Internal and Emergency Medicine

, 1:15

Clinical and diagnostic aspects of encephalopathy associated with autoimmune thyroid disease (or Hashimoto’s encephalopathy)

  • Gianluca Tamagno
  • Giovanni Federspil
  • Giovanni Murialdo
Internal Medicine Review Article Neurology

DOI: 10.1007/BF02934715

Cite this article as:
Tamagno, G., Federspil, G. & Murialdo, G. Int Emergency Med (2006) 1: 15. doi:10.1007/BF02934715

Abstract

Encephalopathy associated with autoimmune thyroid disease, currently known as Hashimoto’s encephalopathy, but also defined as corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis, is a relatively rare condition observed in a small percentage of patients presenting with autoimmune thyroid disease. It consists of a subacute, relapsing-remitting, steroid-responsive encephalopathy characterised by protean neurologic and neuropsychiatric symptoms, diffuse electroencephalographic abnormalities and increased titres of antithyroid antibodies in serum and/or in cerebrospinal fluid. Most of the cases presenting this neurologic complication are affected by Hashimoto’s thyroiditis or, less frequently, by other autoimmune thyroid diseases, chiefly Graves’ disease.

The pathogenesis of this encephalopathy is still unknown and largely debated, because of extremely varied clinical presentation, possibly referable to different aetiologic and pathophysiologic mechanisms, as confirmed by the two clinical cases we report in this paper. Autoimmune aetiology is, however, very likely in view of the well established favourable response to corticosteroid administration. Both vasculitis and autoimmunity directed against common brainthyroid antigens represent the most probable aetiologic pathways.

Clinical manifestations include consciousness changes, neurologic diffuse or focal signs, headache, and altered cognitive function. Although unspecific, cerebral oedema has also been described. Cerebrospinal fluid examination often discloses an inflammatory process, with a mild increase in protein content and occasionally in lymphocyte count. In this review, clinical criteria for the diagnosis of defined, probable, or possible encephalopathy associated with autoimmune thyroid disease are suggested. Corticosteroid therapy currently allows us to obtain rapid remission of disease symptoms, but adverse outcomes as well as spontaneous remissions have also been reported.

Key words

autoimmune thyroiditisautoimmunitycorticosteroidsHashimoto’s thyroiditis

Copyright information

© SIMI, Italian Society of Internal Medicine 2006

Authors and Affiliations

  • Gianluca Tamagno
    • 1
  • Giovanni Federspil
    • 1
  • Giovanni Murialdo
    • 2
  1. 1.Medical Clinic 3, Department of Medical and Surgical SciencesUniversity of PaduaPadua
  2. 2.Clinica di Medicina Interna 2, Dipartimento di Scienze Endocrinologiche e MetabolicheUniversità degli StudiGenovaItaly