Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology Future Forecasting Series
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology Future Forecasting Series
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Neurology Video Journal Club
  • Residents & Fellows

User menu

  • Subscribe
  • My Alerts
  • Log in

Search

  • Advanced search
Neurology
Home
The most widely read and highly cited peer-reviewed neurology journal
  • Subscribe
  • My Alerts
  • Log in
Site Logo
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Neurology Video Journal Club
  • Residents & Fellows

Share

March 14, 2017; 88 (11) Article

Investigations in GABAA receptor antibody-associated encephalitis

Marianna Spatola, Mar Petit-Pedrol, Mateus Mistieri Simabukuro, Thaís Armangue, Fernanda J. Castro, Maria I. Barcelo Artigues, Maria R. Julià Benique, Leslie Benson, Mark Gorman, Ana Felipe, Ruben L. Caparó Oblitas, Myrna R. Rosenfeld, Francesc Graus, Josep Dalmau
First published February 15, 2017, DOI: https://doi.org/10.1212/WNL.0000000000003713
Marianna Spatola
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Mar Petit-Pedrol
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Mateus Mistieri Simabukuro
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Thaís Armangue
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Fernanda J. Castro
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Maria I. Barcelo Artigues
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Maria R. Julià Benique
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Leslie Benson
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Mark Gorman
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ana Felipe
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ruben L. Caparó Oblitas
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Myrna R. Rosenfeld
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Francesc Graus
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Josep Dalmau
From the Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.S., M.P.-P., T.A., M.R.R., F.G., J.D.), University of Barcelona, Spain; University of Lausanne (UNIL) (M.S.), Switzerland; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (M.P.-P., T.A., M.R.R., J.D., F.G.), Madrid, Spain; Neurology Service (T.A., J.D.), Hospital Sant Joan de Déu, Barcelona, Spain; Neurology Division, Hospital das Clínicas (M.M.S.), São Paulo University, Brazil; Hospital de Base (F.J.C.), Brasília, Brazil; Service of Neurology (M.I.B.A.) and Service of Immunology (M.R.J.B.), University Hospital of Son Espases, Mallorca, Spain; Department of Neurology (L.B., M.G.), Children's Hospital, Boston, MA; Pediatric Neurology Section (A.F.), Vall d'Hebron University Hospital, Barcelona, Spain; Hospital Nacional Edgardo Rebagliati Martins (R.L.C.O.), Lima, Peru; Department of Neurology (M.R.R., J.D.), University of Pennsylvania, Philadelphia; Service of Neurology (F.G., J.D.), Hospital Clínic; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Full PDF
Citation
Investigations in GABAA receptor antibody-associated encephalitis
Marianna Spatola, Mar Petit-Pedrol, Mateus Mistieri Simabukuro, Thaís Armangue, Fernanda J. Castro, Maria I. Barcelo Artigues, Maria R. Julià Benique, Leslie Benson, Mark Gorman, Ana Felipe, Ruben L. Caparó Oblitas, Myrna R. Rosenfeld, Francesc Graus, Josep Dalmau
Neurology Mar 2017, 88 (11) 1012-1020; DOI: 10.1212/WNL.0000000000003713

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
843

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Abstract

Objective: To report the clinical features, comorbidities, receptor subunit targets, and outcome in patients with anti-GABAA receptor (GABAAR) encephalitis.

Methods: Clinical study of 26 patients, including 17 new (April 2013–January 2016) and 9 previously reported patients. Antibodies to α1, β3, and γ2 subunits of the GABAAR were determined using reported techniques.

Results: Patients' median age was 40.5 years (interquartile range 48.5 [13.75–62.35] years; the youngest 2.5 months old; 13 female). Symptoms included seizures (88%), alteration of cognition (67%), behavior (46%), consciousness (42%), or abnormal movements (35%). Comorbidities were identified in 11 (42%) patients, including 7 tumors (mostly thymomas), 2 herpesvirus encephalitis (herpes simplex virus 1, human herpesvirus 6; coexisting with NMDAR antibodies), and 2 myasthenia without thymoma. Brain MRI was abnormal in 23 (88%) patients, showing in 20 (77%) multifocal, asynchronous, cortical-subcortical T2/fluid-attenuated inversion recovery abnormalities predominantly involving temporal (95%) and frontal (65%) lobes, but also basal ganglia and other regions. Immunologic or tumor therapy resulted in substantial improvement in 18/21 (86%) assessable patients; the other 3 (14%) died (2 status epilepticus, 1 sepsis). Compared with adults, children were more likely to have generalized seizures (p = 0.007) and movement disorders (p = 0.01) and less likely to have a tumor (p = 0.01). The main epitope targets were in the α1/β3 subunits of the GABAAR.

Conclusions: Anti-GABAAR encephalitis is characterized by frequent seizures and distinctive multifocal cortical-subcortical MRI abnormalities that provide an important clue to the diagnosis. The frequency of symptoms and comorbidities differ between children (more viral-related) and adults (more tumor-related). The disorder is severe but most patients respond to treatment.

GLOSSARY

CBA=
cell-based assay;
FLAIR=
fluid-attenuated inversion recovery;
GABAAR=
GABAA receptor;
HHV6=
human herpesvirus 6;
HSV1=
herpes simplex 1;
PSCT=
peripheral stem cell transplantation

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Editorial, page 1010

  • Supplemental data at Neurology.org

  • Received June 24, 2016.
  • Accepted in final form October 18, 2016.
  • © 2017 American Academy of Neurology
View Full Text

AAN Members

We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.

Google Safari Microsoft Edge Firefox

Click here to login

AAN Non-Member Subscribers

Click here to login

Purchase access

For assistance, please contact:
AAN Members (800) 879-1960 or (612) 928-6000 (International)
Non-AAN Member subscribers (800) 638-3030 or (301) 223-2300 option 3, select 1 (international)

Sign Up
Information on how to subscribe to Neurology and Neurology: Clinical Practice can be found here 

Purchase
Individual access to articles is available through the Add to Cart option on the article page.  Access for 1 day (from the computer you are currently using) is US$ 39.00.  Pay-per-view content is for the use of the payee only, and content may not be further distributed by print or electronic means.  The payee may view, download, and/or print the article for his/her personal, scholarly, research, and educational use.  Distributing copies (electronic or otherwise) of the article is not allowed.

Letters: Rapid online correspondence

No comments have been published for this article.
Comment

REQUIREMENTS

If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org

Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.

If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.

Submission specifications:

  • Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
  • Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
  • Submit only on articles published within 6 months of issue date.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Publishing Agreement Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
    • Abstract
    • GLOSSARY
    • METHODS
    • RESULTS
    • DISCUSSION
    • AUTHOR CONTRIBUTIONS
    • STUDY FUNDING
    • DISCLOSURE
    • ACKNOWLEDGMENT
    • Footnotes
    • REFERENCES
  • Figures & Data
  • Info & Disclosures
Advertisement

Hemiplegic Migraine Associated With PRRT2 Variations A Clinical and Genetic Study

Dr. Robert Shapiro and Dr. Amynah Pradhan

► Watch

Related Articles

  • G2A1B3AA receptor antibodies and their clinical associations

Topics Discussed

  • All Epilepsy/Seizures
  • Status epilepticus

Alert Me

  • Alert me when eletters are published
Neurology: 100 (5)

Articles

  • Ahead of Print
  • Current Issue
  • Past Issues
  • Popular Articles
  • Translations

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Activate a Subscription
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Education
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology | Print ISSN:0028-3878
Online ISSN:1526-632X

© 2023 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise