ◽  
Underlying Mechanisms ◽  
Ventricle Hypertrophy

Background: Neonatal asphyxia caused acute kidney injury and severe hypertension in a newborn patient. An unusually dilatated ascending aorta developed within a few weeks. Dialysis and hypertensive treatment led to partial recovery of the aortic diameters. It was hypothesized that the aortic dilatation may be associated with cardiovascular changes induced by the acute kidney injury. Mathematical modelling was used to better understand the underlying mechanisms of hypertension and aortic dilatation.Methods: Patient observation included systolic blood pressure recording and echocardiographic exams. To explore underlying mechanisms of aortic dilatation and hypertension, a previous whole-body lumped parameter hemodynamics model was adapted to this study. Computer simulations were designed to permit dissection of individual mechanisms. The hypertension inducing effects of altering systemic vascular resistances, stiffnesses, and heart rate on blood flows and pressures were simulated.Results: In agreement with our clinical diagnosis, the mathematical model showed that an increase of systemic small vessel resistance is the prime cause of hypertension. Further, aortic stiffening may also cause hypertension, it was found to be secondary to the potency of systemic small vessel resistance. The cardiac output, as quantified using pressure-volume loop area, reduced significantly due to hypertension. Simultaneous left ventricle hypertrophy and small vessel blocking increased ascending aorta blood flow as well as pressure indicating an enlarged ascending aorta. In contrast, increased arterial stiffness appeared to lower the aortic blood flow and pressures.Conclusions and discussion: Systemic small vessel resistance is an important factor in arterial hypertension, and may also be a key clinical therapeutic target. Left ventricle hypertrophy may also be simultaneously ameliorated when treating systemic small vessels. Treatment of arterial stiffness appears to provide significant benefit but may be secondary to treatment of the systemic small vessels. The quantitative grading of pathophysiological mechanisms provided by the modelling may contribute to treatment recommendations. Further development and individualization of the model will augment its applicability in clinical practice.

Small vessel vasculitis

2020 ◽  
pp. 4573-4579
Author(s):  
Richard A. Watts
Keyword(s):  
Antineutrophil Cytoplasmic Antibody ◽  
Consensus Conference ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Iga Vasculitis ◽  
Small Vessels ◽  
Chapel Hill ◽  
Familial Association ◽  
Chapel Hill Consensus Conference ◽  
Inflammatory Drugs

Small vessel vasculitis is vasculitis affecting predominately small intraparenchymal arteries, arterioles, capillaries, and venules. There are two main types: antineutrophil cytoplasmic antibody associated and immune complex mediated. The ANCA associated vasculitides are discussed in chapter 19.3 IgA vasculitis (IgAV) was formerly known as Henoch Schönlein purpura. The revised nomenclature reflects the importance of IgA vasculitis in pathogenesis. The Chapel Hill Consensus Conference defined IgA vasculitis as ‘vasculitis with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles)’. IgA vasculitis often involves skin and gut, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur. Its aetiology is unknown, but it frequently occurs after an infection several days to weeks before. The most frequently isolated organism is beta-haemolytic streptococcus. Drugs such as a penicillin, ampicillin, erythromycin, and non-steroidal anti-inflammatory drugs have been reported as precipitating agents. There is an association with HLA-DRB1*01 in Caucasians and there appears to be a familial association.

Vascular basement membrane alterations and β-amyloid accumulations in an animal model of cerebral small vessel disease

2017 ◽  
Vol 131 (10) ◽  
pp. 1001-1013 ◽  
Author(s):  
Friederike Held ◽  
Alan W.J. Morris ◽  
Daniel Pirici ◽  
Solveig Niklass ◽  
Matthew M.G. Sharp ◽  
...  
Keyword(s):  
Blood Vessels ◽  
Small Vessel Disease ◽  
Collagen Iv ◽  
Cerebral Small Vessel Disease ◽  
Basement Membranes ◽  
Small Vessel ◽  
Small Vessels ◽  
Vessel Disease ◽  
Percentage Area ◽  
Β Amyloid

