Journal of Neurology

, Volume 243, Issue 8, pp 585–593

Encephalopathy associated with Hashimoto thyroiditis: Diagnosis and treatment

  • I. Kothbauer-Margreiter
  • M. Sturzenegger
  • J. Komor
  • R. Baumgartner
  • C. W. Hess
Original Communication

Abstract

Six patients with Hashimoto thyroiditis (HT) and associated encephalopathy (HE) are described and compared with 14 well-documented cases retrieved from the literature. HE typically affects patients when they are euthyroid and, in an appropriate clinical situation, antithyroid autoantibodies are the main indicators of HE. Since clinical features of HE are unspecific, other aetiologies such as infectious, metabolic, toxic, vascular, neoplastic, and paraneoplastic causes have to be excluded. Our own six cases and those from the literature show that two types of initial clinical presentation can be differentiated: a vasculitic type with stroke-like episodes and mild cognitive impairment in nine patients, and a diffuse progressive type with dementia, seizures, psychotic episodes or altered consciousness in 11 patients. These types may overlap, particularly in the long-term course without treatment. Response to steroids was usually excellent with complete remission in 80%. Eighteen of the 20 patients were women. Characteristic, though unspecific, findings were abnormal EEG (90%) and CSF (80%). Together with quantitative neuropsychological testing, these proved sensitive for monitoring the efficacy of therapy. Conversely, antithyroid autoantibody titres did not correlate with the severity or type of clinical presentation. The link between HE and HT is not clear. A pathogenetic role for antithyroid autoantibodies in the central nervous system seems unlikely.

