Being treatable, steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), or Hashimoto's encephalopathy, should be distinguished from untreatable conditions. Our patient was a previously healthy 89-year-old man, who presented with cognitive and balance deterioration over several months. His cerebrospinal fluid (CSF) examination was positive for protein 14-3-3 but no other test suggested Creutzfeldt-Jacob disease. His condition improved markedly, although not fully, with intravenous corticosteroids. In control CSF sampling, protein 14-3-3 was negative but a biomarker signature consistent with Alzheimer's disease was observed. SREAT should be considered also in the very elderly in case of subacute encephalopathy.
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http://dx.doi.org/10.1093/omcr/omy073 | 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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