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. 2019 Jun;90(6):724-726.
doi: 10.1136/jnnp-2018-318584. Epub 2018 Jul 21.

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

James A Varley  1 , Alastair J S Webb  1 , Bettina Balint  1   2 , Victor S C Fung  3 , Kapil D Sethi  4 , Marina A J Tijssen  5 , Timothy Lynch  6 , Shakeeb S Mohammad  7 , Fiona Britton  1 , Matthew Evans  1 , Yael Hacohen  8 , Jean-Pierre Lin  8 , Nardo Nardocci  9 , Tiziana Granata  9 , Russell C Dale  7 , Ming J Lim  8 , Kailash P Bhatia  10 , Anthony E Lang  11 , Sarosh R Irani  12
Affiliations

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.
No abstract available

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

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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
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

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