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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hashimoto's encephalitis


Other Names for this Disease
  • Hashimoto's encephalopathy
  • Steroid-responsive encephalopathy associated with autoimmune thyroiditis
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What causes Hashimoto's encephalitis?

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What causes Hashimoto's encephalitis?

Although the exact cause of Hashimoto's encephalitis (HE) is unknown, it is thought to be autoimmune due to it association with other autoimmune disorders, predominance in the female population, inflammatory findings and typical response to steroid treatment.[1] One proposed mechanism of pathogenesis suggests that HE is an autoimmune cerebral vasculitis, perhaps related to immune complex deposition. This hypothesis is supported by the focal and/or global neurological symptoms and findings on EEG, MRI and SPECT seen in HE.[1] However, while pathological findings can suggest an inflammatory process, features of a severe vasculitis are often absent.[2][1] A more recent review suggests that HE may be a recurrent form of acute disseminated encephalomyelitis (ADEM) with a presumed T-cell mediated lymphocytic vasculopathy accompanied by blood-brain barrier breakdown. Vasculopathy - damage to or irregularity of the blood vessels -  rather than vasculitis (inflammation of the blood vessels) appears to better describe the mechanism at work in HE. This is supported by the reversible rather than destructive nature of the disease, the low mortality rate, and the frequent near complete recovery of patients.[1] Research continues in an effort to better understand the exact cause.
Last updated: 4/16/2010

References