Autoimmune limbic encephalitis in association with acute stroke

<b><i>Introduction:</i></b> Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is clinically heterogeneous, especially at presentation, and though it is sometimes found in association with tumor, this is by no means the rule. <b><i>Methods:</i></b> Clinical data for 10 patients with anti-LGI1 encephalitis were collected including one case with teratoma and nine cases without and compared for clinical characteristics. Microscopic pathological examination and immunohistochemical assay of the LGI1 antibody were performed on teratoma tissue obtained by laparoscopic oophorocystectomy. <b><i>Results:</i></b> In our teratoma-associated anti-LGI1 encephalitis case, teratoma pathology was characterized by mostly thyroid tissue and immunohistochemical assay confirmed positive nuclear staining of LGI1 in some tumor cells. The anti-LGl1 patient with teratoma was similar to the non-teratoma cases in many ways: age at onset (average 47.3 in non-teratoma cases); percent presenting with rapidly progressive dementia (67% of non-teratoma cases) and psychiatric symptoms (33%); hyponatremia (78%); normal cerebrospinal fluid results except for positive LGI1 antibody (78%); bilateral hippocampal hyperintensity on magnetic resonance imaging (44%); diffuse slow waves on electroencephalography (33%); good response to immunotherapy (67%); and mild residual cognitive deficit (22%). Her chronic anxiety and presentation with status epilepticus were the biggest differences compared with the non-teratoma cases. <b><i>Conclusion:</i></b> In our series, anti-LGI1 encephalitis included common clinical features in our series: rapidly progressive dementia, faciobrachial dystonic seizures, behavioral disorders, hyponatremia, hippocampal hyperintensity on magnetic resonance imaging, and residual cognitive deficit. We observed some differences (chronic anxiety and status epilepticus) in our case with teratoma, but a larger accumulation of cases is needed to improve our knowledge base.

Background Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is clinically heterogeneous, especially at presentation, and though it is sometimes found in association with tumor, this is by no means the rule. Methods Clinical data for 10 people with anti-LGI1 encephalitis and 3 people with anti-N-Methyl-D-aspartate receptor (NMDAR) encephalitis with teratoma were collected. Microscopic pathological examination and immunohistochemical (IHC) assay of the LGI1 antibody were performed on teratoma tissue obtained by laparoscopic oophorocystectomy. Results In our teratoma associated anti-LGI1 encephalitis case, teratoma pathology was characterized by mostly thyroid tissue and IHC assay confirmed partial or focal positive nuclear staining of LGI1 in some tumor cells. The case was similar to the non-teratoma (NT) group in many ways: age at onset; percent presenting with rapidly progressive dementia (RPD) and psychiatric symptoms; hyponatremia; normal cerebrospinal fluid (CSF) results except for positive LGI1 antibody; bilateral hippocampal hyperintensity on magnetic resonance imaging (MRI); diffuse slow waves on electroencephalogram (EEG); good response to immunotherapy and mild residual cognitive deficit. Her chronic anxiety and status epilepticus (SE) were the biggest differences compared with NT group. Interestingly, the case presented many differences compared with anti-NMDAR encephalitis with teratoma: older onset age, prominent anxiety, SE, hyponatremia, normal CSF cell count, hippocampal hyperintensity on MRI and slowly recovered and residual short-term memory impairment. Conclusion In our series, anti-LGI1 encephalitis included common clinical features: RPD, faciobrachial dystonic seizures, behavioral disorders, hyponatremia, T2-MRI hyperintensity of hippocampus and residual cognitive deficit, but a larger accumulation of cases is needed to improve our knowledge base.

Background Leucine-rich glioma-inactivated 1 (LGI1) encephalitis is clinically heterogeneous, especially at presentation, and though it is sometimes found in association with tumor, this is by no means the rule. The aim of this study is to report a case of teratoma-associated LGI1 encephalitis, to summarize a retrospective series of LGI1 encephalitis and compare them. Methods Clinical data for 10 people with LGI1 encephalitis were collected. Patients were divided into those with and without associated teratoma, and compared for clinical characteristics. Microscopic pathological examination and immunohistochemical (IHC) assay of the LGI1 antibody were performed on teratoma tissue obtained by laparoscopic oophorocystectomy. Results The LGl1 patient with teratoma was similar to the non-teratoma (NT) group in many ways: age at onset (average 47.3 in NT group); percent presenting with rapidly progressive dementia (RPD) (67% of NT group) and psychiatric symptoms (33%); hyponatremia (78%); normal cerebrospinal fluid results except for positive LGI1 antibody (78%); bilateral hippocampal hyperintensity on magnetic resonance imaging (MRI) (44%); diffuse slow waves on electroencephalography (EEG) (33%); good response to immunotherapy (67%); and mild residual cognitive deficit (22%). Her chronic anxiety and presentation with status epilepticus were the biggest differences compared with the NT group. Teratoma pathology was characterized by mostly thyroid tissue and IHC assay confirmed positive LGI1 expression. Conclusion In our series, LGI1 encephalitis included common clinical features: RPD, faciobrachial dystonic seizures, behavioral disorders, hyponatremia, T2-MRI hyperintensity of hippocampus and residual cognitive deficit. We observed some differences in our case with teratoma, but a larger accumulation of cases is needed to improve our knowledge base.