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
 Most common cause of sporatic goiter in
children (1.2% prevalence)
 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 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 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 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 Encephalitis
 Possible underlying mechanisms
 CNS autoimmune vasculitis ( Odie et al)
 Formation of antineural antibodies (Archambeaud et al;
alpha enolase ab, Ochi et al)
 Severity of symptoms notrelated to thyroid function
 Reported symptoms: seizures, myoclonus, Stiff-limb
syndrome, stroke-like symptoms, cerebellar
dysfunction, psychosis, confusion, depression,
headache, hypothermia, coma
 Hashimoto’s Encephalitis
 Severity of symptoms notrelated to thyroid
function
 Reported symptoms: seizures, myoclonus,
Stiff-limb syndrome, stroke-like symptoms,
cerebellar dysfunction, psychosis, confusion,
depression, headache, hypothermia, coma
 Hashimoto’s Encephalitis
 CSF protein elevated 75%
 CSF pleocytosis 25%
 EEG changes nonspecific (slowing)
 MRI typically normal (occasional T2-weighted
abnormalities)
 Hashimoto’s Encephalitis
 Rapid response to steroids
 Full recovery 3-4 weeks
 Acute form is frequently recurrent, consider
prophylaxis