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Inflammatory2023Neuromyelitis opticaClinicalExtraglandular manifestationsLesionsSpinal cordEncephalomyelitisNMOSDAstrocytopathyNeurological disordersDisordersInitial manifestationInflammationAQP4Optic neuritisOcularPsychiatricDiseasesProteinAntibodiesSpectrum2020AcuteAntigenDiseaseVaccinePatientsDiagnosisDepartmentsVaccinationDiagnostic criteriaCommonPrevalenceFocalImportant
Inflammatory4
  • Autoimmune glial fibrillary acidic protein astrocytopathy is a form of monophasic or relapsing steroid-responsive meningoencephalomyelitis often accompanied by a hallmark radial periventricular brain MRI enhancement pattern and inflammatory cerebrospinal fluid with lymphocytic pleocytosis. (medlink.com)
  • Microfibrillar-associated protein 4 as a potential marker of acute relapse in inflammatory demyelinating diseases of the central nervous system: Pathological and clinical aspects. (cuanschutz.edu)
  • Multiple sclerosis (MS) is an autoimmune inflammatory illness that affects the central nervous system (CNS) when the body's immune system attacks and destroys myelin, the fatty substance that coats and protects nerve fibers in the brain and spinal cord. (suzannegazdamd.com)
  • However it can be associated with variable causes (demyelinating lesions, autoimmune disorders, infectious and inflammatory conditions). (openophthalmologyjournal.com)
20232
Neuromyelitis optica2
  • Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). (wikipedia.org)
  • Longitudinal lesions were frequent in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) (n = 7/7, 100%) and myelin oligodendrocyte glycoprotein-IgG-associated disorder (MOGAD) (n = 6/7, 86%), accompanied by bright spotty (n = 5/7, 71%) and central-grey-restricted (n = 4/7, 57%) T2-lesions on axial sequences, respectively. (bvsalud.org)
Clinical8
  • 15 The key role of aberrant B cell autoreactivity in SLE was revealed in a landmark murine study using a knockout gene mutation to prevent lupus mice from developing B cells, which resulted in a lack of autoantibody formation and clinical manifestations (nephritis or vasculitis). (bmj.com)
  • Given the overlap of Sjögren syndrome with many other rheumatic disorders, it is sometimes difficult to determine whether a clinical manifestation is solely a consequence of Sjögren syndrome or is due to one of its overlapping disorders. (medscape.com)
  • We believe the answer is probable, since some of the systemic disease clinical manifestations of COVID-19 cannot be explained solely by the binding of SARS-CoV-2 spike proteins with cell membranes of tissues that exhibit angiotensin-converting enzyme 2 (ACE2). (frontiersin.org)
  • Although neurologic complications of acute MPXV infections are rare, suspected cases should be reported to state, tribal, local, or territorial health departments to improve understanding of the range of clinical manifestations of and treatment options for MPXV infections during the current outbreak. (cdc.gov)
  • They are sometimes considered different diseases from Multiple Sclerosis, [4] [5] but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course. (mdwiki.org)
  • The anti-mog spectrum in children is equally variated: Out of a sample of 41 children with MOG-antibodies 29 had clinical NMOSD (17 relapsing), 8 had ADEM (4 relapsing with ADEM-ON), 3 had a single clinical event CIS, and 1 had a relapsing tumefactive disorder. (mdwiki.org)
  • I am board certified in Anatomic Pathology (National Cancer Institute, Bethesda, MD) and Dermatopathology (UT MD Anderson Cancer Center) and have a previous residency in Anatomic and Clinical Pathology (Universidad Cayetano Heredia, Lima, Peru). (mdanderson.org)
  • The recognized range of potential clinical manifestations of NMOSD has widened and is still being defined. (medscape.com)
Extraglandular manifestations2
  • 2 pSS is characterized by mononuclear infiltration and destruction of the exocrine glands, mainly the lachrymal and salivary glands, but extraglandular manifestations are also reported (eg, arthralgia, pulmonary involvement, renal tubular acidosis, etc). 1 , 3 Involvement of both the peripheral and the CNS has also been reported in pSS. (ajnr.org)
  • Rituximab has shown promise in the treatment of severe extraglandular manifestations (eg, vasculitis, cryoglobulinemia, peripheral neuropathy). (medscape.com)
Lesions3
  • Lesions may also affect the diencephalon, mostly in Aquaporin-4-Immunoglobulin-G (AQP4-IgG) NMOSD. (wikipedia.org)
  • 4 ⇓ - 6 The current data from MRI studies support an increased frequency of high signal intensity lesions in the periventricular and/or subcortical WM on FLAIR and T2-weighted imaging, observed mainly in patients with pSS and evidence of CNS disease. (ajnr.org)
  • 34062569 ) This is most closely associated with demyelinating lesions, but may also occur with other pathologies. (emcrit.org)
Spinal cord3
  • citation needed] The most common initial manifestation of the disease is inflammation of the spinal cord (myelitis). (wikipedia.org)