Key words

Hashimoto thyroiditis Encephalopathy Antithyroid autoantibodies Autoimmune disorder 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Althaus B, Staub JJ, Müller-Brand J, Carmann H, Viollier M, Matter L, Berger W (1983) Autoimmunantikörperprofil bei idiopathischer Hypothyreose und bei Hypothyreose nach Radiojodbehandlung wegen Morbus Basedow: Vergleich mit einem Normkollektiv aus der weiblichen Bevölkerung der Schweiz. Schweiz Med Wochenschr 113: 1319–1327Google Scholar
  2. 2.
    Becker KL, Ferguson RH, McConahey WM (1963) The connective-tissue diseases and symptoms associated with Hashimoto's thyroiditis. N Engl J Med 268:277–280Google Scholar
  3. 3.
    Bogner U, Kotulla P, Peters H, Schleusener H (1990) Thyroid peroxidase/microsomal antibodies are not identical with thyroid cytotoxic antibodies in autoimmune thyroiditis. Acta Endocrinol 123: 431–437Google Scholar
  4. 4.
    Brain L, Jellinek EH, Ball K (1966) Hashimoto's disease and encephalopathy. Lancet II: 512–514Google Scholar
  5. 5.
    Claussmann C, Offner C, Chevalier Y, Sellal F, Collard M (1994) Encephalopathie et thyroidite de Hashimoto. Rev Neurol (Paris) 150: 166–168Google Scholar
  6. 6.
    Collins JA, Zimmer FE, Johnsen NS, Kough RH (1964) The many faces of hypothyroidism. Postgrad Med 371–384Google Scholar
  7. 7.
    Ewins DL, Rossor MN, Butler J, Roques PK, Mullan MJ, McGregor AM (1991) Association between autoimmune thyroid disease and familial Alzheimer's disease. Clin Endocrinol (Oxf) 35: 93–96Google Scholar
  8. 8.
    Flynn SD, Nishiyama RH, Bigos ST (1988) Autoimmune thyroid disease: immunological, pathological and clinical aspects. Crit Rev Clin Lab Sci 26: 43–94Google Scholar
  9. 9.
    Ghawche F, Bordet R, Destée A (1992) Encephalopathie d'Hashimoto: toxique ou auto-immune? Rev Neurol (Paris) 148: 371–373Google Scholar
  10. 10.
    Ghika-Schmid F, Ghika J, Bogousslavsky J, Dworak N, Staedler C, Portmann L, Regli F, Despland PA (1995) Encephalopathie myoclonique de Hashimoto. Schweiz Arch Neurol Psychiatr 146: 75Google Scholar
  11. 11.
    Hall RCW, Popkin MK, Vaul RD, Hall AK, Gardner ER, Beresford TP (1982) Psychiatric manifestations of Hashimoto's thyroiditis. Psychosomatics 23: 337–342Google Scholar
  12. 12.
    Henderson LM, Behan PO, Aarli J, Hadley D, Draper IT (1987) Hashimoto's encephalopathy: a new neuroimmunological syndrome. Ann Neurol 22: 140–141Google Scholar
  13. 13.
    Ishii K, Hayashi A, Tamaoka A, Mizusawa H, Shoji S (1993) A case of Hashimoto's encephalopathy with a relapsing course related to menstrual cycle. Rinsho Shinkeigaku 33: 995–997Google Scholar
  14. 14.
    Jellinek EH (1962) Fits, faints, coma, and dementia in myxoedema. Lancet II: 1010–1012Google Scholar
  15. 15.
    Jellinek EH, Ball K (1976) Hashimoto's disease, encephalopathy and splenic atrophy. Lancet I: 1248Google Scholar
  16. 16.
    Latinville D, Bernardi O, Cougoule JP, Bioulac B, Henry P (1982) Thyroidite d'Hashimoto et encephalopathie myoclonique, hypotheses pathogeniques. Rev Neurol (Paris) 141: 55–58Google Scholar
  17. 17.
    Mauriac L, Roger P, Kern AM, Manciet G, Riviere L, Henry P (1982) Thyroidite de Hashimoto et encephalopathie. Rev Fr Endocrinol Clin 23: 147–150Google Scholar
  18. 18.
    McLachlan SM, Rapoport B (1989) Evidence for a potential common T-cell epitope between human thyroid peroxidase and human thyroglobulin with implications for the pathogenesis of autoimmune thyroid disease. Autoimmunity 5: 101–106Google Scholar
  19. 19.
    Mulhern LM, Masi AT, Shulman LE (1966) Hashimoto's disease: a search for associated disorders in 170 clinically detected cases. Lancet I: 508–511Google Scholar
  20. 20.
    Notsu K, Oka N, Furuya H, Kato Y (1991) Incidence of abnormal thyroid function in patients with low titres of thyroid microsomal antibodies. Nippon Naibunpi Gakkai Zasshi 67: 702–709Google Scholar
  21. 21.
    Reichlin S (1993) Neuroendocrine — immune interactions. N Engl J Med 329: 1246–1253Google Scholar
  22. 22.
    Royce PC (1971) Severely impaired consciousness in myxedema — a review. Am J Med Sci 261: 46–50Google Scholar
  23. 23.
    Sanders V (1962) Neurologic manifestations of myxedema. N Engl J Med 266: 547–552Google Scholar
  24. 24.
    Shaw PJ, Walls TJ, Newman PK, Cleland PG, Cartlidge NEF (1991) Hashimoto' s encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers — a report of 5 cases. Neurology 41: 228–233Google Scholar
  25. 25.
    Shein M, Apter A, Dickerman Z, Tyano S, Gadoth N (1986) Encephalopathy in compensated Hashimoto thyroiditis: a clinical expression of autoimmune cerebral vasculitis. Brain Dev 8: 60–64Google Scholar
  26. 26.
    Shibata N, Yamamoto Y, Sunami N, Suga M, Yamashita Y (1992) Isolated angiitis of the CNS associated with Hashimoto's disease. Rinsho Shinkeigaku 32: 191–198Google Scholar
  27. 27.
    Suzuki N, Mitamura R, Ohmi H, Itoh Y (1994) Hashimoto thyroiditis, distal renal tubular acidosis, pernicious anemia, and encephalopathy: a rare combination of auto-immune disorders in a 12 year old girl. Eur J Pediatr 153: 78–79Google Scholar
  28. 28.
    Thrush DC, Boddie HG (1974) Episodic encephalopathy associated with thyroid disorders. J Neurol Neurosurg Psychiatry 37: 696–700Google Scholar

Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • I. Kothbauer-Margreiter
    • 1
  • M. Sturzenegger
    • 1
  • J. Komor
    • 1
  • R. Baumgartner
    • 1
  • C. W. Hess
    • 1
  1. 1.Neurologische KlinikBernSwitzerland

Personalised recommendations