August 2019 By PsychDB.com

Hashimoto's Encephalitis

Hashimoto's Encephalopathy (HE) (also known as Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis [SREAT]), is a rare, clinically heterogeneous neurological disorder associated with Hashimoto's disease and positive thyroid autoantibodies. It is increasingly recognized as an important and treatable cause of autoimmune encephalitis, though remains somewhat controversial as a diagnosis.

Epidemiology

HE predominantly affects women in a wide age range, from 10 to 80 years of age.

Comorbidity

Clinical or subclinical thyroid disease, usually hypothyroidism, occurs in over half of the cases.

Diagnosis can be made when all 6 of the following criteria have been met (Graus et al. 2016):[1]

  1. Encephalopathy with seizures, myoclonus, hallucinations, or stroke-like episodes
  2. Subclinical or mild overt thyroid disease (usually hypothyroidism)
  3. Brain MRI normal or with non-specific abnormalities
  4. Presence of serum thyroid antibodies (thyroid peroxidase, thyroglobulin)
  5. Absence of well characterized neuronal antibodies in serum and CSF
  6. Reasonable exclusion of alternative causes (see Differential Diagnosis)

Symptoms

The clinical presentation of HE can include seizures, myoclonus, hallucinations, and stroke-like episodes with normal or non-specific CSF and brain MRI abnormalities.

Controversial Diagnosis

Hashimoto’s encephalopathy should be diagnosed only when a thorough clinical assessment has been performed, including comprehensive testing for well characterized neuronal antibodies, and ruling out other potential causes of encephalopathy or encephalitis. In essence, it should be a diagnosis of exclusion.

anti-TPO, anti-thyroglobulin, and anti-dsDNA can be elevated in HE.

Thyroid Antibodies Are Non-Specific!

Thyroid antibodies are not specific for Hashimoto’s encephalopathy. They can be present in up to 13% of healthy individuals, and up to 27% in white women older than 60 years. It can also be present in other autoimmune encephalitis disorders.

Patients with a non-specific encephalopathy with subclinical or overt thyroid disease, anti-thyroid antibodies, and no other explanation for the symptoms should be considered for a trial of steroids. Most patients treated with corticosteroids with or without levothyroxine will have clinical improvement.[2]