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Hashimoto Encephalopathy (Rare Steroid-Responsive Disorder)

Steroid-responsive encephalopathy associated with autoimmune thyroiditis

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Hashimoto Encephalopathy (Rare Steroid-Responsive Disorder)?

Hashimoto encephalopathy is a rare autoimmune encephalopathy associated with high titers of antithyroid antibodies (anti-TPO, anti-thyroglobulin).

The pathogenesis is unclear; the antibodies are markers rather than direct mediators, and immune-mediated cerebral vasculitis or autoimmune encephalitis is suspected.

It typically affects women more than men in middle age, with prevalence estimated at 2 per 100,000.

Most patients are clinically euthyroid or have subclinical hypothyroidism — thyroid dysfunction does not explain the neurologic symptoms.

Diagnosis requires exclusion of other encephalopathies (infectious, metabolic, structural, paraneoplastic, autoimmune encephalitis) and demonstration of dramatic steroid response.

Symptoms

Acute or subacute confusion, disorientation and altered consciousness
Cognitive decline, memory impairment and executive dysfunction
Seizures (focal or generalized) sometimes presenting as status epilepticus
Myoclonus, tremor or other movement disorders
Psychiatric symptoms: psychosis, hallucinations, depression, personality changes
Stroke-like episodes with focal deficits that wax and wane
Headache, ataxia and rarely coma
Relapsing-remitting course is characteristic

Risk Factors

Female sex (5–7 times more common than men)
Middle-aged adults (peak incidence age 40–50)
Personal or family history of autoimmune thyroid disease (Hashimoto thyroiditis)
Other concurrent autoimmune conditions (vitiligo, type 1 diabetes, pernicious anemia)
Genetic predisposition with HLA associations

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Unexplained acute or subacute confusion or cognitive decline
  • New-onset seizures, especially with abnormal mental status
  • Stroke-like episodes without imaging abnormalities
  • Psychiatric symptoms with cognitive impairment in a patient with thyroid antibodies
  • Myoclonus or tremor with encephalopathy
  • Status epilepticus without identified cause
  • Personality or behavioral changes with relapsing course

Treatment Methods

01
Confirm diagnosis: exclude alternative causes (CSF analysis, neuroimaging, autoimmune encephalitis panel, paraneoplastic antibodies)
02
Document elevated antithyroid antibodies (anti-TPO or anti-thyroglobulin) with normal or near-normal thyroid function
03
Consult neurology, often in a referral center experienced with autoimmune encephalopathy
04
First-line: high-dose corticosteroids (IV methylprednisolone 1 g/day for 5 days, then oral prednisone 1 mg/kg/day)
05
Most patients show dramatic clinical improvement within days to weeks
06
Taper steroids slowly over months to prevent relapse
07
Steroid-sparing immunosuppressants (azathioprine, mycophenolate, methotrexate) for relapsing or steroid-dependent disease
08
IVIG, plasma exchange or rituximab for refractory cases
09
Treat thyroid dysfunction if present (levothyroxine for hypothyroidism)
10
Long-term follow-up with neurology for relapse monitoring

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.