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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1999 Feb;66(2):172–176. doi: 10.1136/jnnp.66.2.172

Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease

M Seipelt 1, I Zerr 1, R Nau 1, B Mollenhauer 1, S Kropp 1, B Steinhoff 1, C Wilhelm-Gossling 1, C Bamberg 1, R Janzen 1, P Berlit 1, F Manz 1, K Felgenhauer 1, S Poser 1
PMCID: PMC1736201  PMID: 10071095

Abstract

OBJECTIVES—During an epidemiological study of Creutzfeldt-Jakob disease in Germany, Hashimoto's encephalitis was encountered as a differential diagnosis, which has not yet been described in this context.
METHODS—The symptoms and findings of seven patients who fulfilled the criteria for "possible"Creutzfeldt-Jakob disease are presented.
RESULTS—A Hashimoto's thyroiditis with antibodies against thyroglobulin or thyroid peroxidase, or both and a hypoechoic thyroid ultrasonogram were found in all cases. Analysis of CSF disclosed an increased leucocyte count in three patients, and a raised CSF:serum concentration ratio of albumin (QAlb) in four patients. The 14-3-3 protein, typical of Creutzfeldt-Jakob disease, could not be detected in any of our patients. No periodic sharp wave complexes, which are typical of Creutzfeldt-Jakob disease, were detected on EEG in any of the cases. By contrast with Creutzfeldt-Jakob disease, which leads to death within a few months, the patients with Hashimoto's encephalitis often recover quickly when treated adequately. All the patients improved after administration of corticosteroids.
CONCLUSION—The clinical symptomatology of both diseases may be very similar: dementia, myoclonus, ataxia, and personality change or psychotic phenomena are characteristic symptoms.



