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Hashimoto’s Encephalitis

Hashimoto’s Encephalitis. Jamie Parrott, MD Carolinas Medical Center Dept. of Neurology. Hashimoto’s Thyroiditis. Chronic autoimmune thyroiditis seen in 8% of females, 3% of males, 10% of females over 55y Hashimoto’s (chronic lymphocytic) thyroiditis is the most common form

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Hashimoto’s Encephalitis

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  1. Hashimoto’s Encephalitis Jamie Parrott, MD Carolinas Medical Center Dept. of Neurology

  2. Hashimoto’s Thyroiditis • Chronic autoimmune thyroiditis seen in 8% of females, 3% of males, 10% of females over 55y • Hashimoto’s (chronic lymphocytic) thyroiditis is the most common form • Most common cause of sporatic goiter in children (1.2% prevalence)

  3. Hashimoto’s Thyroiditis • Exponential increase in last 40 years • T-cell mediated • Usually asymptomatic • Present with hypothyroidism (20%), goiter, or both • 5% incidence of hypothyroidism per year following diagnosis • 95% Female, usually between ages 30-50y

  4. Hashimoto’s Thyroiditis • Thyroid antibodies targeted • Thyroglobulin • Thyroid microsomal antigen (thyroid peroxidase) • TSH receptor • Association with type I DM, multiple sclerosis, rheumatoid arthritis, Turner syndrome, celiac disease, vitaligo

  5. Hashimoto’s Encephalitis • Rare condition associated with HT • 2 to 3 per 100,000 prevalence • Triad of encephalopathy, high serum antithyroid antibodies, and responsiveness to steroids • Majority euthyroid • 82% female • All pediatric case reports female, 9-18y

  6. Hashimoto’s Encephalitis • Two distinct patterns • Acute type characterized by seizures, stroke-like episodes with transient neurologic deficits, altered mental status. Recurrent. • Subacute with insidious onset over weeks. Confusion, agitation, restless, hallucinations, dementia in the absence of focal neurologic deficits • No relationship with presence of hypothyroidism and severity of symptoms

  7. Hashimoto’s Encephalitis • Possible underlying mechanisms • CNS autoimmune vasculitis (lymphocytic perivascular infiltrate seen pathologically in 2 of 9 patients, Odie et al) • Formation of antineural antibodies (Archambeaud et al; alpha enolase ab, Ochi et al; antithyroid antibody titers in CSF not related to severity of disease)

  8. Hashimoto’s Encephalitis • Severity of symptoms unrelated to antibody titers, thyroid function • Reported symptoms: seizures, myoclonus, Stiff-limb syndrome, stroke-like symptoms, cerebellar dysfunction, psychosis, confusion, depression, headache, hypothermia, coma

  9. Hashimoto’s Encephalitis • CSF protein elevated 75% • CSF pleocytosis 25% • EEG changes nonspecific (slowing) • MRI typically normal (occasional T2-weighted abnormalities)

  10. Hashimoto’s Encephalitis • Rapid response to steroids • Full recovery 3-4 weeks • Acute form is frequently recurrent, consider prophylaxis

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