Hashimoto's encephalopathy: A rare proteiform disorder

Area of Science:

• Neurology
• Endocrinology
• Immunology

Background:

• Hashimoto's encephalopathy (HE) is a rare, poorly understood neurological disorder.
• It is characterized by seizures, cognitive decline, and psychiatric symptoms, often linked to thyroid disease and anti-thyroperoxidase antibodies (TPOAb).
• Diagnosis is challenging due to non-specific symptoms and findings.

Purpose of the Study:

• To present a case of HE in a 16-year-old boy with hypothyroidism.
• To review the literature on Hashimoto's encephalopathy.
• To emphasize the effectiveness of steroid therapy in HE.

Main Methods:

• Case report of a 16-year-old male with HE and hypothyroidism.
• Systematic literature review of Hashimoto's encephalopathy cases.
• Analysis of clinical presentation, diagnostic challenges, and treatment response.

Main Results:

• The presented case demonstrated a significant response to high-dose steroid treatment.
• Literature review indicates HE affects all age groups, with a predilection for females.
• Elevated TPOAb levels are common, but the exact pathophysiology linking them to encephalopathy remains unclear.

Conclusions:

• Hashimoto's encephalopathy, though rare, should be considered in patients with neurological symptoms and thyroid autoimmunity.
• Early diagnosis and prompt treatment with corticosteroids can lead to favorable outcomes.
• Further research is needed to elucidate the pathophysiology of HE.