Skip to main content

Advertisement

SpringerLink
Log in

The neurological disorder associated with thyroid autoimmunity

  • REVIEW
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

The neurological disorder associated with thyroid autoimmunity is an elusive disease that neurologists have learned to recognize in the last few years. The diagnosis is made, after excluding more common diseases, when neuropsychiatric symptoms develop in a patient with high serum concentrations of anti-thyroid antibodies. The clinical presentations of the disease and the many controversial issues surrounding the diagnosis, the pathogenesis, the role of thyroid autoantibodies, and the choice of therapy are reviewed and discussed in the light of the medical literature in English.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Arain A, Abou-Khalil B, Moses H (2001) Hashimoto’s encephalopathy: documentation of mesial temporal seizure by ictal EEG. Seizure 10:438–441

    PubMed  CAS  Google Scholar 

  2. Avila A, Serrado A, Reig L, Famades A, Maho P (2003) Early presentation of gait disturbance in a steroid-responsive encephalopathy associated with autoimmune thyroiditis. Eur J Neurol 10:601

    Article  PubMed  CAS  Google Scholar 

  3. Azuma T, Uemichi T, Funauchi M, Doi S, Matsubara T (2000) Myelopathy associated with Hashimoto’s disease. J Neurol Neurosurg Psychiatry 68:681–682

    Article  PubMed  CAS  Google Scholar 

  4. Balestri P, Grosso S, Garibaldi G (1999) Alternating hemiplegia of childhood or Hashimoto’s encephalopathy?. J Neurol Neurosurg Psychiatry 66:548–549

    PubMed  CAS  Google Scholar 

  5. Barker R, Zajicek J, Wilkinson I (1996) Thyrotoxic Hashimoto’s encephalopathy. J Neurol Neurosurg Psychiatry 60:234

    PubMed  CAS  Google Scholar 

  6. Berio A, Piazzi A (2002) A case of Kearns-Sayre syndrome with autoimmune thyroiditis and possible Hashimoto encephalopathy. Panminerva Med 44:265–269

    PubMed  CAS  Google Scholar 

  7. Bertoni M, Falcini M, Sestini S, et al. (2003) Encephalopathy associated with Hashimoto’s thyroiditis: an additional case. Eur J Intern Med 14:434–437

    Article  PubMed  Google Scholar 

  8. Boers PM, Colebatch JG (2001) Hashimoto’s encephalopathy responding to plasmapheresis. J Neurol Neurosurg Psichiatry 70:132

    Article  CAS  Google Scholar 

  9. Bohnen NILJ, Parnell KJ, Harper CM (1997) Reversible MRI findings in a patient with Hashimoto’s encephalopathy. Neurology 49:246–247

    PubMed  CAS  Google Scholar 

  10. Bostantjopoulou S, Zafiriou D, Katsarou Z, Kazis A (1996) Hashimoto’s encephalopathy: clinical and laboratory findings. Funct Neurol 11:247–251

    PubMed  CAS  Google Scholar 

  11. Brain, Lord, Jellinek EH, Ball K (1966) Hashimoto’s disease and encephalopathy. Lancet 2:512–514

    Article  PubMed  CAS  Google Scholar 

  12. Byrne OC, Zuberi SM, Madigan CA, King MD (2000). Hashimoto’s thyroiditis-a rare but treatable cause of encephalopathy in children. Eur J Paediatr Neurol 4:279–282

    Article  PubMed  CAS  Google Scholar 

  13. Cantón A, de Fàbregas O, Tintoré M, et al. (2000) Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition?. J Neurol Sci. 176:65–69

    Article  PubMed  Google Scholar 

  14. Cao NJ, Tselis AC, Li J, Gorman M (2005) A case of Hashimoto’s encephalopathy: association with sensory ganglionopathy. J Neurol Sci 238:105–107

