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Hashimoto Encephalopathy — RACP Adult Medicine MCQ

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HardNeurologyHashimoto EncephalopathyRACP Adult Medicine

A 55-year-old woman with Hashimoto thyroiditis presents with subacute cognitive decline, tremor, myoclonus, and seizures. TSH is elevated (15 mU/L), anti-TPO titres are very high (>1000 IU/mL). MRI brain is normal. EEG shows generalised slowing. CSF is normal except mildly elevated protein. She responds dramatically to IV methylprednisolone. What is the most likely diagnosis?

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Correct answer: E — Hashimoto encephalopathy

Subacute encephalopathy with very high anti-TPO titres, normal MRI, and dramatic response to corticosteroids is steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT/Hashimoto encephalopathy). It is a diagnosis of exclusion. The pathogenesis is unclear (anti-TPO antibodies are likely a marker rather than pathogenic). Treatment is corticosteroids with excellent prognosis.

Reference: eTG Neurology – 2024; Laurent 2016 SREAT Review