Department of Psychiatry and Psychotherapy, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Department of Neurology and Neurophysiology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
J Neuroimmunol. 2023 Sep 15;382:578177. doi: 10.1016/j.jneuroim.2023.578177. Epub 2023 Aug 9.
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.
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.
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.
伴有自身免疫性甲状腺炎的类固醇反应性脑病(SREAT)是一种经常讨论的神经精神综合征,在各种临床神经精神表型中伴有甲状腺抗体升高。MRI 异常通常是非特异性的,治疗可能很复杂。
我们报告了一例 60 多岁的女性 SREAT 患者,其在皮质类固醇逐渐减少的过程中精神病不断恶化。在皮质类固醇副作用持续了三年后,治疗改为血浆置换和利妥昔单抗治疗,初始反应良好,随后出现亚急性原因不明的恶化,MRI 显示广泛的脑白质病,随后脑白质病消退,完全恢复。
SREAT 在临床表现和诊断上存在差异。其确切的病理生理学尚不清楚,最佳治疗方案也不清楚。该病例表明,一些 SREAT 综合征患者可能会出现难以逐渐减少类固醇治疗的情况,并说明了其他治疗选择,如血浆置换和/或利妥昔单抗。此外,它强调了脑白质病可能是 SREAT 背景下的 MRI 发现。需要进一步研究以充分了解 SREAT 的(潜在不同的)发病机制和病程。