    Article  PubMed  Google Scholar 

  15. Castillo PR, Mignot E, Woodruff BK, Boeve BF (2004) Undetectable CSF hypocretin-1 in “Hashimoto’s encephalopathy” associated with coma. Neurology 62:1909

    PubMed  Google Scholar 

  16. Chaudhuri A, Behan PO (2003) The clinical spectrum, diagnosis, pathogenesis and treatment of Hashimoto’s encephalopathy (recurrent acute disseminated encephalomyelitis). Curr Med Chem 10:1945–1953

    Article  PubMed  CAS  Google Scholar 

  17. Chen HC, Masharani U (2000) Hashimoto’s encephalopathy. South Med J 93:504–506

    PubMed  CAS  Google Scholar 

  18. Chen PL, Wang PY, Hsu HY (2005). Reversible electroencephalographic and single emission computed tomography abnormalities in Hashimoto’s encephalopathy. J Chin Med Assoc 68:77–81

    Article  PubMed  Google Scholar 

  19. Chong JY, Rowland LP, Utiger RD (2003) Hashimoto encephalopathy: syndrome or myth?. Arch Neurol 60:164–171

    Article  PubMed  Google Scholar 

  20. Cossu G, Melis M, Molari A, et al. (2003) Creutzfeld-Jakob disease associated with high titer of antithyroid antibodies. Case report and literature review. Neurol Sci 24:138–140

    Article  PubMed  CAS  Google Scholar 

  21. Creutzfeldt CJ, Haberl Rl (2005) Hashimoto encephalopathy: a do-not-miss in the differential diagnosis of dementia. J Neurol 1–3

    Google Scholar 

  22. De Vries PJ, Hunt N, Crawford A, Hodges JR, Balan K (2000) Hashimoto’s encephalopathy presenting as “myxoedematous madness”. J Neurol Neurosurg Psychiatry 68:100–126

    Article  Google Scholar 

  23. Desai J, Wadia N (1999) Hashimoto’s encephalopathy. J Neurol Sci 163: 202–203

    Article  PubMed  CAS  Google Scholar 

  24. Deutsch M, Koskinas J, Tzannos K, et al (2005) Hashimoto encephalopathy with pegylated interferon alfa-2b and ribavirin. Ann Pharmacother 39:1745–1748

    Article  PubMed  Google Scholar 

  25. Doherty CP, Schlossmacher M, Torres N, et al. (2002) Hashimoto’s encephalopathy mimicking Creutzfeld-Jakob disease: brain biopsy findings. J Neurol Neurosurg Psychiatry 73: 601–602

    Article  PubMed  CAS  Google Scholar 

  26. Duffey P, Yee S, Reid IN, Bridges LR (2003) Hashimoto’s encephalopathy. Postmortem findings after fatal status epilepticus. Neurology 61:1124–1126

    PubMed  Google Scholar 

  27. Erickson JC, Carrasco H, Grimes JB, Jabbari B, Cannard KR (2002) Palatal tremor and myorhythmia in Hashimoto’s encephalopathy. Neurology 58:504–505

    PubMed  CAS  Google Scholar 

  28. Fatemi S, Bedri J, Nicoloff JT (2003) Encephalopathy associated with Hashimoto’s thyroiditis: use of serum immunoglobulin G as a marker of disease activity. Thyroid 13:227–228

    Article  PubMed  Google Scholar 

  29. Ferracci F, Bertiato G, Moretto G (2004) Hashimoto’s encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci 217:165–168

    Article  PubMed  Google Scholar 

  30. Ferracci F, Moretto G, Candeago RM, et al. (2003) Anti-thyroid antibodies in the CSF: their role in the pathogenesis of Hashimoto’s encephalopathy. Neurology 60:712–714

    PubMed  CAS  Google Scholar 

  31. Forchetti C, Katsamakis G, Garron DC (1997). Autoimmune thyroiditis and a rapidly progressive dementia: global hypoperfusion on SPECT scanning suggests a possible mechanism. Neurology 49:623–626

