Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition defined by encephalopathy with acute or subacute onset, the presence of serum anti-thyroid antibodies, and reasonable exclusion of alternative causes. Despite strong response towards corticosteroid treatment, some patients exhibit a chronic-relapsing course and require long-term immunosuppression. Markers for early identification of those patients are still absent.
We retrospectively evaluated a cohort of 22 SREAT patients treated in our Hospital since January 2014.
14 patients with a monophasic disease course and 8 patients with multiple relapses were identified. Baseline characteristics as well as routine CSF parameters did not distinguish between those patients. Flow cytometry following initial relapse therapy displayed a treatment-resistant sequestration of activated CD4+ T cells in patients with a relapsing disease course whereas other lymphocyte subsets showed uniform changes. Such changes were also present in long-term follow-up CSF examination.
Our findings indicate a potential biomarker for risk stratification in SREAT patients. Currently, it remains unclear whether the observed two phenotypes are different spectra of SREAT or represent separate diseases in terms of pathophysiology.

This article is protected by copyright. All rights reserved.

References

PubMed