Autoimmune limbic encephalitis: A manifestation of systemic lupus erythematosus in the central nervous system

Abstract

Autoimmune limbic encephalitis (ALE) associated with systemic lupus erythematosus (SLE) is a rare entity with few reports in the literature to date. In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment (RIT), but the pathogenesis of this association is poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active Systemic Lupus Erythematosus disease (SLE) and past medical history of cancer (endometrial adenocarcinoma in 2004 and papillary urothelial carcinoma in 2011 with curative treatment), followed over a one-year period. We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening. Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems (such as hematologic) during the period. However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies.

Neurological complexity in systemic lupus erythematosus: insights into autoimmune encephalitis

“…In most cases, patients had a confirmed history of SLE before experiencing encephalitis symptoms (Angst et al, 2015;Asmaa et al, 2022;Kelley et al, 2018;Li et al, 2022;Raventhiranathan et al, 2022;Selcuk et al, 2011;Stubgen et al, 1998;Sugi et al, 2018;Tsuchiya et al, 2021;Zhang et al, 2021). The time interval between the initial SLE diagnosis and the onset of neurological complications varied from two weeks to 14 years (Angst et al, 2015;Asmaa et al, 2022;Kelley et al, 2018;Li et al, 2022;Selcuk et al, 2011;Stubgen et al, 1998;Zhang et al, 2021). However, four cases initially showed neuropsychiatric symptoms.…”

Central Nervous System Systemic Lupus Erythematosus: Pathophysiologic, Clinical, and Imaging Features

“…Autoimmune antibody-mediated encephalitis (Fig 17) is rare, but the incidence of this condition in SLE may be underestimated. One study (44) showed antibody-mediated inflammation of the striatum in 7% (44,63) of patients with NPSLE, with associated hippocampal lesions (Figs 18, 19) (44,63,64). The mechanism is unclear but is believed to be related to breached neuroimmune interfaces in the BBB and choroid plexus, with invasion by anti-NMDAR, aPL, or anti-Rib-P antibodies resulting in neuronal excitotoxicity.…”

Sisteminės Raudonosios Vilkligės Neurologinės Komplikacijos

“…Šiuo konkrečiu atveju autoimunitetas buvo svarbiausias veiksnys, susijęs su ALE. Stebėtas geras atsakas į imunoterapiją [34]. 34 metų moteriai, sergančiai SRV, pasireiškė bendras nuovargis, traukuliai, atminties praradimas.…”