    PubMed  CAS  Google Scholar 

  32. Fujii A, Yoneda M, Ito T, et al. (2005) Autoantibodies against the aminoterminal of alpha-enolase are a useful diagnostic marker of Hashimoto’s encephalopathy. J Neuroimmunol 162:130–136

    Article  PubMed  CAS  Google Scholar 

  33. Galluzzi S, Geroldi C, Zanetti O, Frisoni GB (2002) Hashimoto’s encephalopathy in the elderly: relationship to cognitive impairment. J Geriatr Psychiatr Neurol 15:175–179

    Google Scholar 

  34. Ghika-Schmid F, Ghika J, Regli N, et al. (1996) Hashimoto’s myoclonic encephalopathy: an underdiagnosed treatable condition?. Mov Disord 11:555–562

    Article  PubMed  CAS  Google Scholar 

  35. Gukuyener K, Serdaroglu A, Bideci A, et al. (2000) Tremor and myoclonus heralding hashimoto’s encephalopathy. J Pediatr End Metab 13:1137–1141

    Google Scholar 

  36. Henchey R, Cibula J, Helveston W, Malone J, Gilmore RL (1995) Electroencephalographic findings in Hashimoto’s encephalopathy. Neurology 45:977–981

    PubMed  CAS  Google Scholar 

  37. Hernàndez Echebarrìa LE, Saiz A, Graus F, et al. (2000) Detection of 14–3-3 protein in the CSF of a patients with Hashimoto’s encephalopathy. Neurology 54: 1539–1540

    PubMed  Google Scholar 

  38. Hussain NS, Rumbaugh J, Kerr D, Nath A, Hillis AE (2005) Effects of prednisone and plasma exchange on cognitive impairment in Hashimoto encephalopathy. Neurology 64: 165–166

    Article  PubMed  CAS  Google Scholar 

  39. Ishii K, Hayashi A, Tamaoka A, et al. (1995) Case report: thyrotropin-releasing hormone-induced myoclonus and tremor in a patient with Hashimoto’s encephalopathy. Am J Med Sci 310:202–205

    PubMed  CAS  Google Scholar 

  40. Isik U, Tennison M, D’Cruz O (2001) Recurrent encephalopathy and seizures in an adolescent girl. Clin Pediatr 40:273–275

    CAS  Google Scholar 

  41. Jacob S, Rajabally YA (2005) Hashimoto’s encephalopathy: steroid resistance and response to intravenous immunoglobulins. J Neurol Neurosurg Psychiatry 76:455–456

    Article  PubMed  CAS  Google Scholar 

  42. Janes SE, Santosh B, Thomas D, Vyas H (2004) Hashimoto’s encephalopathy: an unusuale cause of seizures in the intensive care unit. Pediatr Crit Care Med 5:578–581

    Article  PubMed  Google Scholar 

  43. Kalita J, Misra UK, Rathore C, et al. (2003) Hashimoto’s encephalopathy: clinical, SPECT and neurophysiological data. QJM 96:455–457

    Article  PubMed  CAS  Google Scholar 

  44. Kastrup O, Maschke M, Schlamann K, Diener HC (2005) Hashimoto encephalopathy and neuralgic amyotrophy – causal link or chance association?. Eur Neurol 53:98–99

    Article  PubMed  CAS  Google Scholar 

  45. Kothbauer-Margreiter I, Sturzenegger M, Komor J, Baumgartner R, Hess CW (1996) Encephalopathy associated with Hashimoto thyroiditis: diagnosis and treatment. J Neurol 243:585–593

    Article  PubMed  CAS  Google Scholar 

  46. Mahad DJ, Staugaitis S, Ruggieri P, et al. (2005) Steroid-responsive encephalopathy associated with autoimmune thyroiditis and primary CNS demyelination. J Neurol Sci 228: 3–5

