A Case of Steroid-Responsive Encephalopathy

Abstract

Hashimoto's encephalopathy (HE), also known as steroid-responsive encephalopathy, is associated with autoimmune-mediated thyroiditis. Onset is typically gradual often with evolution over the course of months. Characteristic symptoms include impaired concentration and memory, delusions, hallucinations, personality changes, incoordination, tremor, hemiparesis, seizures, and speech difficulties. Diagnosis is predicated upon discovery of elevated anti-thyroid antibodies, specifically anti-thyroid peroxidase (TPO) or anti-M antibodies. Some patients may also present with subclinical hypothyroidism but many are euthyroid. Of note, neither thyroid function tests or antibody titers correlate with disease severity. Other common laboratory findings include elevations in sedimentation rate, liver enzymes, and cerebrospinal fluid (CSF) protein. Radiological work-up, including cerebral angiography, is often normal. Successful treatment includes administration of steroids and/or intravenous immunoglobulin (IVIg) or plasmapheresis. We describe a case of a 74-year-old male who presented for evaluation of abrupt onset confusion ultimately determined to be a consequence of autoimmune-mediated thyroiditis.

The use of intravenous immunoglobulin in the treatment of Hashimoto’s encephalopathy: case based review

“…Depending on the case, corticosteroid therapy duration can vary from months to years ( 79 ). There is typically an improvement or complete resolution of the symptoms within a few months ( 5 ). In the event of disease recurrence or occurrence of side effects associated with steroids, other immunotherapies can be added, such as mycophenolate, azathioprine, cyclophosphamide, methotrexate, rituximab ( 80 , 81 ).…”

“…In some cases, IVIG was used as the first-line therapy, either alone or in combination with steroid therapy ( 5 , 6 , 9 , 10 ). These cases were published in the last 7 years, so the initial use of IVIG may be the result of the data found in literature which sustains the beneficial effects of IVIG in the treatment of HE.…”

Hashimoto’s Thyroiditis Presents as an Acute Encephalopathy: A Case Report of Unusual Presentation

“…It is suggested that ATA are the hallmark of HE rather than playing a direct role in the pathogenesis of HE [7]. Hence, the more accurate name for this condition is steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) [8,9].…”

“…There is a broad spectrum of presenting manifestations; however, encephalopathy manifesting with abrupt confusion with an altered level of consciousness is the hallmark presentation [8,10]. Two patterns of cognitive dysfunction are predominant; a stroke-like pattern which is more acute to subacute, and a gradual diffuse progressive pattern which might result in dementia, hallucination, or even lethargy, progressing to coma in extreme cases [8,10].…”

“…Accompanying non-cognitive symptoms include seizure (in approximately two-thirds of patients), myoclonus (seen in up to 38%), loss of consciousness [10], tremors, hyperreflexia, poor appetite, ataxia, and dysarthria [8]. Besides, psychosis, in the form of paranoid delusion and visual hallucinations, has been reported in 25%-36% [4,8]. Anxiety, depression, hypersomnia, and difficulty initiating sleep are initial presenting symptoms [11].…”