PT  - JOURNAL ARTICLE
AU  - Amanda L. Piquet
AU  - Murtaza Khan
AU  - Judith E.A. Warner
AU  - Matthew P. Wicklund
AU  - Jeffrey L. Bennett
AU  - Maureen A. Leehey
AU  - Lauren Seeberger
AU  - Teri L. Schreiner
AU  - M. Mateo Paz Soldan
AU  - Stacey L. Clardy
TI  - Novel clinical features of glycine receptor antibody syndrome
AID  - 10.1212/NXI.0000000000000592
DP  - 2019 Sep 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e592
VI  - 6
IP  - 5
4099  - http://nn.neurology.org/content/6/5/e592.short
4100  - http://nn.neurology.org/content/6/5/e592.full
SO  - Neurol Neuroimmunol Neuroinflamm2019 Sep 01; 6
AB  - Objective To describe novel clinical features of GlyRα1-IgG–positive patients.Methods Patients with a positive serum GlyRα1-IgG were identified during a 2-year period from July 2016 to December 2018 at 2 academic centers and followed prospectively. All patients in this series were evaluated in the Neuroimmunology and Autoimmune Neurology clinics at the University of Utah or the University of Colorado.Results Thirteen of 17 patients had phenotypes more typically associated with glutamic acid decarboxylase (GAD65) antibody syndromes, consisting of stiff-person syndrome (SPS) with parkinsonism or cerebellar signs. One patient with parkinsonism had a presentation similar to rapidly progressive multiple system atrophy with severe dysautonomia. Ten of 17 patients had various visual symptoms including visual snow, spider web–like images forming shapes and 3-dimensional images, palinopsia, photophobia, visual hallucinations, synesthesia, and intermittent diplopia. Three of 17 patients presented with primarily autoimmune epilepsy accompanied by psychiatric symptoms.Conclusions Clinicians should consider testing for GlyR antibodies in GAD65 antibody–negative or low-positive GAD65 antibody patients with SPS-like presentations, especially in the setting of atypical features such as visual disturbances, parkinsonism, or epilepsy.DaT=dopamine transporter; ERG=electroretinogram; GAD=glutamic acid decarboxylase; MSA=multiple system atrophy; PD=Parkinson disease; PERM=progressive encephalomyelitis with rigidity and myoclonus; SPS=stiff-person syndrome; SPSD=stiff-person spectrum disorder