Anti-NMDA Receptor Encephalitis Foundation Newsletter

Immunotherapy in Autoimmune Encephalitis – Full Text View.

 




PHILADELPHIA — A small percentage of people diagnosed with a mysterious neurological condition may only experience psychiatric changes – such as delusional thinking, hallucinations, and aggressive behavior – according to a new study by researchers in the Perelman School of Medicine at the University of Pennsylvania. In addition, people who had previously been diagnosed with this disease, called anti-NMDA receptor (anti-NMDAR) encephalitis, had relapses that only involved psychiatric behavior. In an article published Online First in JAMA Neurology, researchers suggest that, while isolated psychiatric episodes are rare in anti-NMDAR encephalitis cases, abnormal test findings or subtle neurological symptoms should prompt screening for the condition, as it is treatable with immunotherapies. Related Links Penn Center for Paraneoplastic Neurologic Disorders Penn Neurology Perelman School of Medicine at the University of Pennsylvania University of Pennsylvania Health System Within a large group of 571 patients with confirmed Anti-NMDAR Encephalitis, only 23 patients (4 percent) had isolated psychiatric episodes. Of the 23, 5 patients experienced the onset of behavior changes as their only symptoms, without neurological changes, while 18 patients had psychiatric symptoms emerge at the outset of a relapse of Anti-NMDAR Encephalitis in which no neurological changes were identified. After being treated for the condition, 83 percent of these patients recovered substantially or completely. “While many patients with Anti-NMDAR Encephalitis present with isolated psychiatric symptoms, most of these patients subsequently develop, in a matter of days, additional neurological symptoms which help to make the diagnosis of the disease. In the current study, we find out that a small percentage of patients do not develop neurological symptoms, or sometimes these are very subtle and transitory. Studies using brain MRI and analysis of the cerebrospinal fluid may help to demonstrate signs of inflammation,” said Josep Dalmau, MD, PhD, adjunct professor of Neurology. “For patients who have been previously diagnosed with Anti-NMDAR Encephalitis and are in remission, any behavior change may present a relapse and should be tested quickly and treated aggressively.” Anti-NMDAR Encephalitis is one of the most common forms of autoimmune encephalitis, and symptoms can include psychiatric symptoms, memory issues, speech disorders, seizures, involuntary movements, and loss of consciousness. In an earlier Penn Medicine study, 38 percent of all patients (and 46 percent of females with the condition) were found to have a tumor, most commonly it was an ovarian tumor. When correctly diagnosed and treated early, Anti-NMDAR Encephalitis can be effectively treated. “For patients with new psychotic symptoms that are evaluated in centers where an MRI, EEG or spinal fluid test may not have been administered, there is a chance that Anti-NMDAR Encephalitis may be missed,” said lead author Matthew Kayser, MD, PhD, postdoctoral fellow and attending physician in Psychiatry at Penn. “However, the likelihood of pure or isolated new-onset psychosis to be anti-NMDAR encephalitis gradually decreases if no other symptoms emerge during the first 4 weeks of psychosis.” Anti-NMDAR Encephalitis was first characterized by Penn’s Josep Dalmau, MD, PhD, adjunct professor of Neurology, and David R. Lynch, MD, PhD, associate professor of Neurology and Pediatrics, in 2007. One year later, the same investigators, in collaboration with Rita Balice-Gordon, PhD, professor of Neuroscience, characterized the main syndrome and provided preliminary evidence that the antibodies have a pathogenic effect on the NR1 subunit of the NMDA receptor in the Lancet Neurology in December 2008. The disease can be diagnosed using a test developed at the University of Pennsylvania and currently available worldwide. With appropriate treatment, approximately 81 percent of patients significantly improve and, with a recovery process that takes an average of 2 years, can fully recover. In addition to Drs. Dalmau and Kayser, the research team includes Maarten J. Titulaer, MD, PhD, and Núria Gresa-Arribas, PhD.  The study was funded by the National Institutes of Health (R25-MH060490, R01 NS077851 and R01 MH094741), KWF fellowship 2009-4451 from the Dutch Cancer Society, PI11/01780 from the Fondo de Investigaciones Sanitarias and by Fundació la Marató TV3.

