A rare case of anti-LGI1 limbic encephalitis with concomitant positive NMDAR antibodies

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A rare case of anti-LGI1 limbic encephalitis with concomitant positive NMDAR antibodies Tuo Ji†, Zhi Huang†, Yajun Lian* , Chengze Wang, Qiaoman Zhang and Jinghong Li

Abstract Background: N-methyl-D-aspartate receptor (NMDAR) and leucine-rich glioma-inactivated 1 (LGI1) antibodies define the most prevalently recognized autoimmune encephalitis syndromes, while the simultaneous occurrence of both conditions has hardly been published before. Case presentation: We report the case of a 67-year-old patient who presented with generalized tonic–clonic seizures (GTCS) followed by behavioral changes, psychosis, sleep disorders, decreased consciousness, and faciobrachial dystonic seizures. Ancillary findings included serum hyponatremia and imaging evidence of high-intensity lesions within bilateral medial temporal lobes on T2-weighted fluid attenuation inversion recovery. Both LGI1 and NMDAR antibodies were positive in serum and cerebral spinal fluid using transfected cell based assays. Despite prominent clinical features of anti-LGI1 limbic encephalitis (LGI1-LE), the patient also exhibited overlapping symptoms of anti-NMDAR encephalitis, like early-onset GTCS, which might be related to the concomitant positive NMDAR antibodies. Conclusions: We report a rare case of LGI1-LE with overlapping symptoms and simultaneous positive NMDAR antibodies. It is necessary to evaluate the presence of NMDAR antibodies in certain LGI1-LE patients with unusual symptoms. However, caution should be exercised in interpreting the observation, given the fact of a single-case study. Keywords: Anti-leucine-rich glioma-inactivated 1, Limbic encephalitis, N-methyl-D-aspartate receptor, Antibody

Background Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (LGI1-LE) is an auto-antibody mediated disorder characterized by an acute to sub-acute onset of confusion and cognitive impairment, facio-brachial dystonic seizures (FBDS) and psychiatric disturbances [1, 2]. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune nervous system disease, the major symptoms of which include abnormal behavior or cognitive dysfunction, speech dysfunction, seizures, movement disorders or dyskinesia or abnormal posture, decreased consciousness, and autonomic dysfunction or central hypoventilation [3]. NMDAR and * Correspondence: [email protected] † Tuo Ji and Zhi Huang contributed equally to this work. Department of neurology, First Affiliated Hospital of Zhengzhou University, Zhengzhou City 450052, Henan Province, China

LGI1 antibodies define the most prevalently recognized autoimmune encephalitis (AE) syndromes, while the simultaneous occurrence of both conditions has hardly been published before [4]. We herein describe the case of a 67-year-old man with LGI1-LE presenting overlapping symptoms and simultaneous positive NMDAR antibodies. The aim of this report is to add knowledge on the possible clinical spectrum of anti-LGI1 and antiNMDAR overlapping syndromes.

Case presentation A 67-year-old Chinese male