Steroid-responsive encephalopathy associated with autoimmune thyroiditis presenting as cortisone sensible psychosis with reversible leukoencephalopathy

J Neuroimmunol. 2023 Sep 15:382:578177. doi: 10.1016/j.jneuroim.2023.578177. Epub 2023 Aug 9.

Abstract

Introduction: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a frequently discussed neuropsychiatric syndrome with elevated thyroid antibodies in the context of various clinical neuropsychiatric phenotypes. MRI abnormalities are usually nonspecific and treatment can be complex.

Case study: We present a case of a woman in her sixties with SREAT whose psychosis kept worsening under cortisone tapering. After three years with cortisone side effects, therapy was changed to plasmapheresis and rituximab treatment with an excellent initial response, subacute unexplained deterioration with extensive leukoencephalopathy on MRI shortly after, and full recovery with regression of leukoencephalopathy afterwards.

Discussion: SREAT varies in clinical and diagnostic presentation. Its precise pathophysiology is unknown, as are the best treatment protocols. The case illustrates that some patients with SREAT syndrome might end up in constellations, in which it proves difficult to wean off steroid treatment and illustrates treatment alternatives such as plasmapheresis and/or rituximab. In addition, it highlights leukoencephalopathy as possible MRI finding in the context of SREAT. Further research is necessary to fully comprehend the (potentially different) pathomechanisms and courses of SREAT.

Keywords: Autoimmune; Depression; Encephalopathy; Epilepsy; Hashimoto; Leukoencephalopathy; Psychosis; SREAT; Thyroiditis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brain Diseases* / drug therapy
  • Cortisone* / therapeutic use
  • Female
  • Hashimoto Disease* / complications
  • Hashimoto Disease* / diagnosis
  • Humans
  • Leukoencephalopathies*
  • Psychotic Disorders* / complications
  • Rituximab / therapeutic use
  • Steroids
  • Thyroiditis, Autoimmune* / complications
  • Thyroiditis, Autoimmune* / drug therapy

Substances

  • Cortisone
  • Rituximab
  • Steroids

Supplementary concepts

  • Hashimoto's encephalitis