The Anti-NMDA Receptor Encephalitis Foundation Newsletter

A podcast answering your questions about Covid-19 (Coronavirus) and encephalitis. Filmed on March 20.

 




PubMed comprises more than 30 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

 




She was a patient without a diagnosis. From the spring of 2016 to that winter, Emily Chan bounced from the neurology department to the psychiatric department and back again as health-care . . .

 




Athletic, coordinated, confident. That’s how Joy would describe her 5-year-old son Aroon, who loved to emulate ninjas with feisty kicks and had been scouted as a model. But suddenly Aroon was tripping down stairs and wobbling like a newborn fawn when he walked. Aroon’s primary doctor near their Stockton home said the kindergartner seemed fine. “But my mommy instinct knew he wasn’t,” Joy says. Test after test failed to explain Aroon’s confusing symptoms, which gradually became more acute. Aroon began to erupt in violent tantrums: flailing wildly, pulling his hair, holding his breath until he turned blue. “Mom, I’m so sorry,” Joy recalls Aroon explaining. “There is something inside of me that I can’t control.” That’s when Joy brought Aroon to UCSF Benioff Children’s Hospital Oakland. He was diagnosed with anti-NMDA receptor encephalitis, a rare disease that causes the immune system to attack parts of the brain that control judgment, perception of reality, memory, and basic functions like breathing and swallowing. During the nearly one year Aroon spent in the hospital, his condition deteriorated to the point that he became unable to move and could communicate only by blinking. With each victory seemed to come a setback. Aroon would regain some abilities only to lose them again, slipping further away. One constant in Aroon’s life was art. At UCSF Benioff Oakland, child life specialists and artists-in-residence work with patients to support their healing through creative expression. “That team played such an important role in his recovery,” Joy says. “Consistently doing art was crucial. No matter how severe Aroon’s condition, the staff got creative and found a way to make art with him.” An adaptive device that extended the length of a regular paintbrush, for example, allowed Aroon to create art with just small movements of his fingertips. Aroon’s caregivers were also a vital part of the family’s journey. Nurses blasted music and sang with Aroon, celebrated his birthday, and even took care of his younger brother so Joy could rest. “I had nights when I just cried and cried,” Joy says. “The staff was so supportive. They became like family.” Through diligent daily therapies, the compassionate care of his multidisciplinary medical team, and the unwavering support of his family, Aroon eventually became well enough to return home.

 




With almost $200 million in NIH funding, UAB scientists are at the forefront of research that is shaping the future of health and healthcare.

 







PubMed comprises more than 30 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

 




