Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

Abstract

Background: Chronic cognitive deficits are frequent in leucin-rich glioma-inactivated 1 protein (LGI1) encephalitis. We examined structural and metabolic brain abnormalities following LGI1 encephalitis and correlated findings with acute and follow-up clinical outcomes. Methods:Nine patients underwent prospective multimodal 3 Tesla MRI 33.1±18 months after disease onset, including automated volumetry, diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS). Data were compared to 9 age-and sex-matched healthy controls. Conclusions:Poor clinical outcome following LGI1 encephalitis is associated with global brain atrophy and disintegration of white matter tracts. The pathological changes affect not only temporomesial structures but also frontal lobes and the cerebellum.

Functional and Structural Brain Alterations in Encephalitis With LGI1 Antibodies

“…The results indicated that abnormal brain connectivity and microstructure in brain areas associated with memory, cognition and motion dysregulation circuits were related to the generic risk of anti-LGI1 encephalitis, which makes it a potential endophenotype for anti-LGI1 encephalitis. The most commonly brain alteration in anti-LGI1 encephalitis was hippocampal atrophy in the previous MRI studies (Finke et al, 2017;Miller et al, 2017;Szots et al, 2017). Correspondingly, we identified the decreased functional connectivity in hippocampus and its correlation with the disease severity in this study.…”

“…The microstructural integrity impairments of the hippocampus (Finke et al, 2017), corona radiata, capsula interna and corpus callosum (Szots et al, 2017) were found in patients with anti-LGI1 encephalitis in the DTI studies. The magnetic resonance spectroscopy (MRS) revealed lower glutamine/glutamate white matter (WM) levels compared with controls (Szots et al, 2017).…”

“…Several positron emission tomography (PET) studies have reported alterations of basal ganglia hyperintensities in LGI1-autoantibody faciobrachial dystonic seizures (FBDS) (Flanagan et al, 2015;d'Orsi et al, 2018). In addition, structural magnetic resonance imaging (MRI) studies have revealed decreased volumes of the hippocampus, pallidum, nucleus accumbens, brainstem and cerebellum in patients with anti-LGI1 encephalitis (Finke et al, 2017;Miller et al, 2017;Szots et al, 2017). Hippocampal atrophy with further reduced mediodorsal thalamic and posteromedial cortical volumes were reported in the limbic encephalitis associated with antibodies to components of the voltage-gated potassium channel complex (VGKCC-Ab-LE), where LGI1 was the prominent autoantibody (Loane et al, 2019).…”

“…Besides, diffusion tensor imaging (DTI) has been extensively applied to unveil white matter abnormalities in diverse neurological diseases (Gatto et al, 2018;Gatto and Weissmann, 2019). The microstructural integrity impairments of the hippocampus (Finke et al, 2017), corona radiata, capsula interna and corpus callosum (Szots et al, 2017) were found in patients with anti-LGI1 encephalitis in the DTI studies. The magnetic resonance spectroscopy (MRS) revealed lower glutamine/glutamate white matter (WM) levels compared with controls (Szots et al, 2017).…”

Single‐center study of autoimmune encephalitis‐related autoantibody testing in Hungary

“…The target molecules of these autoantibodies play important roles in synaptic signal transmission and neuronal plasticity. The autoimmune reaction to these antigens in the majority of cases leads to epileptic seizures and neuropsychiatric symptoms (Table ) (Celicanin et al, ; Fukata, Yokoi, & Fukata, ; Honnorat & Plazat, ; van Sonderen, Petit‐Pedrol, Dalmau, & Titulaer, ; Szots et al, ). In autoimmune encephalitis (AIE), the autoantibodies bind to the extracellular epitopes of the neuronal cell surface receptors or their associated proteins, which can lead to alteration of the structure and function of target antigens by different mechanisms.…”