 




Fellows’ Journal Club: Zhang T, et al.Brain MRI Characteristics of Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Their Associations with 2-Year Clinical Outcome…

 




Long-term neuropsychological outcome following pediatric anti-NMDAR encephalitis…

 




Background and purpose Data concerning the characteristics and duration of the critical manifestations, treatment response, and long-term outcomes of severe anti- N-methyl- d-aspartate receptor…

 




Paul Braker needs your help today! Nurse Has Autoimmune Encephalitis –   ER nurse, age 30, stricken by rare autoimmune disease  Anti-NMDA Receptor Encephalitis, a rare and devastating disease Anti-NMDA receptor encephalitis is a disease occurring when antibodies produced by the body’s own immune…

 




After tragedy strikes, young fighter has reason to hope…

 




Anti-NMDAR encephalitis is a condition in which acute psychiatric symptoms and physical signs develop following the development of autoimmune antibodies to N-methyl D-aspartate (NMDAR) receptors. These receptors are made up of 2 subunits, the NR1 and the NR2 (which is of several types – A, B, C or…

 




SHARE May 2018; 90 (22) Listen: Audio summary of highlighted articles In Focus Spotlight on the May 29 issue Robert A. Gross Full Text Full Text (PDF) Editorials Sauna and risk of stroke Does frequent sauna bathing prevent stroke? Josef G. Heckmann, Katriina Kukkonen-Harjula Full Text Full Text (PDF) Sex differences in stroke outcomes A case for better health care for older women Nadine E. Andrew, Velandai Srikanth Full Text Full Text (PDF) Free health care, great data, and new clues on multiple sclerosis Gary M. Franklin, Gavin McDonnell Full Text Full Text (PDF) Articles Sauna bathing reduces the risk of stroke in Finnish men and women A prospective cohort study Setor K. Kunutsor, Hassan Khan, Francesco Zaccardi, Tanjaniina Laukkanen, Peter Willeit, Jari A. Laukkanen Abstract Full Text Full Text (PDF) Factors contributing to sex differences in functional outcomes and participation after stroke Hoang T. Phan, Christopher L. Blizzard, Mathew J. Reeves, Amanda G. Thrift, Dominique A. Cadilhac, Jonathan Sturm, Emma Heeley, Petr Otahal, Konstantinos Vemmos, Craig Anderson, Priya Parmar, Rita Krishnamurthi, Suzanne Barker-Collo, Valery Feigin, Yannick Bejot, Norberto Luiz Cabral, Antonio Carolei, Simona Sacco, Nicolas Chausson, Stephane Olindo, Peter Rothwell, Carolina Silva, Manuel Correia, Rui Magalhães, Peter Appelros, Janika Kõrv, Riina Vibo, Cesar Minelli, Seana L. Gall Abstract Full Text Full Text (PDF) Incidence of MS has increased markedly over six decades in Denmark particularly with late onset and in women Nils Koch-Henriksen, Lau Caspar Thygesen, Egon Stenager, Bjarne Laursen, Melinda Magyari Abstract Full Text Full Text (PDF) Encephalitis with mGluR5 antibodies Symptoms and antibody effects Marianna Spatola, Lidia Sabater, Jesús Planagumà, Eugenia Martínez-Hernandez, Thaís Armangué, Harald Prüss, Takahiro Iizuka, Ruben L. Caparó Oblitas, Jean-Christophe Antoine, Richard Li, Nicholas Heaney, Niall Tubridy, Elvira Munteis Olivas, Myrna R. Rosenfeld, Francesc Graus, Josep Dalmau Abstract Full Text Full Text (PDF) CME Course Visual stimulation leads to activation of the nociceptive trigeminal nucleus in chronic migraine Laura H. Schulte, Angie Allers, Arne May Abstract Full Text Full Text (PDF) Pattern of polyphenol intake and the long-term risk of dementia in older persons Sophie Lefèvre-Arbogast, David Gaudout, Julien Bensalem, Luc Letenneur, Jean-François Dartigues, Boris P. Hejblum, Catherine Féart, Cécile Delcourt, Cécilia Samieri Abstract Full Text Full Text (PDF) OPEN