  • Multiple sclerosis (MS) is recognized as a demyelinating disease of the central nervous system through autoimmune involvement of the brain and spinal cord (1-4). (drnasermoghadasi.com)
  • The first step towards diagnosis and therapy is recognizing that the patient has some sort of spinal cord pathology. (emcrit.org)
Encephalomyelitis2
  • In MS and EAE or experimental autoimmune encephalomyelitis, which is an animal model of brain inflammation, there are excess glutamate levels and multiple abnormalities in glutamate- degrading enzymes, glutamate transporters, glutamate receptors, and glutamate signaling. (suzannegazdamd.com)
  • Anti-MOG associated spectrum , often clinically presented as an anti- MOG autoimmune encephalomyelitis , [21] [22] but can also appear as negative NMO or atypical multiple sclerosis. (mdwiki.org)
NMOSD6
  • However, with the discovery of disease-causing antibodies, a broader spectrum of disease manifestations has been grouped with NMO into the diagnosis of NMOSD. (wikipedia.org)
  • NMOSD is caused by an autoimmune attack on the nervous system. (wikipedia.org)
  • citation needed] NMOSD is usually caused by autoantibodies targeting aquaporin 4 (AQP4), a channel protein in the cell membrane that allows water to pass through the membrane. (wikipedia.org)
  • Thus, NMOSD involving AQP4-IgG can be considered an astrocytopathy or autoimmune astrocytic channelopathy, since the astrocytes are semi-selectively destroyed. (wikipedia.org)
  • 2 3 4 5 6 ] However, a more recent population-based survey conducted in urban Mangalore has shown a prevalence of 8.3/100,000 for MS and 2.6/100,000 for neuromyelitis optic spectrum diseases (NMOSD). (lww.com)
  • Interestingly, it seems that about 20% of patients who are seronegative for the aquaporin-4 protein and who have NMOSD features also have antibodies against myelin oligodendrocyte glycoproteins (MOGs). (medscape.com)
Astrocytopathy2
  • Autoimmune GFAP astrocytopathy manifests as a meningoencephalomyelitis (or limited form thereof) with dramatic steroid-responsiveness. (medlink.com)
  • Autoimmune GFAP astrocytopathy presentation is subacute (over days to a few weeks). (medlink.com)
Neurological disorders2
  • Autoimmune and paraneoplastic neurological disorders: A review of relevant neuroimaging findings. (cuanschutz.edu)
  • The discovery of NMDAR autoantibodies in 2007/8 by Josep Dalmau and his group and the since-emerging recognition and definition of Autoimmune Encephalitis (AE) for a widening spectrum of neurological disorders ( 4 , 5 ) have especially moved the field, because AE can be well diagnosed as a result of presenting with severe neurologic symptoms. (edupony.com)
Disorders2
  • Rarely, NMO may occur in the context of other autoimmune diseases (e.g. connective tissue disorders, paraneoplastic syndromes) or infectious diseases. (wikipedia.org)
  • Many endocrine disorders have characteristic manifestations pertaining to the eye, the classical being the exophthalmos in thyrotoxicosis. (neurologyindia.com)
Initial manifestation2
  • The second most common initial manifestation of the disease is inflammation of the optic nerve and/or optic chiasm (optic neuritis, ON). (wikipedia.org)
  • Ocular symptoms are correlated to systemic disease activity and can present as an initial manifestation of SLE. (bmj.com)
Inflammation2
  • Within a framework of the gut-brain axis, Dr. Severance will review the role of inflammation, infectious agents, food antigens, gut dysbioses, endothelial barrier instabilities and autoantibody propagation on CNS pathologies such as neurotransmitter receptor hypofunction and complement pathway-mediated synaptic pruning. (neuroimmune.org)
  • Optic neuritis (ON) is a common manifestation of multiple sclerosis (MS), and refers to inflammation of the optic nerve. (clevelandclinic.org)
AQP41
  • In more than 80% of cases, NMO is caused by immunoglobulin G autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in the central nervous system. (wikipedia.org)
Optic neuritis1
  • Longitudinally extensive myelitis or optic neuritis associated with systemic autoimmune disease. (mdwiki.org)
Ocular2
  • Ocular manifestations of SLE are common and may lead to permanent blindness from the underlying disease or therapeutic side effects. (bmj.com)
  • Secondary Sjögren syndrome is diagnosed when, in the presence of a connective-tissue disease, symptoms of oral or ocular dryness exist in addition to criterion 3, 4, or 5, above. (medscape.com)
Psychiatric2
  • 4 , 5 , 7 The estimated frequency of CNS involvement ranges from 10-60% in different reports, depending on the parameters studied (eg, patient selection, diagnostic criteria, etc). 4 ⇓ - 6 Patients with pSS can present with a wide range of focal or diffuse neurologic or psychiatric manifestations, including motor/sensory deficits, transverse myelitis, and cognitive impairment. (ajnr.org)
  • Most important, the early stages of AE are associated with various and varying, initially pure psychiatric syndromes, with neurological symptoms appearing only later in more severe stages, in addition to psychiatric syndromes ( 4 , 5 ). (edupony.com)
Diseases1