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Selected References

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  1. Bohnen N. I., Parnell K. J., Harper C. M. Reversible MRI findings in a patient with Hashimoto's encephalopathy. Neurology. 1997 Jul;49(1):246–247. doi: 10.1212/wnl.49.1.246. [DOI] [PubMed] [Google Scholar]
  2. Bostantjopoulou S., Zafiriou D., Katsarou Z., Kazis A. Hashimoto's encephalopathy: clinical and laboratory findings. Funct Neurol. 1996 Sep-Oct;11(5):247–251. [PubMed] [Google Scholar]
  3. Brain L., Jellinek E. H., Ball K. Hashimoto's disease and encephalopathy. Lancet. 1966 Sep 3;2(7462):512–514. doi: 10.1016/s0140-6736(66)92876-5. [DOI] [PubMed] [Google Scholar]
  4. Brown M., Vale W. Central nervous system effects of hypothalamic peptides. Endocrinology. 1975 May;96(5):1333–1336. doi: 10.1210/endo-96-5-1333. [DOI] [PubMed] [Google Scholar]
  5. Brown P., Gibbs C. J., Jr, Rodgers-Johnson P., Asher D. M., Sulima M. P., Bacote A., Goldfarb L. G., Gajdusek D. C. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol. 1994 May;35(5):513–529. doi: 10.1002/ana.410350504. [DOI] [PubMed] [Google Scholar]
  6. Flynn S. D., Nishiyama R. H., Bigos S. T. Autoimmune thyroid disease: immunological, pathological, and clinical aspects. Crit Rev Clin Lab Sci. 1988;26(1):43–95. doi: 10.3109/10408368809105889. [DOI] [PubMed] [Google Scholar]
  7. Forchetti C. M., Katsamakis G., Garron D. C. Autoimmune thyroiditis and a rapidly progressive dementia: global hypoperfusion on SPECT scanning suggests a possible mechanism. Neurology. 1997 Aug;49(2):623–626. doi: 10.1212/wnl.49.2.623. [DOI] [PubMed] [Google Scholar]
  8. Ghawche F., Bordet R., Destée A. Encéphalopathie d'Hashimoto: toxique ou auto-immune? Rev Neurol (Paris) 1992;148(5):371–373. [PubMed] [Google Scholar]
  9. Ghika-Schmid F., Ghika J., Regli F., Dworak N., Bogousslavsky J., Städler C., Portmann L., Despland P. A. Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition? Mov Disord. 1996 Sep;11(5):555–562. doi: 10.1002/mds.870110511. [DOI] [PubMed] [Google Scholar]
  10. Henchey R., Cibula J., Helveston W., Malone J., Gilmore R. L. Electroencephalographic findings in Hashimoto's encephalopathy. Neurology. 1995 May;45(5):977–981. doi: 10.1212/wnl.45.5.977. [DOI] [PubMed] [Google Scholar]
  11. Hsich G., Kenney K., Gibbs C. J., Lee K. H., Harrington M. G. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. N Engl J Med. 1996 Sep 26;335(13):924–930. doi: 10.1056/NEJM199609263351303. [DOI] [PubMed] [Google Scholar]
  12. Ishii K., Hayashi A., Tamaoka A., Usuki S., Mizusawa H., Shoji S. Case report: thyrotropin-releasing hormone-induced myoclonus and tremor in a patient with Hashimoto's encephalopathy. Am J Med Sci. 1995 Nov;310(5):202–205. doi: 10.1097/00000441-199511000-00005. [DOI] [PubMed] [Google Scholar]
  13. Jellinek E. H., Ball K. Letter: Hashimoto's disease, encephalopathy, and splenic atrophy. Lancet. 1976 Jun 5;1(7971):1248–1248. doi: 10.1016/s0140-6736(76)92207-8. [DOI] [PubMed] [Google Scholar]
  14. Jordan M. W. Hashimoto's thyroiditis. J Am Osteopath Assoc. 1967 Jul;66(11):1262–1269. [PubMed] [Google Scholar]
  15. Kothbauer-Margreiter I., Sturzenegger M., Komor J., Baumgartner R., Hess C. W. Encephalopathy associated with Hashimoto thyroiditis: diagnosis and treatment. J Neurol. 1996 Aug;243(8):585–593. doi: 10.1007/BF00900946. [DOI] [PubMed] [Google Scholar]
  16. Latinville D., Bernardi O., Cougoule J. P., Bioulac B., Henry P., Loiseau P., Mauriac L. Thyroïdite d'Hashimoto et encéphalopathie myoclonique. Hypothèses pathogéniques. Rev Neurol (Paris) 1985;141(1):55–58. [PubMed] [Google Scholar]
  17. Maeda K., Tanimoto K. Epileptic seizures induced by thyrotropin releasing hormone. Lancet. 1981 May 9;1(8228):1058–1059. doi: 10.1016/s0140-6736(81)92226-1. [DOI] [PubMed] [Google Scholar]
