Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): case report of reversible coma and status epilepticus in an adolescent patient and review of the literature. | LitMetric
Department of Pediatrics, Klinikum Traunstein, Cuno-Niggl-Str. 3, 83278, Traunstein, Germany,
Published: October 2014
AI Article Synopsis
Article Abstract
Unlabelled: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), also termed Hashimoto's encephalopathy (HE), is a rare immune-mediated disorder and is also affecting children and adolescents. It is characterized by altered mental status, seizures, and cognitive dysfunction. Therapeutic options include steroid treatment and prognosis range from complete recovery, a relapsing course to long-term cognitive sequelae. We describe a previously healthy 13-year-old girl presenting to the emergency room with coma and refractory status epilepticus. Generalized tonic-clonic seizures persisted after pre-hospital infusion of antiepileptic medication. She was found to have highly elevated levels of thyroid-stimulating hormone and anti-thyroid peroxidase antibodies not only in blood but also in cerebrospinal fluid while showing negative results for traumatic, infectious, metabolic, toxic, neoplastic, or other known specific autoimmune diseases. Cranial neuroimaging revealed no abnormality. A diagnosis of SREAT was established, and the patient improved rapidly on corticosteroids and levothyroxine therapy. However, 3 months after the discontinuation of steroid treatment, the girl relapsed. The current literature regarding SREAT is reviewed and summarized.
Conclusion: In children with SREAT, early diagnosis and treatment with corticosteroids is crucial and can lead to rapid clinical improvement. Clinicians should be aware of this uncommon but treatable condition, especially in female adolescents with unexplained seizures or an encephalopathic state.
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.
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.
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.
Steroid-responsive encephalopathy associated with thyroiditis (SREAT) is a rare autoimmune disorder that causes cognitive dysfunction, often going unnoticed in medical settings.
A case report highlights a male patient who suffered from various neuropsychiatric symptoms linked to SREAT, stressing the importance of early diagnosis.
The report advocates for recognizing SREAT in patients with unexplained symptoms, showcases the role of steroids in treatment, and emphasizes the need for better diagnostic criteria and personalized treatment plans to improve patient care.
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.
