Rasmussen encephalitis and comorbid autoimmune diseases

Abstract

Objective: To describe a potential association between comorbid autoimmune disease and Rasmussen encephalitis (RE) and discuss potential insights into underlying RE pathogenesis. Methods:We report a case series of 4 patients with RE in whom a comorbid autoimmune disease was subsequently diagnosed and review the literature on possible common susceptibility factors.Results: In 4 patients who presented with typical clinical features of RE, a comorbid autoimmune disease was subsequently diagnosed: Hashimoto thyroiditis, ulcerative colitis, Crohn disease, and systemic lupus erythematosus. We discuss the possible common predisposing factors. Conclusions:The association of RE, a rare entity, with a comorbid autoimmune disease raises the possibility of shared mechanisms of susceptibility, including common immunogenetic and/or environmental risk factors. Neurology ® 2014;83:1049-1055 GLOSSARY a7nAChR 5 a7 nicotinic acetylcholine receptors; AID 5 autoimmune disease; ANA 5 antinuclear antibodies; CD 5 Crohn disease; GWAS 5 genome-wide association studies; HT 5 Hashimoto thyroiditis; Ig 5 immunoglobulin; IVIg 5 IV immunoglobulin; RE 5 Rasmussen encephalitis; SLE 5 systemic lupus erythematosus; T1D 5 type 1 diabetes; UC 5 ulcerative colitis.

Shared HLA Class I and II Alleles and Clonally Restricted Public and Private Brain-Infiltrating αβ T Cells in a Cohort of Rasmussen Encephalitis Surgery Patients

“…The co-occurrence of four different autoimmune diseases, Hashimoto thyroiditis, ulcerative colitis, Crohn disease, and systemic lupus erythematosus in four different RE patients has led to the suggestion that genes that predispose to autoimmunity are risk factors for RE (61). Our finding that some RE patients in our study cohort carry HLA alleles that are known to confer susceptibility to different autoimmune diseases strongly supports this idea.…”

Occupational complexity and lifetime cognitive abilities

“…This association raises the possibility of immunogenetic susceptibility in these patients. 1 We reported a very unusual presentation of RE in a young girl. 2 Diagnosis was difficult because the presenting symptom was hemiparesis while the seizures developed only 4 months later.…”

“…Isabelle Korn-Lubetzki, Jerusalem: Amrom et al 1 reported 4 patients with Rasmussen encephalitis (RE) who were subsequently diagnosed with comorbid autoimmune conditions (ulcerative colitis and Crohn disease in 2). This association raises the possibility of immunogenetic susceptibility in these patients.…”

“…4 In our recent article, we reported another AID, diabetes type 1, in the brother of one individual with RE. 1 Several examples of multiple AIDs occurring in individual patients and within families with a given AID suggested a common underlying etiology, and the concept of shared autoimmunity. 5 We expect that an increasing number of clinical observations of comorbid AID in individual patients with RE, as well as of the occurrence of AIDs in their relatives, will be reported.…”

Epileptic Syndromes With Possible Immunological Mechanisms (Rasmussen Encephalitis, FIRES, West Syndrome, Landau-Kleffner Syndrome)

“…[17] Ayrıca, dört RE'li hastanın seyri sırasın-da diğer otoimmün hastalıkların (Hashimoto tiroiditi, ülse-ratif kolit, Crohn hastalığı, sistemik lupus eritematozus) geliştiği bildirilmiştir. [18] Aynı hastada RE ve komorbid immün Olası İmmünolojik Mekanizmalı Epileptik Sendromlar langıçtaki progresif seyrine rağmen, hastalık süreci orta-ağır nörolojik hasar gelişimini izleyerek sonunda kendi kendine söner. Bu dönemde daha ileri nörolojik hasar görülmez, nöbet sıklığı ve ağırlığı azalır.…”