Hashimoto Encephalopathy Presenting with New Onset Refractory Status Epilepticus and Clinical Hyperthyroidism

Case Report

Austin Med Sci. 2022; 7(1): 1063.

Hashimoto Encephalopathy Presenting with New Onset Refractory Status Epilepticus and Clinical Hyperthyroidism

Aljaberi N¹, Elkambergy H¹, Achi E² and Dibu JR¹*

¹Neurologic Critical Care Unit, Critical Care Institute, Cleveland Clinic Abu Dhabi, United Arab Emirates

²Epilepsy Department, Neurological institute, Cleveland Clinic Abu Dhabi, United Arab Emirates

*Corresponding author: Jamil R. Dibu, Neurologic Critical Care Unit, Critical Care Institute, Cleveland Clinic Abu Dhabi, United Arab Emirates

Received: March 23, 2022; Accepted: April 14, 2022; Published: April 21, 2022

Abstract

NORSE occurs secondary to multiple etiologies including a rare diagnosis of Hashimoto Encephalopathy (HE). The mechanism of HE is not fully understood. Most reported patients with HE are hypothyroid or euthyroid at the time of presentation. Patients with HE commonly present with subacute clinical features, namely behavioral changes, and rarely with the acute presentation of status epilepticus. We report a case of a 14 years old female presenting with New Onset Refractory Status Epilepticus (NORSE) and clinical hyperthyroidism, subsequently developing behavioral symptoms with multiple relapses requiring various treatment modalities including pulse steroid therapy, intravenous immunoglobulin therapy, plasmapheresis and eventually partial thyroidectomy.

Keywords: Hashimoto Encephalopathy; Status Epilepticus; Hyperthyroidism

Background

Status epilepticus is a life-threatening medical emergency that occurs secondary to multiple etiologies including an uncommon but increasingly recognized diagnosis of Hashimoto Encephalopathy (HE). HE, also known as Steroid- Responsive Encephalopathy associated with Autoimmune Thyroiditis (SREAT), was originally described by Brain et al in 1966 [1], has an estimated prevalence of 2:100.000 [2] and is associated with Hashimoto thyroiditis with elevated antithyroid antibodies titers. The mechanism of HE is unknown however it is believed to be an immune mediated disorder with an inflammatory process affecting the central nervous system rather than the direct effect of an altered thyroid state on the brain [3] as most reported patients with HE were euthyroid at the time of presentation [2]. Patients with HE commonly present with subacute clinical features such as cognitive and behavioral changes in a hypothyroid state and rarely present acutely with status epilepticus [2]. We report a case of a 14-year-old female presenting acutely with New Onset Status Epilepticus (NORSE) and clinical hyperthyroidism.

Case Presentation

A 14-year-old female presented to an outside hospital with refractory convulsive status epilepticus resistant to initial therapy with intravenous lorazepam and phenytoin. She was subsequently intubated, loaded with levetiracetam and started on propofol infusion. Past medical history includes asymptomatic untreated hyperthyroidism and a goiter that was diagnosed approximately a year prior to initial presentation. She has a strong family history of autoimmune disease with multiple siblings diagnosed with hypothyroidism and type I diabetes mellitus.

On day 3 she was transferred to our hospital’s Neurologic Critical Care Unit for further evaluation and management. Upon arrival, continuous EEG revealed non-convulsive status epilepticus (Figure 1). The anti-epileptic treatment was hence optimized by increasing levetiracetam dose, adding lacosamide with a loading dose, and starting a midazolam infusion titrated up to seizures cessation, which was achieved on day 5.

Citation: Aljaberi N, Elkambergy H, Achi E and Dibu JR. Hashimoto Encephalopathy Presenting with New Onset Refractory Status Epilepticus and Clinical Hyperthyroidism. Austin Med Sci. 2022; 7(1): 1063.