Hashimoto's encephalopathy: four cases and review of literature

Abstract

Hashimoto's encephalopathy is a rare clinically heterogenous condition consisting of encephalopathy, seizures and variable neurological and psychiatric manifestations, accompanied by high titres of serum antithyroid antibodies. We described the clinical and laboratory findings of four children (aged 8-17 years) with Hashimoto's encephalopathy. The clinical features of three patients at presentation included refractory epilepsy, and confusion, and one patient presented with behavioral and cognitive changes. During their presentation, two of them were in euthyroid, and the others were in hypothyroid status. All patients manifested increased antithyroid antibodies. Two patients improved with steroid treatment. The others responded to plasmapheresis instead of corticosteroid treatment. Physicians' awareness of this complication is of great importance because most patients respond dramatically to the treatment.

The use of plasma exchange in Hashimoto's encephalopathy: A case report and review of the literature

“…While steroids remain the first line treatment for this disease process , some case reports have documented clinical improvement with plasma exchange . The current case report documents an unsuccessful trial of plasma exchange therapy in a patient with HE.…”

“…Second, azathioprine or cyclophosphamide after pulse steroid therapy has also been successfully reported . Lastly, plasma exchange, while not common, has been reported in nine published cases to date (six adult and three pediatric) . As shown in Table , all reported cases demonstrate symptomatic improvement after plasma exchange, and three to nine plasma exchange procedures were used prior to the documented improvement.…”

Application of Plasma Exchange in Steroid-Responsive Encephalopathy

“…Afterward, the patient experienced two relapses, and the symptoms were relieved after plasma exchange. Multiple studies have subsequently supported this treatment (5, 6, 40, 43–45). Nieuwenhuis et al (45) reported a 48-year-old patient with subacute Hashimoto's encephalopathy, and the main manifestations were rapid progressive dementia, visual hallucinations, and myoclonus.…”

“…The most common clinical manifestations are seizures, followed by psychiatric symptoms (39). Elevated levels of anti-thyroid peroxidase antibodies (anti-TPOAb) and/or anti-thyroglobulin antibodies (anti-TgAb) are important laboratory characteristics for the diagnosis of Hashimoto's encephalopathy; elevated anti-thyroid peroxidase antibody levels are most common and are observed in 86% of patients with Hashimoto's encephalopathy, while 48% of the patients with Hashimoto's encephalopathy have elevated anti-thyroglobulin antibody levels (6). Although the pathophysiological mechanism of Hashimoto's encephalopathy is still not clear, high concentrations of anti-thyroid antibodies and effective treatment with immunosuppressive agents both support the important role of autoimmune mechanisms in Hashimoto's encephalopathy (40), which is the theoretical basis of plasma exchange in the treatment of Hashimoto's encephalopathy.…”

“…Nieuwenhuis et al (45) used three sessions plasma exchange to treat Hashimoto's encephalopathy successfully. Bektas et al (43) used nine sessions plasma exchange, while Nagpal and Pande (5) and Gul Mert et al (6) reports that the number of plasma exchange sessions for the treatment of Hashimoto's encephalopathy should be five. These results are consistent with the American Society for Apheresis, which recommends a total of 3–9 sessions plasma exchange for Hashimoto's encephalopathy, with the most common number of plasma exchange being five and exchanges being performed once every other day.…”