Non-amyloid cerebral small vessel disease (CSVD) and cerebral amyloid angiopathy (CAA) may be interrelated through the damaged basement membranes (BMs) and extracellular matrix changes of small vessels, resulting in a failure of β-amyloid (Aβ) transport and degradation. We analyzed BM changes and the pattern of deposition of Aβ in the walls of blood vessels in spontaneously hypertensive stroke-prone rats (SHRSP), a non-transgenic CSVD model. In 45 SHRSP and 38 Wistar rats aged 18 to 32 weeks: (i) the percentage area immunostained for vascular collagen IV and laminin was quantified; (ii) the capillary BM thickness as well as endothelial and pericyte pathological changes were analysed using transmission electron microscopy (TEM); and (iii) the presence of vascular Aβ was assessed. Compared with controls, SHRSP exhibited a significantly higher percentage area immunostained with collagen IV in the striatum and thalamus. SHRSP also revealed an age-dependent increase of the capillary BM thickness and of endothelial vacuoles (caveolae) within subcortical regions. Endogenous Aβ deposits in the walls of small blood vessels were observed in the cortex (with the highest incidence found within fronto-parietal areas), striatum, thalamus and hippocampus. Vascular β-amyloid accumulations were frequently detected at sites of small vessel wall damage. Our data demonstrate changes in the expression of collagen IV and of the ultrastructure of BMs in the small vessels of SHRSP. Alterations are accompanied by vascular deposits of endogenous Aβ. Impaired β-amyloid clearance along perivascular and endothelial pathways and failure of extracellular Aβ degradation may be the key mechanisms connecting non-amyloid CSVD and CAA.

Visualization of mouse spinal cord intramedullary arteries using phase- and attenuation-contrast tomographic imaging

2016 ◽  
Vol 23 (4) ◽  
pp. 966-974 ◽  
Author(s):  
Yong Cao ◽  
Xianzhen Yin ◽  
Jiwen Zhang ◽  
Tianding Wu ◽  
Dongzhe Li ◽  
...  
Keyword(s):  
Spinal Cord ◽  
High Resolution ◽  
Synchrotron Radiation ◽  
Three Dimensional ◽  
Small Vessel ◽  
Imaging Method ◽  
Central Sulcus ◽  
Contrast Imaging ◽  
Mouse Spinal Cord ◽  
Small Vessels

Many spinal cord circulatory disorders present the substantial involvement of small vessel lesions. The central sulcus arteries supply nutrition to a large part of the spinal cord, and, if not detected early, lesions in the spinal cord will cause irreversible damage to the function of this organ. Thus, early detection of these small vessel lesions could potentially facilitate the effective diagnosis and treatment of these diseases. However, the detection of such small vessels is beyond the capability of current imaging techniques. In this study, an imaging method is proposed and the potential of phase-contrast imaging (PCI)- and attenuation-contrast imaging (ACI)-based synchrotron radiation for high-resolution tomography of intramedullary arteries in mouse spinal cord is validated. The three-dimensional vessel morphology, particularly that of the central sulcus arteries (CSA), detected with these two imaging models was quantitatively analyzed and compared. It was determined that both PCI- and ACI-based synchrotron radiation can be used to visualize the physiological arrangement of the entire intramedullary artery network in the mouse spinal cord in both two dimensions and three dimensions at a high-resolution scale. Additionally, the two-dimensional and three-dimensional vessel morphometric parameter measurements obtained with PCI are similar to the ACI data. Furthermore, PCI allows efficient and direct discrimination of the same branch level of the CSA without contrast agent injection and is expected to provide reliable biological information regarding the intramedullary artery. Compared with ACI, PCI might be a novel imaging method that offers a powerful imaging platform for evaluating pathological changes in small vessels and may also allow better clarification of their role in neurovascular disorders.

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