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Critical Care |

A Rare Neurologic Disorder, Steroid Resistant Hashimoto’s Encephalopathy

Anita Rajagopal, MD; John Lucia, MD
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Department of Internal Medicine, St Vincent Medical Center, Indianapolis, IN


Chest. 2014;145(3_MeetingAbstracts):163A. doi:10.1378/chest.1714064
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Abstract

SESSION TITLE: Critical Care Case Report Posters

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

INTRODUCTION: Hashimoto’s encephalopathy (HE) is a rare neuropsychiatric syndrome which has been controversial due to its unknown pathogenesis and variable clinical course. It is defined by signs of encephalopathy with high thyroid antibody levels demonstrating good responsiveness to glucocorticoid therapy. We report a case of initially steroid responsive HE which became resistant, but responded well to plasmapheresis.

CASE PRESENTATION: A 55-year-old male, with a history of depression and hypothyroidism, presents with acute paranoid psychosis and ideas of reference. Symptoms progressed with further episodes. However, symptoms serendipitously improved with treatment for acute respiratory infection with steroids. Work up revealed antibody positive Hashimoto’s. Initially, he responded well to steroids. However, with time dose increases were required. Once he started to have relapses it became apparent that his condition was now resistant to steroids. Plasmapheresis was started and symptoms began to improve.

DISCUSSION: First described in 1966 [1] HE is characterized by neuropsychiatric symptoms associated with high levels of anti-thyroid antibodies that responds to treatment with corticosteroids. It is often under-recognized and misdiagnosed due to lack of awareness of the condition and to lack of widely accepted diagnostic criteria. Although steroid response is characteristic of HE, other treatments such as azathioprine, cyclophosphamide, plasmapheresis, and IVIG have rarely been used. We report a rare case of HE which was initially steroid responsive that became resistant, but responded well to plasmapheresis.

CONCLUSIONS: This case illustrates that although HE is a rare syndrome, it should be considered in patients presenting with acute encephalopathy or in patients with psychosis with an intermittent course. If steroid resistance is noted, plasmapheresis should be considered. Because the symptoms often respond to treatment, prompt diagnosis and treatment are imperative.

Reference #1: Lord et al. Hashimoto's disease and encephalopathy. Lancet 1966; 2:512-514

DISCLOSURE: The following authors have nothing to disclose: Anita Rajagopal, John Lucia

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