Over the past 10 years, the continual discovery of novel forms of encephalitis associated with antibodies to cell‐surface or synaptic proteins has changed the paradigms for diagnosing and treating disorders that were previously unknown or mischaracterized. We review here the process of discovery, the symptoms, and the target antigens of 11 autoimmune encephalitic disorders, grouped by syndromes and approached from a clinical perspective. Anti‐N‐methyl‐d‐aspartate receptor (NMDAR) encephalitis, several subtypes of limbic encephalitis, stiff‐person spectrum disorders, and other autoimmune encephalitides that result in psychosis, seizures, or abnormal movements are described in detail. We include a novel encephalopathy with prominent sleep dysfunction that provides an intriguing link between chronic neurodegeneration and cell‐surface autoimmunity (IgLON5). Some of the caveats of limited serum testing are outlined. In addition, we review the underlying cellular and synaptic mechanisms that for some disorders confirm the antibody pathogenicity. The multidisciplinary impact of autoimmune encephalitis has been expanded recently by the discovery that herpes simplex encephalitis is a robust trigger of synaptic autoimmunity, and that some patients may develop overlapping syndromes, including anti‐NMDAR encephalitis and neuromyelitis optica or other demyelinating diseases.
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Publication date: Available online 9 October 2020Source: Neurología (English Edition)Author(s): G. Alvarez Bravo, L. RamióTorrentà
Authors: Jang Y, Kim DW, Yang KI, Byun JI, Seo JG, No YJ, Kang KW, Kim D, Kim KT, Cho YW, Lee ST, Drug Committee of Korean Epilepsy Society Abstract Autoimmune epilepsy is a newly emerging area of epilepsy. The concept of "autoimmune" as an etiology has recently been revisited thanks to advances in autoimmune encephalitis and precision medicine with immunotherapies. Autoimmune epilepsy presents with specific clinical manifestations, and various diagnostic approaches including cerebrospinal fluid analysis, neuroimaging, and autoantibody tests are essential for its differential diagnosis. The diagnosis is o...
Publication date: Available online 10 October 2020Source: IJC Heart &VasculatureAuthor(s): Dominik Linz, Jeroen Hendriks
CONCLUSIONS: As the present study was the first investigation on the coagulation status in patients with AA, elevated D-dimer levels in alopecia areata may suggest a deficient coagulation in these patients that may contribute to an increase in the risk of thrombosis. Further studies are needed to evaluate this hypothesis using a larger sample size. PMID: 33034439 [PubMed - as supplied by publisher]
Authors: Spiegel DR, O'Connell K, Stocker G, Slater J, Spiegel A PMID: 33031649 [PubMed - in process]
Background: Obesity is strongly associated with both Blount disease and obstructive sleep apnea (OSA). Obesity increases risks for anesthetic and postoperative complications, and OSA can further exacerbate these risks. Since children with Blount disease might have both conditions, we sought to determine the perioperative complications and the prevalence of OSA among these children. Methods: Patients younger than 18 years undergoing corrective surgery for Blount disease were identified from 2 sources as follows: a retrospective review of records at a single institution and querying of the Kids’ Inpatient Database...
Publication date: Available online 8 October 2020Source: IDCasesAuthor(s): Nathan VanderVeen, Nikki Nguyen, Kenny Hoang, Jason Parviz, Tahuriah Khan, Andrew Zhen, Brett W. Jagger
AbstractRheumatoid arthritis (RA) is a progressive autoimmune inflammatory disease affecting 1% of the population with three times as many women as men. As many as 86% of patients suffering from RA have cervical spine involvement. Synovial inflammation in the cervical spine causes instability and injuries including atlantoaxial subluxation, retroodontoid pannus formation, cranial settling, and subaxial subluxation. While many patients with cervical spine involvement are asymptomatic, symptomatic patients often present with nonspecific symptoms resulting from inflammation and additional secondary symptoms that are due to co...
This article reviews the recent advances of SFKs in B lymphocytes in autoimmune diseases.