Significance Antibodies against neuronal receptors and synaptic proteins are associated with a group of ill-defined central nervous system (CNS) autoimmune diseases named autoimmune encephalitides (AE), characterized by an abrupt onset of seizures and movement and psychiatric deficits. How these antibodies enter the brain to trigger neuroinflammation, and how they affect the function of neural circuits, remains poorly understood. Here, we demonstrate that Th17 lymphocytes are critical for entry of autoantibodies into the CNS, persistent microglia activation, and neurophysiological deficits in odor processing, in a mouse model of postinfectious autoimmune encephalitis triggered by multiple infections with group A Streptococcus. Our findings emphasize the critical role that Th17 lymphocytes play in disease pathogenesis to impair CNS function in AE syndromes. Abstract Antibodies against neuronal receptors and synaptic proteins are associated with a group of ill-defined central nervous system (CNS) autoimmune diseases termed autoimmune encephalitides (AE), which are characterized by abrupt onset of seizures and/or movement and psychiatric symptoms. Basal ganglia encephalitis (BGE), representing a subset of AE syndromes, is triggered in children by repeated group A Streptococcus (GAS) infections that lead to neuropsychiatric symptoms. We have previously shown that multiple GAS infections of mice induce migration of Th17 lymphocytes from the nose into the brain, causing blood–brain barrier (BBB) breakdown, extravasation of autoantibodies into the CNS, and loss of excitatory synapses within the olfactory bulb (OB). Whether these pathologies induce functional olfactory deficits, and the mechanistic role of Th17 lymphocytes, is unknown. Here, we demonstrate that, whereas loss of excitatory synapses in the OB is transient after multiple GAS infections, functional deficits in odor processing persist. Moreover, mice lacking Th17 lymphocytes have reduced BBB leakage, microglial activation, and antibody infiltration into the CNS, and have their olfactory function partially restored. Th17 lymphocytes are therefore critical for selective CNS entry of autoantibodies, microglial activation, and neural circuit impairment during postinfectious BGE. Footnotes ↵1To whom correspondence may be addressed. Email: franks{at}neuro.duke.edu or da191{at}cumc.columbia.edu. Author contributions: M.P.P., T.C., K.M.F., and D.A. designed research; M.P.P., K.A.B., C.R.W., S.C., T.C., K.M.F., and D.A. performed research; K.A.B. contributed new reagents/analytic tools; M.P.P., K.A.B., C.R.W., S.C., T.C., K.M.F., and D.A. analyzed data; and M.P.P., K.A.B., C.R.W., T.C., K.M.F., and D.A. wrote the paper. The authors declare no competing interest. This article is a PNAS Direct Submission. This article contains supporting information online at https://www.pnas.org/lookup/suppl/doi:10.1073/pnas.1911097117/-/DCSupplemental. Published under the PNAS license.

 




Scientists are looking more closely at how viruses and infections could influence our minds.

 




PubMed comprises more than 30 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

 




My name is Kertisha Brabson. I was left fighting for my life when I developed a rare autoimmune disease that can attack the brain. This is my story.

 




The autoimmune encephalitis (AE) syndromes have been characterised by the detection of autoantibodies in serum and/or cerebrospinal fluid which target the extracellular domains of specific neuroglial antigens.

 




The causes of encephalitis are numerous, and extensive investigations for infectious agents and other etiologies are often negative. The discovery that many of these encephalitis are immune mediated has changed the approach to the diagnosis and treatment …

 




Review Plasma exchange in neurological disease Chinar Osman1, Rachel Jennings1, Khaled El-Ghariani2, Ashwin Pinto1 Neurosciences, Wessex Neurological Centre, Southampton, UK Therapeutics and Tissue Services, NHS Blood and Transplant, Sheffield Teaching Hospitals NHS Trust and the University of Sheffield, Sheffield, UK Correspondence to Dr Chinar Osman, Neurosciences, Wessex Neurological Centre, Southampton SO16 6YD, UK; chinar.osman{at}uhs.nhs.uk Abstract Plasma exchange is a highly efficient technique to remove circulating autoantibodies and other humoral factors rapidly from the vascular compartment. It was the first effective acute treatment for peripheral disorders such as Guillain-Barré syndrome and myasthenia gravis before intravenous immunoglobulin became available. The recent recognition of rapidly progressive severe antibody-mediated central nervous system disorders, such as neuromyelitis optica spectrum disorders and anti-N-methyl-D-aspartate-receptor encephalitis, has renewed interest in using plasma exchange for their acute treatment also. In this review we explain the principles and technical aspects of plasma exchange, review its current indications, and discuss the implications for its provision in the UK. View Full Text Statistics from Altmetric.com View Full Text Footnotes Contributors CO wrote the draft manuscript. RJ and KE-G contributed to the practicalities of PLEX. AAP reviewed the manuscript. Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors. Competing interests None declared. Patient consent for publication Not required. Provenance and peer review Commissioned. Externally peer reviewed by Marguerite Hill, Swansea, UK and Aisling Carr, London, UK Request Permissions If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways. Copyright information: © Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ. Read the full text or download the PDF: Subscribe Log in

 




Angie was a busy, young working-from-home wife and mother until July 2019 when Anti-NMDA Receptor Encephalitis turned her and her family’s life upside down. Here …Read More…

 







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