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February 2006

What's in a NAIM?Hashimoto Encephalopathy, Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis, or Nonvasculitic Autoimmune Meningoencephalitis?

Arch Neurol. 2006;63(2):175-176. doi:10.1001/archneur.63.2.175

Hashimoto encephalopathy was first described by Lord Brain in 1966.1 Multiple case reports followed and the term Hashimoto encephalopathy became generally accepted for the syndrome of encephalopathy in association with Hashimoto thyroiditis. Although Brain's original patient with Hashimoto encephalopathy did not improve with corticosteroid treatment, most of the patients described later showed marked improvement with some form of immunosuppression. This has led to the renaming of Hashimoto encephalopathy by some authors as “steroid-responsive encephalopathy associated with autoimmune thyroiditis” (SREAT)2 or “nonvasculitic autoimmune meningoencephalitis” (NAIM), a heterogeneous group that would include autoimmune disorders such as Sjögren syndrome and systemic lupus erythematosus–associated meningoencephalitis as well as Hashimoto encephalopathy. These disorders are unified by the feature of steroid responsiveness.3