Hashimoto’s Encephalopathy: Benefit from Chronic Immunosuppressive Therapy in Steroid Non-Responder
Hashimoto’s Encephalopathy
Keywords:
Encephalopathy, Non-vasculitic autoimmune meningoencephalitis (NAIM), Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT)Abstract
Hashimoto’s Encephalopathy (HE) is thought to be a steroid-responsive process. However, there have been several cases reported in the literature that did not respond to steroids and requiredintravenous immunoglobulin, plasmapheresis or oral immunosuppressants. This is a case of a 67-year-old lady with rapidly progressing altered mental status over four weeks. MRI brain showed extensive diffuse white matter changes without contrast enhancement. EEG showed diffuse slowing and triphasic waves. Meningitis-encephalitis, paraneoplastic and autoimmune encephalitides, and Creutzfeldt-Jacob disease were ruled out through cerebrospinal fluid analysis and serological studies. Work-up revealed an elevated thyroid-peroxidase (TPO) antibody titer. Patient was treated for HE initially with 6-day course of high-dose IV methylprednisolone with a failure to improve; followed by a 5-day course of IVIG (2 mg/kg) with minimal improvement;then maintenance methotrexate 2.5 mg weekly with a good response -neurological exam improved significantly, and brain MRI demonstrated improvement of supratentorial lesions, and resolution of cerebellar T2/FLAIR hyperintensities. At 2-month clinic follow-up, patient’s mentation was back to baseline with ability to perform activities of daily living (ADLs) with minimal support. Hence, methotrexate taper was attemptedbut had subsequent worsening of confusion and functional status, which required resumption of the therapy. The chronic management of this debilitating illness is poorly understood, especially in the steroid non-responders. Further clinical studies should be directed towards better understanding of the natural course of this disease and to reach a consensus about the chronic management of the relapsing forms. [Shah V Natl J Integr Res Med, 2019; 10(1):54-57]
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