Glycine receptor modulating antibody predicting treatable stiff-person spectrum disorders

Neurology. Neuroimmunology and Neuroinflammation
Shannon R HinsonAndrew McKeon

Abstract

Glycine receptor alpha-1 subunit (GlyRα1)-immunoglobulin G (IgG) is diagnostic of stiff-person syndrome (SPS) spectrum but has been reported detectable in other neurologic diseases for which significance is less certain. 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. 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. Among 21 seropositive patients, 20 had confirmed SPS spectrum clinically, but 1 was later determined to have a functional neurologic disorder. Sera from 9 patients with SPS spectrum , but not 7 controls, nor the functional patient, caused GlyRα1 modulation (100% specificity). SPS spectrum phenotypes included progressive encephalomyelitis with rigidity and myoclonus (PERM) (8), classic SPS (5), stiff limb (5), stiff trunk (1), and isolated exaggerated startle (hyperekplexia, 1). Neuropsychiatric symptoms present in 12 patients (60%) were anxiety (11), depression (6), and delirium (3). Anxiety was particularly severe in 3...Continue Reading

References

Jan 1, 1987·Annals of the New York Academy of Sciences·F M HowardL R Elveback
Aug 30, 2008·Neuropharmacology·Joseph W Lynch
Apr 30, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Ethan G HughesRita J Balice-Gordon
Nov 25, 2011·Parkinsonism & Related Disorders·Kathrin CzarneckiJ Eric Ahlskog
Feb 15, 2012·Archives of Neurology·Andrew McKeonSean J Pittock
Oct 24, 2012·JAMA Neurology·Andrew McKeonJosep Dalmau
May 23, 2013·BMJ Case Reports·Angelika DerksenRüdiger J Seitz
Aug 13, 2013·Case Reports in Neurological Medicine·Sofie N De BlauweLudo J Vanopdenbosch
Oct 9, 2013·JAMA Neurology·Stacey L ClardyAndrew McKeon
Jun 22, 2014·Brain : a Journal of Neurology·Alexander Carvajal-GonzálezAngela Vincent
Jan 18, 2015·American Journal of Clinical Pathology·Bucky K LozierHarry R Hill
Apr 12, 2016·JAMA Neurology·Eugenia Martinez-HernandezJosep Dalmau
May 3, 2016·JAMA Neurology·Anastasia ZekeridouVanda A Lennon

❮ Previous
Next ❯

Citations

Jul 30, 2019·Neurology. Neuroimmunology and Neuroinflammation·Amanda L PiquetStacey L Clardy
Jun 17, 2020·Brain : a Journal of Neurology·Anastasia Zekeridou, Sean J Pittock
Oct 27, 2018·Movement Disorders Clinical Practice·Bettina Balint, Hans-Michael Meinck
Aug 14, 2019·Frontiers in Neurology·Tetyana Blinder, Jan Lewerenz
Aug 28, 2019·Neurology. Neuroimmunology and Neuroinflammation·Amanda L PiquetStacey L Clardy
Sep 7, 2018·Frontiers in Molecular Neuroscience·Natascha SchaeferCarmen Villmann
Jun 27, 2020·Brain Sciences·Dominique EndresLudger Tebartz van Elst
Aug 21, 2018·Journal of Clinical Movement Disorders·James A G CrispoAllison W Willis
Nov 23, 2020·Clinical Chemistry·Adrian BudhramJohn R Mills
Aug 5, 2019·Journal of the Neurological Sciences·Rima El-AbassiJohn D England
Feb 2, 2021·Continuum : Lifelong Learning in Neurology·Sebastian Lopez Chiriboga, Eoin P Flanagan
Apr 16, 2021·Therapeutic Advances in Neurological Disorders·Popianna TsiortouMarinos C Dalakas
May 20, 2021·Neurology. Neuroimmunology and Neuroinflammation·Francesc GrausJerome Honnorat
Jul 30, 2021·Clinical Neurology and Neurosurgery·Anna ChangWei-Hung Chen
Jul 19, 2021·Schizophrenia Research·Anne KershenbaumJesus Perez

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autism

Autism spectrum disorder is associated with challenges with social skills, repetitive behaviors, and often accompanied by sensory sensitivities and medical issues. Here is the latest research on autism.

CSF & Lymphatic System

This feed focuses on Cerebral Spinal Fluid (CSF) and the lymphatic system. Discover the latest papers using imaging techniques to track CSF outflow into the lymphatic system in animal models.