    Article  PubMed  Google Scholar 

  47. Mahmud FH, Ranaud DL, Reed AM, Brands CK (2003) Steroid-responsive encephalopathy associated with Hashimoto’s thyroiditis in an adolescent with chronic hallucinations and depression: case report and review. Pediatrics 112:686–690

    Article  PubMed  Google Scholar 

  48. Mancardi MM, Fazzini F, Rossi A, Gaggero R (2005) Hashimoto’s encephalopathy with selective involvement of the nucleus accumbens: a case report. Neuropediatrics 36:218–220

    Article  PubMed  CAS  Google Scholar 

  49. Maydell Bv, Kopp M, Komorowski GV, et al. (2002) Hashimoto encephalopathy-is it underdiagnosed in pediatric patients?. Neuropediatrics 33:86–89

    Article  Google Scholar 

  50. McCabe DJ, Burke T, Connolly S, Hutchinson M (2000) Amnesic syndrome with bilateral mesial temporal lobe involvement in Hashimoto’s encephalopathy. Neurology 54:737–739

    PubMed  CAS  Google Scholar 

  51. McGinley J, McCabe DJ, Fraser A, et al. (2000) Hashimoto’s encephalopathy: an unusual cause of status epilepticus. Ir Med J 93:118

    PubMed  CAS  Google Scholar 

  52. McKeon A, McNamara B, Sweeney B (2004) Hashimoto’s encephalopathy presenting with psychosis and generalized absence status. J Neurol 251:1025–1027

    Article  PubMed  Google Scholar 

  53. Mignot E, Lammers GJ, Ripley B, et al. (2002) The role of cerebrospinal fluid hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias. Arch Neurol 59:1553–1562

    Article  PubMed  Google Scholar 

  54. Müssig K, Bartels M, Galwitz B, et al. (2005) Hashimoto’s encephalopathy presenting with bipolar affective disorder. Bipolar Disord 7:1–6

    Google Scholar 

  55. Nagpal T, Pande S (2004) Hashimoto’s encephalopathy: response to plasma exchange. Neurol India 52:245–247

    PubMed  Google Scholar 

  56. Nemeroff CB, Simon JS, Haggerty JJ, Evans DL (1985) Antithyroid antibodies in depressed patients. Am J Psychiatry 142:840–843

    PubMed  CAS  Google Scholar 

  57. Nieuwenhuis L, Santens P, Vanwalleghem P, Boon P (2004) Subacute Hashimoto’s encephalopathy treated with plasmapheresis. Acta Neurol Belg 104:80–83

    PubMed  CAS  Google Scholar 

  58. Nolte KW, Unbehaun A, Sieker H, Kloss TM, Paulus W (2000) Hashimoto encephalopathy: a brainstem vasculitis?. Neurology 54:769–770

    PubMed  CAS  Google Scholar 

  59. Ochi H, Horiuchi I, Araki N, et al. (2002) Proteonomic analysis of human brain identifies (-enolase as a novel autoantigen in Hashimoto’s encephalopathy. FEBS 528:197–202

    Article  CAS  Google Scholar 

  60. Oide T, Tokuda T, Masahide Y, et al. (2004) Anti-neuronal antibody in Hashimoto’s encephalopathy: neuropathological, immunohistochemical, and biochemical analysis of two patients. J Neurol Sci 217: 7–12

    Article  PubMed  CAS  Google Scholar 

  61. Papathanasopoulos P, Mallioris K, Karanasios P, Dimopoulos D, Papapetropoulos T (2000) Febrile Hashimoto’s encephalopathy. J Neurol Neurosurg Psychiatry 68:795

    Article  PubMed  CAS  Google Scholar 

  62. Passarella B, Negro C, Nozzoli C., et al. (2005) Cerebellar subacute sindrome due to corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called “Hashimoto’s encephalopathy”). Clin Ter 156:13–17

