Objective: To report anti NMDAR encephalitis patient, with evidence of AT, and to explain her clinical presentation, management and outcome.

Background: Autoimmune encephalitis(AE) is nowadays under the focus of extensive studies, due to the emergence of discrete clinical and laboratory specifiers(1). Some reports correlate combined AE and AT with severe presentation and poor recovery(2,3,4), as compared to pure anti NMDAR syndrome (5). With our experience with that patient, we report the contrary.

Method: Our 17-year-old female patient, had presented to the ER, with two successive attacks of generalized tonic fits. Preictally, her family reported few days of irritability, inattention, bizarre behaviors, insomnia, and easy to cry, which was attributed then to her high school exams pressure.
Evaluation after recovery revealed persistent behavioral changes; which in addition to mononuclear pleocytosis in the CSF, suggested the diagnosis of viral encephalitis.
However, the emergence of abnormal movements in the form of orolingual dyskinetic movements, hand and mouth dystonia and generalized rigidity, beside bouts of autonomic instability (resting tachycardia and palpitation), all pointed toward autoimmune etiology rather than infective. This possibility had been confirmed upon getting the CSF auto antibodies report, that showed positive anti NMDAR antibodies.
Based on that, a 5-day methylprednisolone infusion course had been given, followed by a 5-day-course of IVIG, in parallel with oral steroid therapy. Full panel of investigations had been requested, including thyroid tests (low TSH, high T4, positive TPO antibodies and enlarged both thyroid lobes on ultrasound), and screening for malignancy (pan CT studies, PET scan and antibodies, which were all negative).

Results: Extended follow-up over the next 4 weeks revealed good clinical recovery; she became seizure free, almost reached her baseline personality back. She was discharged home on oral steroid therapy and anticonvulsant agents. Follow up visits at the outpatient clinic and more immune testing are mandatory to ensure remission and detect relapse early.

Conclusion: Despite having concomitant AE and AT, our patient responded well on the management protocol for anti NMDAR encephalitis. Either a pure entity or overlapped with other autoimmune disorders, AE demands further clinical studies to accurately specify its boundaries.

References: 1.Qianhui Xu, Yong Zhu, Qian Wang, Jing Han, Fengju Mao, Silin Zeng, Siyan Chen, Chenyong Zhao, Mei Gu, Zaiwang Li, Xiaoguang Luo, Xuejun Fu, Ying Huang. Clinical features, treatments, and outcomes of patients with anti-N-methyl-d-aspartate encephalitis—a single-center, retrospective analysis in China. Front. Biosci. (Landmark Ed) 2021, 26(9), 496–503.
2.Ma X, Yin Q, Zeng Z, Wang C, Yang Y, Guo S. Thyroid Function and Autoimmune Indications in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis. Neuroimmunomodulation. 2018;25(2):110-117. .
3.Chen Z, Zhang Y, Cui L, Huang H, Chen W, Su Y. Presence of Anti-Thyroid Antibodies Correlate to Worse Outcome of Anti-NMDAR Encephalitis. Front Immunol. 2021 Sep 8;12:725950.
4.Lin JF, Wang JR, Wang JQ, Li JM. The detection of up-regulated anti-thyroid antibodies and autoimmune thyroid diseases in patients with autoimmune encephalitis: a retrospective study of 221 patients. Neurol Sci. 2022 Feb 8.
5.Jung Y, Park S, Son HJ, et al. Thyroid Autoantibody Positive Anti-N-Methyl-D-Aspartate Receptor Encephalitis. Dement Neurocogn Disord. 2016;15(1):24-27.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/combined-anti-n-methyl-d-aspartate-receptor-nmdar-encephalitis-with-autoimmune-thyroiditis-at-with-good-clinical-recovery/