Hashimoto s encephalitis
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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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Hashimoto s encephalitis

Hashimoto’s Encephalitis

Jamie Parrott, MD

Carolinas Medical Center

Dept. of Neurology


Hashimoto s thyroiditis

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)


Hashimoto s thyroiditis1

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


Hashimoto s thyroiditis2

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


Hashimoto s encephalitis1

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


Hashimoto s encephalitis2

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


Hashimoto s encephalitis3

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)


Hashimoto s encephalitis4

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


Hashimoto s encephalitis5

Hashimoto’s Encephalitis

  • CSF protein elevated 75%

  • CSF pleocytosis 25%

  • EEG changes nonspecific (slowing)

  • MRI typically normal (occasional T2-weighted abnormalities)


Hashimoto s encephalitis6

Hashimoto’s Encephalitis

  • Rapid response to steroids

  • Full recovery 3-4 weeks

  • Acute form is frequently recurrent, consider prophylaxis


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