  • This extensive immune cross-reactivity between SARS-CoV-2 antibodies and different antigen groups may play a role in the multi-system disease process of COVID-19, influence the severity of the disease, precipitate the onset of autoimmunity in susceptible subgroups, and potentially exacerbate autoimmunity in subjects that have pre-existing autoimmune diseases. (frontiersin.org)
Protein2
  • I have written about how lingering viral fragments and in particular spike protein and mRNA, originating either post infection or post vaccine, are strong drivers of molecular mimicry and autoimmune disease. (suzannegazdamd.com)
  • Increased cAMP promotes protein kinase A activity, among other effectors, and, in turn, leads to cyst growth by promoting proliferation and fluid secretion of cyst-lining cells through chloride and aquaporin channels in ADPKD kidneys. (basicmedicalkey.com)
Antibodies4
  • In more than 80% of cases, IgG autoantibodies against aquaporin-4 (anti-AQP4+) are the cause, and in 10-40% of the remaining cases, IgG antibodies against MOG are the cause. (wikipedia.org)
  • This case highlights the difficulties in reaching a rheumatological diagnosis in the early stages with typical negative antibodies, and shows a rare neurological manifestation of a systemic rheumatological condition. (bvsalud.org)
  • In this mechanism, antibodies formed against SARS-CoV-2 would also bind to human tissue proteins leading to autoimmune reactivity. (frontiersin.org)
  • The discovery of new antibodies has improved our understanding of the pathology of the disease. (lww.com)
Spectrum2
  • Methods To assess GlyRα1-IgGs as biomarkers of SPS spectrum among patients and controls, specimens were tested using cell-based assays (binding [4°C] and modulating [antigen endocytosing, 37°C]). Medical records of seropositive patients were reviewed. (neurology.org)
  • Results GlyRα1-IgG (binding antibody) was detected in 21 of 247 patients with suspected SPS spectrum (8.5%) and in 8 of 190 healthy subject sera (4%) but not CSF. (neurology.org)
20201
Acute1
  • The pattern of RNFL thinning most specific to MS is temporal.3 RNFL can appear normal in the acute setting but starts to decline within 4 weeks of ON. (clevelandclinic.org)
Antigen1
  • His research focuses on what provokes relapses and remissions in multiple sclerosis (MS), and on the quest for antigen specific therapy in autoimmune disease. (neuroimmune.org)
Disease3
  • 13 Aberrant epigenetic regulation including DNA methylation, histone modifications and microRNA-mediated regulation may contribute to the complex array of immune abnormalities and disease manifestations in SLE. (bmj.com)
  • Sjögren syndrome is a chronic systemic autoimmune disease that can be classified as primary Sjögren syndrome (pSS) when presenting in isolation or secondary when related to another connective tissue disease. (ajnr.org)
  • 4 ⇓ - 6 Although involvement of the peripheral nervous system is a well-documented feature of the disease, the prevalence, the type, and the underlying mechanism of CNS involvement remain unclear. (ajnr.org)
Vaccine1
  • An insufficiently vetted vaccine might mean trading freedom from COVID-19 to an autoimmune assault in the future. (frontiersin.org)
Patients3
  • We found propranolol to be effective in controlling many of the manifestations of neurogenic fever in our patients with severe TBI. (neurologyindia.com)
  • 4.Sahraian MA, Azimi A, Navardi S, Ala S, Naser Moghadasi A . Evaluation of the rate of COVID-19 infection, hospitalization and death among Iranian patients with multiple sclerosis . (drnasermoghadasi.com)
  • Objective improvements in SPS neurologic symptoms were recorded in 16 of 18 patients who received first-line immunotherapy (89%, 9/10 treated with corticosteroids, 8/10 treated with IVIg, 3/4 treated with plasma exchange, and 1 treated with rituximab). (neurology.org)
Diagnosis1
  • 1, 4, 7, 8 A clinician should further narrow the diagnosis based on the timing of symptoms and associated features (Below). (clevelandclinic.org)
Departments1
Vaccination1
  • Third, are there any concerns for autoimmune development with either infection or vaccination with SARS-CoV-2? (frontiersin.org)
Diagnostic criteria1
  • This was strongly reinforced by single case reports of successful immune modulatory treatments of cases of Autoimmune Psychosis (AP), with AP cases not fulfilling the diagnostic criteria of AE ( 8 - 13 ). (edupony.com)
Common2
  • Keratoconjunctivitis sicca is the most common manifestation. (bmj.com)
  • Hypernatremia is a common electrolyte problem that is defined as a rise in serum sodium concentration to a value exceeding 145 mmol/L.[1, 2, 3] It is strictly defined as a hyperosmolar condition caused by a decrease in total body water (TBW)[4] relative to electrolyte content. (medscape.com)
Prevalence1
  • 4-6 The prevalence of SLE is different between age, gender, geographic and racial distributions. (bmj.com)
Focal1
  • MRI spine showed focal T2 hyperintensity in the left hemicord at C3-4 level. (bvsalud.org)
Important1
  • It is characterized by demyelination and varying degrees of axonal loss, which is an important component of the pathology of MS. (suzannegazdamd.com)