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.
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http://dx.doi.org/10.1007/s10072-020-04891-8 | DOI Listing |
Neurol Sci
December 2024
Neurophysiopathology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Introduction: Biallelic variants in QARS1, a house-keeping gene involved in protein synthesis, cause a rare encephalopathy classically characterized by severe developmental delay, drug-resistant neonatal-onset epilepsy, microcephaly, and brain atrophy. We aim to raise awareness on mild QARS1-related phenotypes describing a 6-year-old patient.
Case Description: Epilepsy onset occurred at 3.
Cureus
November 2024
Neurology, Sheikh Khalifa Medical City, Abu Dhabi, ARE.
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition that can present with multiple neurological and psychiatric manifestations. SREAT diagnosis poses a challenge due to the nature of its nonspecific symptomatology and its overlap with numerous autoimmune, metabolic, infectious, and neuropsychiatric disorders. It is associated with elevated anti-thyroid antibodies, occurs in correspondence with autoimmune thyroiditis, and shows great response to corticosteroid treatment.
View Article and Find Full Text PDFCureus
September 2024
Internal Medicine, Saveetha Medical College and Hospitals, Saveetha Institute of Medical and Technical Sciences, Chennai, IND.
Steroid-responsive encephalopathy, also known as Hashimoto's encephalopathy, is an uncommon autoimmune neuroendocrine disorder linked to thyroiditis. It presents a variable range of clinical symptoms, making it challenging to diagnose. SREAT (steroid-responsive encephalopathy associated with autoimmune thyroiditis) is considered a diagnosis of exclusion.
View Article and Find Full Text PDFClin Neuropsychol
August 2024
Department of Neuropsychology, Semmes-Murphey Neurologic and Spine Institute, Memphis, TN, USA.
Hashimoto's encephalopathy (HE), a rare immune-mediated disorder, manifests as altered mental state, cognitive and psychological dysfunction, seizures, and myoclonus. Little is known, however, about the neuropsychological profiles of individuals with HE due to the sparse amount of research. This report overviews HE, summarizes findings from available published neuropsychological evaluations, and details neuropsychological examinations of a 57-year-old White woman with a confirmed HE diagnosis evidencing persistent neuropsychological impairment at two discrete timepoints.
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