Clinical and diagnostic characteristics of Hashimoto's encephalopathy: a single-center, retrospective study.

Background and purpose: Diagnosing Hashimoto's encephalopathy (HE) is challenging. In contrast to other types of autoimmune encephalitis, HE shows an excellent response to steroid treatment. We aimed to investigate the rates of antithyroid antibodies (ATAs) and probable HE in patients with unexplained mental dysfunction and compare the clinical characteristics between the good- and poor-outcome groups.

Methods: We retrospectively reviewed the medical records and electroencephalography (EEG) and neuroimaging findings of patients admitted to the Department of Neurology of our hospital from March 1, 2006, to February 28, 2023. Using our proposed diagnostic criteria for probable HE, we compared the clinical characteristics between the good- and poor-outcome groups. We also investigated the rates of ATA positivity and probable HE.

Results: In total, 198 patients exhibited altered mentation, rapidly progressive cognitive decline, or myoclonus. ATA tests were performed on 86 patients, and the detection rates of ATAs and probable HE were 29.1% and 25.6%, respectively. Of the 22 patients enrolled, the good- and poor-outcome groups comprised 19 and 3 patients, respectively. Clinical seizures occurred in seven patients. Nonconvulsive status epilepticus on EEG was observed in six patients, all of whom were intractable to antiepileptic drugs. Nineteen of 21 patients (90.5%) treated with immunosuppressants showed good outcomes.

Conclusions: HE is a rare clinical disorder, but not as rare as previously thought. When HE is suspected, steroids should be considered the first-line treatment. Early diagnosis and adequate treatment are critical to achieve good outcomes in HE.