Seizures in steroid-responsive encephalopathy
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REVIEW ARTICLE
Seizures in steroid-responsive encephalopathy Xin Xu 1 & Aolei Lin 1 & Xuefeng Wang 1 Received: 3 June 2020 / Accepted: 6 November 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract Steroid-responsive encephalopathy is a general term for diseases that are characterized by diffuse brain injury and respond well to corticosteroids or immunosuppressive agents, including Hashimoto’s encephalopathy (HE), limbic encephalitis (LE), systemic lupus erythematosus encephalopathy (SLEE), antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitis encephalopathy (AASV), viral encephalitis (VE), and primary central nervous system lymphoma (PCNSL). Epilepsy and status epilepticus are the main manifestations of steroid-responsive encephalopathy. The spectrum of “autoimmune epilepsy” diseases, which has been approved by the epilepsy diagnostic recommendations of the International Antiepileptic League, is characterized by a high prevalence of epilepsy in central nervous system (CNS) autoimmune diseases and a variety of neuron-specific autoantibodies. Steroid-responsive encephalopathy with different causes may have different pathogeneses and has been suggested to be associated with some internal commonality producing seizure as the main symptom. Determining the regularity of seizures caused by steroid-responsive encephalopathy and implementing appropriate measures will help us improve the prognosis of patients. This paper summarizes the epidemiology, seizure onset, seizure type, and other characteristics of seizures in steroid-responsive encephalopathy (including HE, LE, SLEE, ANCA-associated systemic vasculitis encephalopathy, VE, and PCNSL) and then discusses the use of antiepileptic drugs to treat steroid-responsive encephalopathy. Keywords Steroid-responsive encephalopathy . Seizure . Status epilepticus . Central nervous system . Autoimmunity
Historical development The relationship between epilepsy and the autoimmune response has long been recognized. In 1966, Brain et al. first described a possible link between the CNS and the autoimmune response, proposing Hashimoto’s encephalitis, later known as steroid-responsive encephalopathy and associated autoimmune thyroiditis (SREAT), in which seizures may complicate the systemic autoimmune disorder [1]. In 1968, Corsellis et al. reported the first case of LE, with cognitive decline, behavioral changes, and seizures as the primary manifestations, and they considered it to be a tumor-associated Xin Xu and Aolei Lin contributed equally to this work and share first authorship. * Xuefeng Wang [email protected] 1
Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Neurology, 1Youyi Road, Chongqing 400016, China
disease [2]. Therefore, tumors and encephalitis have been suggested to have autoimmune associations to some extent. The increased frequency of seizures in systemic autoimmune diseases, such as systemic lupus erythematosus, provides further evidence for the link between autoimmune
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