Clinical features of anti-leucine-rich glioma-inactivated 1 encephalitis in Northeast China

Abstract Aim To examine the clinical characteristics, laboratory tests, imaging and electroencephalography presentation, treatment, and prognosis of anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis and improve the awareness of this disease. Method We performed a retrospective analysis of the clinical data of 41 patients who were diagnosed with anti-LGI1 encephalitis. Their clinical characteristics, laboratory tests, and imaging and electroencephalography data were collected, and the treatment results and prognosis were evaluated. The modified Rankin Scale (mRS) was used to evaluate neurological function. Results A total of 41 patients were included in the study, the average follow-up time is 33.0 months.The initial symptoms included cognitive impairment (n = 16, 39.0%),faciobrachial dystonic seizures (FBDS) (n = 12, 29.3%), grand mal seizures (n = 5, 12.2%) hallucinations (n = 4, 9.8%), loss of consciousness (n = 2, 4.9%), nausea and vomiting (n = 1, 2.4%),and head discharge-like sensation and radiate one limb (n = 1, 2.4%). There were 20 and 21 patients in the good (mRS ≤ 2) and poor (mRS > 2) prognosis groups, respectively. In the good prognosis group, the initial symptoms included faciobrachial dystonic seizures (FBDS) (n = 6, 30.0%), cognitive impairment (n = 6, 30.0%), hallucinations (n = 4, 20.0%), grand mal seizures (n = 3, 15.0%), loss of consciousness (n = 2, 10.0%), and nausea and vomiting (n = 1, 5.0%). There were 10 patients with hyponatremia. Magnetic resonance imaging (MRI) showed limbic system involvement in 12 patients (60.0%).17 patients (85.0%) recovered, 2 (10.0%) showed significant improvement, and 1 (5.0%) died after a mean follow-up period of 36.9 months. In the poor prognosis group, the initial symptoms included FBDS (n = 6, 28.5%), cognitive impairment (n = 10, 47.6%), grand mal seizures (n = 2, 9.5%), and electric shock-like sensation in the left limbs (n = 1, 4.7%). There were 20 patients with hyponatremia. MRI showed limbic system involvement in 11 patients (52.4%). 11 patients (52.4%) recovered, 8 (38.1%) showed significant improvement, and 2 (9.5%) died after a mean follow-up period of 29.0 months. Conclusions The clinical characteristics of anti-LGI1 encephalitis include hyponatremia, FBDS, epileptic seizures, hallucinations, cognitive impairment, and loss of consciousness, while the rarely seen characteristics are nausea, vomiting, and other autonomic dysfunctions and electric shock-like sensation. The appearance of hallucinations often indicates a good prognosis.Hyponatremia and elevated cerebrospinal fluid protein levels can be used as indicators that affect the prognosis of patients.Limbic system involvement has nothing to do with prognosis.Attention should be paid to early diagnosis and timely first-line immunotherapy.