[Hashimoto Encephalopathy].

Area of Science:

• Neurology
• Immunology
• Endocrinology

Background:

• Hashimoto encephalopathy (HE) is an autoimmune neurological disorder associated with Hashimoto's thyroiditis.
• Steroid therapy is an effective treatment for HE.
• Serum autoantibodies against the NH2-terminal of α-enolase (NAE) were identified as a specific biomarker for HE in 2005.

Purpose of the Study:

• To analyze serum anti-NAE autoantibodies and clinical features in patients with HE.
• To investigate the prevalence and clinical characteristics of HE across international cohorts.
• To understand the varied clinical presentations and diagnostic challenges of HE.

Main Methods:

• Retrospective analysis of serum samples and clinical data from HE patients.
• Detection of serum anti-NAE autoantibodies.
• Clinical assessment including neurological examination, EEG, brain SPECT, and MRI.

Main Results:

• Approximately 50% of HE patients tested positive for anti-NAE antibodies.
• HE commonly affects individuals aged 50-60 years, often in a euthyroid state but with anti-thyroid antibodies.
• Common neuropsychiatric symptoms include consciousness disturbance, psychosis, cognitive dysfunction, and ataxia; EEG and SPECT abnormalities are frequent, while MRI findings are less common.

Conclusions:

• Anti-NAE antibodies are a valuable diagnostic marker for HE, though not present in all cases.
• HE exhibits diverse clinical phenotypes, mimicking other neurological conditions like spinocerebellar degeneration and Creutzfeldt-Jakob disease.
• Recognition of HE's varied presentations and association with thyroid disorders is crucial for timely diagnosis and treatment.