Neuropsychological and Structural Neuroimaging Outcomes in LGI1-Limbic Encephalitis: A Case Study

OBJECTIVE: Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (LGI1-LE) is a rare autoimmune condition that affects the structural integrity and functioning of the brain’s limbic system. Little is known about its impact on long-term neuropsychological functioning and the structural integrity of the medial temporal lobe. Here we examined the long-term neuropsychological and neuroanatomical outcomes of a 68-year-old male who acquired LGI1-LE. METHODS: Our case patient underwent standardized neuropsychological testing at two time points. Volumetric analyses of T1-weighted images were undertaken at four separate time points and qualitatively compared with a group of age-matched healthy controls. RESULTS: At the time of initial assessment, our case study exhibited focal impairments in verbal and visual episodic memory and these impairments continued to persist after undergoing a course of immunotherapy. Furthermore, in reference to an age-matched healthy control group, over the course of 11 months, volumetric brain imaging analyses revealed that areas of the medial temporal lobe including specific hippocampal subfields (e.g., CA1 and dentate gyrus) underwent a subacute period of volumetric enlargement followed by a chronic period of volumetric reduction in the same regions. CONCLUSIONS: In patients with persisting neurocognitive deficits, LGI1-LE may produce chronic volume loss in specific areas of the medial temporal lobe; however, this appears to follow a subacute period of volume enlargement possibly driven by neuro-inflammatory processes.