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Clinical features of nine cases of leucine-rich glioma inactivated 1 protein antibody-associated encephalitis

Abstract

To investigate clinical features of leucine-rich glioma inactivated 1 protein (LGI1) antibody-associated autoimmune encephalitis (AE). The clinical data were collected and analyzed in nine patients with LGI1 AE. All nine patients (100%) presented acute/subacute onset, had seizures, cognitive impairment, mental/behavioral abnormalities, six had sleep disorders and seven showed hyponatremia. Seizures manifested in three types: faciobranchial dystonia seizure (FBDS) (44%), mesial temporal lobe epilepsy (MTLE)-like seizure (66%), and focal to bilateral tonic–clonic seizure (FBTCS) (77%). Six of nine cases (66%) showed abnormalities in brain MRI, among them four showed high T2/flair signal on unilateral/bilateral hippocampus, two showed high T1/T2 signal on unilateral basal ganglia. All nine patients (100%) showed abnormalities in EEG, among them 1 (11%) showed diffuse slow waves, 8 (88%) showed focal slow waves; 6 (66%) revealed interictal epileptic discharges; ictal EEG was recorded in five patients, two were FBDS, three were MTLE-like seizure.LGI1 antibodies in serum and cerebrospinal fluid were both positive. No signs of tumor were found in all patients. Eight of nine patients received immunotherapy and antiepileptic drug (AED) treatment, one only treated with AED without immunotherapy. Eight patients improved significantly with seizure-free after immunotherapy, only one still had FBDS after immunotherapy and AED treatment. In LGI1 AE hippocampus and basal ganglia were two main targets, the corresponding seizure type was MTLE-like seizure and FBDS respectively. Diagnosis depended on detection of LGI1 antibodies in CSF. The incidence of tumor was low. The effect of immunotherapy was good and AEDs should be considered as add-on symptomatic treatment.

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Acknowledgement

We are grateful to all the patients who participated in this study. This study was supported by grant from Liaoning Province Science and Technology Plan Project (Grant No: 20180550768).

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Ying Wang is the corresponding author who contributed to the study design and manuscript writing. Ying Li and Fan Song are co-first authors who contributed equally in clinical data collection, analysis and manuscript writing. Wei Liu contributed to EEG analysis. All authors read and approved the final manuscript.

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Correspondence to Ying Wang.

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Li, Y., Song, F., Liu, W. et al. Clinical features of nine cases of leucine-rich glioma inactivated 1 protein antibody-associated encephalitis. Acta Neurol Belg 121, 889–897 (2021). https://doi.org/10.1007/s13760-020-01336-z

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  • DOI: https://doi.org/10.1007/s13760-020-01336-z

Keywords

  • LGI1 antibody
  • Autoimmune encephalitis
  • Faciobranchial dystonia seizure
  • Hyponatremia
  • Immunotherapy