    PubMed  CAS  Google Scholar 

  63. Peschen-Rosin R, Schabet M, Dichgans J (1999) Manifestation of Hashimoto’s encephalopathy years before onset of thyroid disease. Eur Neurol 41:79–84

    Article  PubMed  CAS  Google Scholar 

  64. Piga M, Serra A, Deiana L, et al. (2004). Brain perfusion abnormalities in patients with euthyroid autoimmune thyroiditis. Eur J Nucl Med Mol Im 31:1639–1644

    Article  CAS  Google Scholar 

  65. Pozo-Rosich P, Villoslada P, Canton A, et al. (2002) Reversible white matter alterations in encephalopathy associated with autoimmune thyroid disease. J Neurol 249:1063–1065

    Article  PubMed  Google Scholar 

  66. Rekand T, Vedeler C, Gramstad A, Bindoff L (2003) Hashimoto’s encephalopathy: a treatable cause of mental impairment, stroke and seizures. Eur J Neurol 10:746–747

    Article  PubMed  CAS  Google Scholar 

  67. Schäuble B, Castillo PR, Boeve BF, Westmoreland BF (2003) EEG findings in steroid-responsive encephalopathy associated with autoimmune thyroiditis. Clin Neurophysiol 114:32–37

    Article  PubMed  Google Scholar 

  68. Seipelt M, Zerr I, Nau R, et al. (1999) Hashimoto’s encephalitis as a differential diagnosis of Creutzfeld-Jakob disease. J Neurol Neurosurg Psychiatry 66:172–176

    Article  PubMed  CAS  Google Scholar 

  69. Sellal F, Berton C, Andriantseheno M, Clerc C (2002) Hashimoto’s encephalopathy. Exacerbations associated with menstrual cycle. Neurology 59:1633–1635

    PubMed  CAS  Google Scholar 

  70. Seo SW, Lee BI, Lee JD, et al. (2003) Thyrotoxic autoimmune encephalopathy: a repeat positron emission tomography study. J Neurol Neurosurg Psychiatry 74:504–506

    Article  PubMed  CAS  Google Scholar 

  71. Shaw PJ, Bates D, Kendall-Taylor P (1988) Hyperthyroidism presenting as pyramidal tract disease. BMJ 297:1395–1396

    Article  PubMed  CAS  Google Scholar 

  72. Shaw PJ, Walls TJ, Newman PK, Cleland PG, Cartlidge NE (1991) Hashimoto’s encephalopathy: a steroid responsive disorder associated with high anti-thyroid antibody titers-report of 5 cases. Neurology 41:228–233

    PubMed  CAS  Google Scholar 

  73. 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 8:60–64

    PubMed  CAS  Google Scholar 

  74. Sheng B, Lau KK, Li HL, Cheng LF (2005) A case of Hashimoto’s encephalopathy with demyelinating peripheral neuropathy. Eur Neurol 53:84–85

    Article  PubMed  Google Scholar 

  75. Skodda S, Kraus M, Durwen H, Gehlen W (1999) Hashimoto’s encephalopathy associated with isolated ACTH deficiency and hyponatremia: a case report. J Neurol 246:970–971

    Article  PubMed  CAS  Google Scholar 

  76. Sommerfield AJ, Stimson R, Campbell IW (2004) Hashimoto’s encephalopathy presenting as an acute medical emergency. Scott Med J 49:155–156

    PubMed  CAS  Google Scholar 

  77. Song YM, Seo DW, Chang GY (2004) MR findings in Hashimoto encephalopathy. Am J Neuroradiol 25:807–808

    PubMed  Google Scholar 

  78. Spiegel J, Hellwig D, Becker G, Műller M (2004) Progressive dementia caused by Hashimoto’s encephalopathy-report of two cases. Eur J Neurol 11:711–713

