Objective: to describe the characters of 4 female patients suffering from SREAT, phenotypes of movement disorders, cognitive impairment, and outcomes.

Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) or Hashimoto encephalopathy was previously known as non-vasculitic autoimmune inflammatory meningoencephalitis. Patients with SREAT mainly presented with encephalopathy with various neurological abnormalities, including behavioral change, seizure, stroke-like episodes, tremors, psychosis, and mood disturbance. Serology study of antithyroid peroxidase (anti-TPO) and anti-thyroglobulin antibodies (anti-TG) plays a role in establishing the diagnosis.

Furthermore, movement disorders such as chorea, ataxia, myoclonus, tics, and multiple abnormal movement phenomenology are reported.

Method: Case series

Results: Four female patients were diagnosed with SREAT from high levels of serum anti-TPO and anti-TG. Two are elderly, and two are in middle age. Three cases developed chorea as the clinical manifestation. Only one case with Holmes tremor and parkinsonism did not develop confusion, only cognitive decline. One case suffered from a neuropsychiatric problem and was treated as having a major depressive disorder. Two cases with confusion had multiple systemic diseases, especially renal failure with uremia, which interfered with the diagnosis of SREAT and made doctors ignore the investigation. The critical point to recognize SREAT is movement disorders. The exclusion of autoimmune encephalitis and other toxic-metabolic disorders confirmed the effect of SREAT on neuropsychiatric problems or movement disorders/seizures in all cases. Although theoretically, this condition responded to steroids, serious complications such as sepsis and septic shock could occur and play a role in harmful outcomes in elderly and multiple organ failure patients. 

Conclusion: Although SREAT is rare, some encephalopathic cases are caused by this autoimmunity. Advanced aging and multiple coexisting medical illnesses seem to be the factor of drawbacks after pulse methylprednisolone treatment.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/case-series-of-steroid-responsive-encephalopathy-associated-with-autoimmune-thyroiditis-sreat-different-presentations-and-different-treatment-outcomes/