Abstract: FR-PO044

Successful Treatment of Hashimoto Encephalopathy with Therapeutic Plasma Exchange

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Attique, Hassan Bin, University of CT Health Center, West Hartford, Connecticut, United States
  • Rao, Arundati, University of CT Health Center, West Hartford, Connecticut, United States
  • Haider, Lalarukh, University of CT Health Center, West Hartford, Connecticut, United States
  • Trivedi, Ruchir D., University of CT Health Center, West Hartford, Connecticut, United States
Background

Hashimoto’s encephalopathy (HE) is a rare neuropsychiatric syndrome characterized by encephalopathy of unknown etiology associated with the high titers of antithyroid antibodies in the absence of alternative diagnoses. HE with robust clinical response to therapeutic plasma exchange (TPE) in the setting of end stage renal disease (ESRD) has not been published. We present steroid-resistant HE with reliable reduction in antithyroid peroxidase antibodies (anti-TPO), after 5 sessions of TPE along with linear improvement in clinical status.

Methods

72 year old lady with ESRD on maintenance hemodialysis (HD) presented with altered mental status. Investigations including CT scan did not suggest central nervous system (CNS) infection, tumor or stroke. There were no signs of uremia or underdialysis as well. Further investigations revealed status epilepticus controlled with propofol infusion apart from maximal dose of three anti-epileptic agents. As part of resistant status epilepticus work up, free thyroxine was 0.52 ng/dl, thyroglobulin antibody level of 2299 IU/ml and anti-TPO antibody level was 4886 IU/ml. Her cerebrospinal fluid (CSF) anti-TPO antibody was 36.4 IU/ml and CSF protein of 50 mg/dl. This led to diagnosis of HE and treatment with pulse dose IV Methylprednisone 500mg every day was initiated for five days. She remained clinically refractory for which 1.5 plasma volume TPE was initiated using 5% albumin as replacement fluid on alternate days in an attempt to remove detectable antibodies and provide immunomodulation. She continued to receive daily HD to optimize fluid electrolyte status during entire course. Clinical condition improved with reduction in anti-TPO antibody. She was continued on maintenance steroids and rituximab.

Conclusion

HE is presumably autoimmune in origin. Proposed etiology includes autoimmune reaction between antibodies and cerebral vascular and brain cells and perivascular lymphocytic inflammation. Available literature is unclear about pathogenic role of antithyroid antibodies. Our case did not improve with steroid but showed remarkable clinical improvement with TPE. This suggests possible immunomodulatory role of TPE in select case of acute HE. Our case is in line with considerable variance that exists in ESRD and non-ESRD population. ESRD may represent a distinct subgroup in which HE may respond favourably to TPE.