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Letter
The Movement disorder associated with NMDAR antibody-encephalitis is complex and characteristic: an expert video-rating study
  1. James A Varley1,
  2. Alastair J S Webb1,
  3. Bettina Balint1,2,
  4. Victor S C Fung3,
  5. Kapil D Sethi4,
  6. Marina A J Tijssen5,
  7. Timothy Lynch6,
  8. Shakeeb S Mohammad7,
  9. Fiona Britton1,
  10. Matthew Evans1,
  11. Yael Hacohen8,
  12. Jean-Pierre Lin8,
  13. Nardo Nardocci9,
  14. Tiziana Granata9,
  15. Russell C Dale7,
  16. Ming J Lim8,
  17. Kailash P Bhatia10,
  18. Anthony E Lang11,
  19. Sarosh R Irani1
  1. 1 Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
  2. 2 Department of Neurology, University Hospital, Heidelberg, Germany
  3. 3 The Movement Disorders Unit, Westmead Hospital, Sydney, New South Wales, Australia
  4. 4 Department of Neurology, Augusta University, Augusta, Georgia
  5. 5 Department of Neurology, University Medical Center Groningen, Groningen, The Netherlands
  6. 6 Centre for Brain Health, The Dublin Neurological Institute at the Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland
  7. 7 Discipline of Child & Adolescent Health, Westmead Children’s Hospital, The University of Sydney, Sydney, New South Wales, Australia
  8. 8 Children's Neurosciences, Evelina Children's Hospital, King's Health Partners Academic Health Science Centre, London, UK
  9. 9 Department of Paediatric Neuroscience, IRCCS Foundation C. Besta Neurological Institute, Milan, Italy
  10. 10 Sobell Department, Institute of Neurology, UCL NHNN, London, UK
  11. 11 Morton and Gloria Shulman Movement Disorders Clinic, The Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, Toronto, Ontario, Canada
  1. Correspondence to Dr Sarosh R Irani, John Radcliffe Hospital, Oxford OX3 9DU, UK; sarosh.irani{at}ndcn.ox.ac.uk

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Introduction

N-methyl-D-aspartate receptor antibody-mediated encephalitis (NMDAR-AbE) is an increasingly recognised and treatable encephalitis, with a predilection for children and young adults.1 2 As earlier immunotherapy improves outcomes, timely and accurate recognition of NMDAR-AbE is a major clinical aim.2

The characteristic polysymptomatic presentation of NMDAR-AbE includes early neuropsychiatric deficits with seizures, autonomic disturbance, reduced consciousness and a movement disorder (MD).1 2 This MD, seen in around 90% of cases, can be the presenting feature, particularly in children,1 and is typically hyperkinetic with limb plus orofacial involvement.1–5 To date, elegant detailed descriptions exist in a few patients.4 However, small series have used highly variable phenomenological descriptions.1–5 Therefore, the phenomenology of the associated MD lacks consensus. Its clearer description will facilitate confident recognition and enable earlier immunotherapy administration in NMDAR-AbE.

Expert-rater descriptions remain the gold standard to define phenotypes in movement disorders. In this study, ratings from seven experts across 76 videos were used to better define the MD in NMDAR-AbE.

Methods

Subjects

Autoimmune neurology researchers contributed 44 videos from 20 patients who met diagnostic criteria for NMDAR-AbE.e1 A PubMed search for ‘anti-NMDAR encephalitis’ and ‘NMDAR-antibody encephalitis’, plus associated references, revealed 32 videos from 14 subjects, in eight papers from 14 subjects.3 , e2–8 Clinical features, temporal progression, outcomes and investigation findings were collated from case note reviews by the researchers and from data in published papers. Twenty videos from 18 age-matched and sex-matched (age range 2–41 years, median 12, 50% female) disease controls were selected from the literature (online supplementary data).3 e9–19

Supplemental material

[jnnp-2018-318584supp004.pdf]

Movement disorder classification

Seven experts (KB, VF, AEL, TL, NN, KS and MT) established a consensus glossary of terms (online supplementary data, modified from Mohammed et al 3). Subsequently, each expert blindly and independently rated 76 videos from 34 patients with NMDAR-AbE (median two videos …

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