    Article  PubMed  CAS  Google Scholar 

  79. Sue CM, Morris JGL (1997). Another case of Hashimoto’s encephalopathy. Mov Disord 12:471–472

    PubMed  CAS  Google Scholar 

  80. Suzuki N, Mitamura R, Ohmi H, et al. (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 153:78–79

    PubMed  CAS  Google Scholar 

  81. Sybesma CA, Pinxteren-Nagler EV, Sinnige LGF, van Oostrom JCH, de Vries TW (1999). Hashimoto encephalopathy in a 12-year-old girl Eur J Pediatr 158: 867

    Article  PubMed  CAS  Google Scholar 

  82. Takahashi S, Mitamura R, Itoh Y, Suzuki N, Okuno A (1994) Hashimoto encephalopathy: etiologic considerations. Pediatr Neurol 11:328–331

    Article  PubMed  CAS  Google Scholar 

  83. Taurin G. Golfier V, Pinel JF, et al. (2002) Choreic syndrome due to Hashimoto’s encephalopathy. Mov Disord 17:1091–1092

    Article  Google Scholar 

  84. Taylor SE, Garalda ME, Tudor-Williams G, Martinez-Alier N (2003) An organic cause of neuropsychiatric illness in adolescence. Lancet 361:572

    Article  PubMed  CAS  Google Scholar 

  85. Thrush DC, Boddie HG (1974) Episodic encephalopathy associated with thyroid disorders. J Neurol Neurosurg Psychiatry 37:696–700

    Article  PubMed  CAS  Google Scholar 

  86. Tumani H, Nölker G, Reiber H (1995) Relevance of cerebrospinal fluid parameters for early diagnosis in neuroborreliosis. Neurology 45:1663–1670

    PubMed  CAS  Google Scholar 

  87. Vander T, Hallevy C, Alsaed I, et al. (2004) 14–3-3 protein in the CSF of a patient with Hashimoto’s encephalopathy. J Neurol 251:1273–1274

    Article  PubMed  CAS  Google Scholar 

  88. Vasconcellos E, Pina-Garza JE, Fakhoury T, Fenichel GM (1999) Pediatric manifestations of Hashimoto’s encephalopathy. Pediatr Neurol 20:394–398

    Article  PubMed  CAS  Google Scholar 

  89. Warren CL, Sawhney IMS (2004) Encephalopathy with hypothyroidism. J R Soc Med 97:287–288

    Article  PubMed  Google Scholar 

  90. Watemberg N, Willis D, Pellock JM (2000). Encephalopathy as the presenting symptom of Hashimoto’s thyroiditis. J Child Neurol 15:66–69

    PubMed  CAS  Google Scholar 

  91. Zettinig G, Asenbaum S, Fueger BJ, et al. (2003) Increased prevalence of subclinical brain perfusion abnormalities in patients with autoimmune thyroiditis: evidence of Hashimoto’s encephalopathy?. Clin Endocrinol 59:637–643

    Article  Google Scholar 

Download references

Acknowledgements

Dr Ferracci wishes to thank his wife, Dr. Carla Camana Ph.D., for her valuable cooperation in the analysis of the literature.

This paper is dedicated to our mentors and friends Prof. Marco Onofrj, M.D and Dr. Angelo Raffaele Massaro, M.D.

Author information

Authors and Affiliations

  1. Divisione di Neurologia, Ospedale San Martino, Viale Europa, 32100, Belluno, Italy

    Franco Ferracci M.D

  2. Dipartimento di Neuroscienze e Organi di Senso, A.C.O. San Filippo Neri, Roma, Italy

    Antonio Carnevale MD

Authors
  1. Franco Ferracci M.D
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Antonio Carnevale MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Franco Ferracci M.D.

Additional information

Received in revised form: 8 January 2006

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ferracci, F., Carnevale, A. The neurological disorder associated with thyroid autoimmunity. J Neurol 253, 975–984 (2006). https://doi.org/10.1007/s00415-006-0170-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-006-0170-7

Keywords

Search

Navigation