The Movement disorder associated with NMDAR antibody-encephalitis is complex and characteristic: an expert video-rating study

James A Varley¹, Alastair J S Webb¹, Bettina Balint^{1

2}, Victor S C Fung³, Kapil D Sethi⁴, Marina A J Tijssen⁵, Timothy Lynch⁶, Shakeeb S Mohammad⁷, Fiona Britton¹, Matthew Evans¹, Yael Hacohen⁸, Jean-Pierre Lin⁸, Nardo Nardocci⁹, Tiziana Granata⁹, Russell C Dale⁷, Ming J Lim⁸, Kailash P Bhatia¹⁰, Anthony E Lang¹¹, Sarosh R Irani¹²

Affiliations

¹ Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
² Department of Neurology, University Hospital, Heidelberg, Germany.
³ The Movement Disorders Unit, Westmead Hospital, Sydney, New South Wales, Australia.
⁴ Department of Neurology, Augusta University, Augusta, Georgia.
⁵ Department of Neurology, University Medical Center Groningen, Groningen, The Netherlands.
⁶ Centre for Brain Health, The Dublin Neurological Institute at the Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland.
⁷ Discipline of Child & Adolescent Health, Westmead Children's Hospital, The University of Sydney, Sydney, New South Wales, Australia.
⁸ Children's Neurosciences, Evelina Children's Hospital, King's Health Partners Academic Health Science Centre, London, UK.
⁹ Department of Paediatric Neuroscience, IRCCS Foundation C. Besta Neurological Institute, Milan, Italy.
¹⁰ Sobell Department, Institute of Neurology, UCL NHNN, London, UK.
¹¹ Morton and Gloria Shulman Movement Disorders Clinic, The Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, Toronto, Ontario, Canada.
¹² Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK sarosh.irani@ndcn.ox.ac.uk.

PMID: 30032119
PMCID: PMC6581096
DOI: 10.1136/jnnp-2018-318584

The Movement disorder associated with NMDAR antibody-encephalitis is complex and characteristic: an expert video-rating study

James A Varley et al. J Neurol Neurosurg Psychiatry. 2019 Jun.

. 2019 Jun;90(6):724-726.

doi: 10.1136/jnnp-2018-318584. Epub 2018 Jul 21.

Authors

Affiliations

¹ Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
² Department of Neurology, University Hospital, Heidelberg, Germany.
³ The Movement Disorders Unit, Westmead Hospital, Sydney, New South Wales, Australia.
⁴ Department of Neurology, Augusta University, Augusta, Georgia.
⁵ Department of Neurology, University Medical Center Groningen, Groningen, The Netherlands.
⁶ Centre for Brain Health, The Dublin Neurological Institute at the Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland.
⁷ Discipline of Child & Adolescent Health, Westmead Children's Hospital, The University of Sydney, Sydney, New South Wales, Australia.
⁸ Children's Neurosciences, Evelina Children's Hospital, King's Health Partners Academic Health Science Centre, London, UK.
⁹ Department of Paediatric Neuroscience, IRCCS Foundation C. Besta Neurological Institute, Milan, Italy.
¹⁰ Sobell Department, Institute of Neurology, UCL NHNN, London, UK.
¹¹ Morton and Gloria Shulman Movement Disorders Clinic, The Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, Toronto, Ontario, Canada.
¹² Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK sarosh.irani@ndcn.ox.ac.uk.

PMID: 30032119
PMCID: PMC6581096
DOI: 10.1136/jnnp-2018-318584

No abstract available

Keywords: NMDA receptor; autoantibody; autoimmune; encephalitis; movement disorders.

PubMed Disclaimer

Conflict of interest statement

Competing interests: SRI is a co-applicant and receives royalties on patent application WO/2010/046716 entitled ‘Neurological Autoimmune Disorders’. The patent has been licensed to Euroimmun AG for the development of assays for LGI1 and other VGKC-complex antibodies.

Figures

**Figure 1**
The clinical features and movement disorder evaluations in 34 patients with N-methyl-D-aspartate-antibody encephalitis (NMDAR-AbE). (A) Clinical and investigation findings across the 34 patients whose videos were rated. By definition, all patients had a movement disorder (MD) and other clinical and paraclinical features included psychiatric (n=33/34), cognitive (n=32/34), seizures (n=30/34), autonomic (n=17/34), abnormal electroencephalogram (n=28/30), abnormal cerebrospinal fluid (CSF) (n=19/33) and abnormal MRI (n=9/34). Abnormal CSF findings included any of pleocytosis, oligoclonal bands or raised protein. Two ovarian teratomas were noted in postpubescent women (25 and 32 years). Overall, three cases were adults. (B) Symptom onset and offset in NMDAR-AbE: timings of onset (black) and offset (grey) of the main five symptom categories after first symptom (day 1; median, minimum and maximum values displayed on box and whisker plot), (C) with a particular focus on the timing of the MD in individual patients. Full datasets were available from coauthors and denominators <34 represent variably reported details from the literature-derived videos. Expert classification of phenomenology for 76 videos from patients with NMDAR-AbE (D–F). (D) Dystonia, chorea and stereotypies were the most commonly used terms. For the ‘other’ category, raters used terms including: mutism, stupor, myorhythmia, myokymia, tics, opisthotonus, cerebellar syndrome/ataxia, orofacial dyskinesia, waxy flexibility, oculogyric crises, athetosis, agitation, seizure, startle and vocal perseveration. (E) The interactions between phenomenologies in NMDAR-AbE with co-occurrence of stereotypies, chorea and dystonia shown in a Circos plot,^e20 based on a co-occurrence matrix within single video ratings. (F) Stereotypies, chorea and dystonia were equally represented in the face, arm and leg, respectively.

See this image and copyright information in PMC

References

1. Florance NR, Davis RL, Lam C, et al. . Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol 2009;66:11–18. 10.1002/ana.21756 - DOI - PMC - PubMed
1. Irani SR, Bera K, Waters P, et al. . N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexes. Brain 2010;133(Pt 6):1655–67. 10.1093/brain/awq113 - DOI - PMC - PubMed
1. Mohammad SS, Fung VS, Grattan-Smith P, et al. . Movement disorders in children with anti-NMDAR encephalitis and other autoimmune encephalopathies. Mov Disord 2014;29:1539–42. 10.1002/mds.25999 - DOI - PubMed
1. Kleinig TJ, Thompson PD, Matar W, et al. . The distinctive movement disorder of ovarian teratoma-associated encephalitis. Mov Disord 2008;23:1256–61. 10.1002/mds.22073 - DOI - PubMed
1. Baizabal-Carvallo JF, Stocco A, Muscal E, et al. . The spectrum of movement disorders in children with anti-NMDA receptor encephalitis. Mov Disord 2013;28:543–7. 10.1002/mds.25354 - DOI - PubMed

Publication types

Actions
Actions

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions

Grants and funding

104079/Z/14/Z/WT_/Wellcome Trust/United Kingdom

LinkOut - more resources

Full Text Sources
- Ovid Technologies, Inc.
Other Literature Sources
- scite Smart Citations

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

The Movement disorder associated with NMDAR antibody-encephalitis is complex and characteristic: an expert video-rating study

Affiliations

The Movement disorder associated with NMDAR antibody-encephalitis is complex and characteristic: an expert video-rating study

Authors

Affiliations

Conflict of interest statement

Figures

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources