Session Time: 1:45pm-3:15pm
Location: Les Muses Terrace, Level 3
Objective: To describe a case of marked asymmetric coarse tremor and dystonia in patient diagnosed with anti-NMDAR encephalitis.
Background: Anti-NMDAR encephalitis has complex and heterogeneous clinical features including psychiatric features and different subtypes of movement disorders (MD). Asymmetric movement disorders in these patients is not a common finding, more frequently described in pediatric patients.
Method: We report a case of a young female with anti-NMDA receptor encephalitis presenting with rapidly progressive cognitive decline and markedly assymetric movement disorders.
Results: A previously healthy 18-year-old female was admitted to the emergency department with a three-week history of abnormal movements, cognitive impairment, and bladder dysfunction. On admission, she showed marked apathy and poor speech, scoring 4 points in MMSE and no word recall in semantic verbal fluency. She remained alert at all times and no abnormal eye movements were seen. Motor examination showed global bradykinesia and a sustained dystonic posture in her right leg, with a right arm high amplitude coarse tremor. Brain MRI showed global atrophy and CSF analysis was unremarkable. Laboratory testing ruled out metabolic, rheumatologic or infectious abnormalities. EEG showed markedly diffuse attenuation with bursts of suppression on electrical activity. A 5-day-course of methylprednisolone and 5 plasmapheresis sessions were administered empirically for a possible autoimmune encephalitis. The patient showed some improvement after plasmapheresis was started. CSF analysis for detection of antibodies against NMDAR turned out to be positive. Her abdominal MRI and pelvic exam ruled out teratomas. She was discharged from the hospital showing discrete improvement, but a 1-month follow-up ambulatorial evaluation showed complete remission of dystonic posturing and tremor and moderate improvement on cognitive tests. Analysis of other autoimmune encephalitis antibodies are still ongoing.
Conclusion: Anti-NMDAR encephalitis may be a cause of severe cognitive disfunction even in the absence of level of consciousness impairment. Asymmetric movement disorders may be related to NMDAR encephalitis in young adults with a good response to plasmapheresis. Acknowledgments – To Livia Dutra Antonio, Orlando Graziani Povoas Barsottini and Romana Hoftberger for technical support on laboratorial results regarding autoimmune encephalitis panel.
References:  Graus, F., Titulaer, M. J., Ramani Balu, R., Benseler, S. [et al]. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol, 2016; 15: 391–404.  Dalmau, J., Gleichman, A. J., Hughes, E. G., Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol, 2008; 7(12): 1091–1098  Benjumea-Cuartas V, Eisermann M, Simonnet H, [et al]. Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis? Epilepsy Behav Case Rep. 2017 Jan 18;7:42-44
To cite this abstract in AMA style:F. Rolim, L. Mendes, P. Braga Neto, R. Sampaio, G. Ferreira, P. Nóbrega, V. Mesquita, F. Maia. Rapidly progressive cognitive decline and hemi-dystonia due to anti-neuronal surface antibodies [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/rapidly-progressive-cognitive-decline-and-hemi-dystonia-due-to-anti-neuronal-surface-antibodies/. Accessed November 29, 2023.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/rapidly-progressive-cognitive-decline-and-hemi-dystonia-due-to-anti-neuronal-surface-antibodies/