  18. Mariotti S., Chiovato L., Vitti P., Marcocci C., Fenzi G. F., Del Prete G. F., Tiri A., Romagnani S., Ricci M., Pinchera A. Recent advances in the understanding of humoral and cellular mechanisms implicated in thyroid autoimmune disorders. Clin Immunol Immunopathol. 1989 Jan;50(1 Pt 2):S73–S84. doi: 10.1016/0090-1229(89)90114-1. [DOI] [PubMed] [Google Scholar]
  19. Mariotti S., Ruf J., Caturegli P., Rossi V., Boniolo A., Piccolo P., Carayon P., Pinchera A. Methodological approach and diagnostic usefulness of a new assay for anti-thyroid peroxidase autoantibodies. Ann Biol Clin (Paris) 1989;47(9):541–545. [PubMed] [Google Scholar]
  20. Masters C. L., Harris J. O., Gajdusek D. C., Gibbs C. J., Jr, Bernoulli C., Asher D. M. Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol. 1979 Feb;5(2):177–188. doi: 10.1002/ana.410050212. [DOI] [PubMed] [Google Scholar]
  21. Otto M., Stein H., Szudra A., Zerr I., Bodemer M., Gefeller O., Poser S., Kretzschmar H. A., Mäder M., Weber T. S-100 protein concentration in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease. J Neurol. 1997 Sep;244(9):566–570. doi: 10.1007/s004150050145. [DOI] [PubMed] [Google Scholar]
  22. Otto M., Wiltfang J., Tumani H., Zerr I., Lantsch M., Kornhuber J., Weber T., Kretzschmar H. A., Poser S. Elevated levels of tau-protein in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease. Neurosci Lett. 1997 Apr 11;225(3):210–212. doi: 10.1016/s0304-3940(97)00215-2. [DOI] [PubMed] [Google Scholar]
  23. Poser S., Zerr I., Schulz-Schaeffer W. J., Kretzschmar H. A., Felgenhauer K. Die Creutzfeldt-Jakob-Krankheit. Eine Sphinx der heutingen Neurobiologie. Dtsch Med Wochenschr. 1997 Sep 12;122(37):1099–1105. doi: 10.1055/s-2008-1047733. [DOI] [PubMed] [Google Scholar]
  24. Shaw P. J., Walls T. J., Newman P. K., Cleland P. G., Cartlidge N. E. Hashimoto's encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers--report of 5 cases. Neurology. 1991 Feb;41(2 ):228–233. doi: 10.1212/wnl.41.2_part_1.228. [DOI] [PubMed] [Google Scholar]
  25. Shein M., Apter A., Dickerman Z., Tyano S., Gadoth N. Encephalopathy in compensated Hashimoto thyroiditis: a clinical expression of autoimmune cerebral vasculitis. Brain Dev. 1986;8(1):60–64. doi: 10.1016/s0387-7604(86)80121-8. [DOI] [PubMed] [Google Scholar]
  26. Shibata N., Yamamoto Y., Sunami N., Suga M., Yamashita Y. [Isolated angiitis of the CNS associated with Hashimoto's disease]. Rinsho Shinkeigaku. 1992 Feb;32(2):191–198. [PubMed] [Google Scholar]
  27. Steinhoff B. J., Räcker S., Herrendorf G., Poser S., Grosche S., Zerr I., Kretzschmar H., Weber T. Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Arch Neurol. 1996 Feb;53(2):162–166. doi: 10.1001/archneur.1996.00550020074017. [DOI] [PubMed] [Google Scholar]
  28. Takahashi S., Mitamura R., Itoh Y., Suzuki N., Okuno A. Hashimoto encephalopathy: etiologic considerations. Pediatr Neurol. 1994 Nov;11(4):328–331. doi: 10.1016/0887-8994(94)90011-6. [DOI] [PubMed] [Google Scholar]
  29. Thrush D. C., Boddie H. G. Episodic encephalopathy associated with thyroid disorders. J Neurol Neurosurg Psychiatry. 1974 Jun;37(6):696–700. doi: 10.1136/jnnp.37.6.696. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Will R. G. The spongiform encephalopathies. J Neurol Neurosurg Psychiatry. 1991 Sep;54(9):761–763. doi: 10.1136/jnnp.54.9.761. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Zerr I., Bodemer M., Gefeller O., Otto M., Poser S., Wiltfang J., Windl O., Kretzschmar H. A., Weber T. Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease. Ann Neurol. 1998 Jan;43(1):32–40. doi: 10.1002/ana.410430109. [DOI] [PubMed] [Google Scholar]
  32. Zerr I., Bodemer M., Räcker S., Grosche S., Poser S., Kretzschmar H. A., Weber T. Cerebrospinal fluid concentration of neuron-specific enolase in diagnosis of Creutzfeldt-Jakob disease. Lancet. 1995 Jun 24;345(8965):1609–1610. doi: 10.1016/s0140-6736(95)90118-3. [DOI] [PubMed] [Google Scholar]

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