ACCESS [11C]PK11195 binding in Alzheimer disease and progressive supranuclear palsy Luca Passamonti, Patricia Vázquez Rodríguez, Young T. Hong, Kieren S.J. Allinson, W. Richard Bevan-Jones, David Williamson, P. Simon Jones, Robert Arnold, Robin J. Borchert, Ajenthan Surendranathan, Elijah Mak, Li Su, Tim D. Fryer, Franklin I. Aigbirhio, John T. O’Brien, James B. Rowe Abstract Full Text Full Text (PDF) OPEN ACCESS Long-term neuropsychological outcome following pediatric anti-NMDAR encephalitis Marienke A.A.M. de Bruijn, Femke K. Aarsen, Marielle P. van Oosterhout, Marieke M. van der Knoop, Coriene E. Catsman-Berrevoets, Marco W.J. Schreurs, Danielle E.M. Bastiaansen, Peter A.E. Sillevis Smitt, Rinze F. Neuteboom, Maarten J. Titulaer; On behalf of the CHANCE Study Group Abstract Full Text Full Text (PDF) CME Course What’s Happening Historical Neurology 150th anniversary of clinical description of multiple sclerosis Leopold Ordenstein’s legacy Helmar C. Lehmann, Alastair Compston, Hans-Peter Hartung Abstract Full Text Full Text (PDF) Views & Reviews Advantages of virtual reality in the rehabilitation of balance and gait Systematic review Desiderio Cano Porras, Petra Siemonsma, Rivka Inzelberg, Gabriel Zeilig, Meir Plotnik Abstract Full Text Full Text (PDF) Clinical/Scientific Notes COL4A1/2 CNVs and cerebral small vessel disease Narrowing in on the critical chromosomal region Avi Saskin, Guillaume Sillon, Natalie Palfreeman, Daniela Buhas Full Text Full Text (PDF) Reflections: Neurology and the Humanities O, say does that star spangled banner yet wave Jack E. Riggs Full Text Full Text (PDF) Resident & Fellow Section Resident & Fellow Rounds John J. Millichap, Roy E. Strowd Full Text Full Text (PDF) Mystery Case: Bilateral temporal crescent sparing after cardiac arrest Daniel Josef Lindegger, Maria Helfenstein, Oliver Job, Misha Pless Full Text Full Text (PDF) Clinical Reasoning: A 23-year-old woman with fever and vertical diplopia David J. Lin, Seth N. Levin, Catherine S.W. Albin, Anna E. Goodheart, Nagagopal Venna Full Text Full Text (PDF) Teaching NeuroImages: Distinct brain microhemorrhage pattern in critical illness associated with respiratory failure Julianne P. Hall, Priyanka Minhas, Marinos Kontzialis, Miral D. Jhaveri Full Text Full Text (PDF) Teaching NeuroImages: Prosopagnosia heralding anti-NMDA receptor encephalitis Grayson Beecher, Amanda Nicole Wagner, Jonathan Abele, Penelope Smyth Full Text Full Text (PDF) Patient Page Regular sauna use shown to decrease risk of first-time strokeAbout stroke Scott Rewinkel Full Text Full Text (PDF) Disputes & Debates: Editors’ Choice Editors’ note: Pilocytic astrocytoma with anaplasia arising from the optic chiasm in a very elderly patient Megan Alcauskas, Steven Galetta Full Text Full Text (PDF) Reader response: Pilocytic astrocytoma with anaplasia arising from the optic chiasm in a very elderly patient Andrea Wasilewski Full Text Full Text (PDF) Author response: Pilocytic astrocytoma with anaplasia arising from the optic chiasm in a very elderly patient Shintaro Hayashi Full Text Full Text (PDF) Editors’ note: ELAPSS score for prediction of risk of growth of unruptured intracranial aneurysms Megan Alcauskas, Steven Galetta Full Text Full Text (PDF) Reader response: ELAPSS score for prediction of risk of growth of unruptured intracranial aneurysms Joshua Z. Willey Full Text Full Text (PDF) Correction Previous Previous Issue Subscribe to e-Alerts RSS Search for this keyword POPULAR ON Neurology Most Read Most Cited

 




Seemingly overnight, an illness known as “autoimmune encephalitis” took my daughter’s sweet disposition, memory, mobility, and speech.