(602) Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis (SREAT)

Sunday, January 21, 2024

2:30 PM – 3:30 PM MT

Location: Connections Central - RST 06

First Author(s)

KM

Ketan Mehta, DO

University of Maryland Medical Center
Baltimore, MD

Co-Author(s)

MW

Matthew Woodward, DO

Neurology, Critical Care Fellow
University of Maryland Medical Center, United States
GP

Gunjan Parikh, MD

Neurology, Critical Care
University of Maryland School of Medicine Shock Trauma, United States
NB

Neeraj Badjatia, MD, MS, FCCM

Neurology, Critical Care
University of Maryland Medical Center, R. Adams Cowley Shock Trauma Center
Baltimore, MD, United States
MP

Melissa Pergakis, MD

Neurology, Critical Care
University of Maryland Medical Center, United States

Introduction: Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) is an uncommon syndrome in patients with Hashimoto thyroiditis. It is difficult to recognize as it is independent of hypo- or hyperthyroidism.

Description: 59-year-old female with complaints of poor appetite and newly diagnosed mild hypothyroidism was intubated for convulsive status epilepticus (SE). Lab results were significant for hyponatremia, anemia, thrombocytopenia, hypomagnesemia, hypophosphatemia, hypoalbuminemia, mild transaminitis. Toxicology negative. She was hemodynamically stable. Full body CT was notable for bibasilar aspiration. Empiric dexamethasone, acyclovir, ceftriaxone, and piperacillin-tazobactam were started.

MRI, LP, and CSF autoimmune and paraneoplastic panel (ENC-2) were unremarkable. EEG showed focal SE with onset in the right posterior temporal and left posterior quadrants treated with levetiracetam, phenytoin, propofol and midazolam infusions, and empiric high dose thiamine. On hospital day (HD) 2, she continued to have seizures and treatment escalation included ketogenic diet, ketamine drip, phenobarbital, and perampanel.

HD12 SE resolved but she remained altered with worsening hyperkinetic movements and agitation. Full body PET/CT, repeat LP, oligoclonal bands, Borrelia Burgdorferi, repeat ENC-2 panel, rheumatoid factor, anti-neutrophil cytoplasm antibodies, anti-cardiolipin, Sjogren, C3/C4, anti-extractable nuclear antigen, and celiac antibodies were negative. Plasmapheresis and high dose steroids were started for possible antibody-mediated encephalitis. Hyperactivity improved on HD29. SREAT was considered, anti-thyroid peroxidase and thyroglobulin antibody were elevated.

Discussion: Differential includes Creutzfeldt-Jakob disease, acute disseminated encephalomyelitis, toxic metabolic encephalopathy, meningoencephalitis, carcinomatous meningitis, paraneoplastic or autoimmune encephalitis, degenerative dementia, stroke, basilar or hemiplegic migraine, cerebral vasculitis, and psychiatric disease. These etiologies were ruled out with negative lumbar puncture, MRI, blood, and CSF testing. Diagnosis is supported by elevated antithyroid peroxidase antibody or antithyroglobulin antibody and improvement with steroids. She is doing well on steroid taper.