Hashimoto’s encepahlopthy – a rare and unusual syndrome
Authors:
J. Payer; L. Lisý; L. Baqi; T. Petrovič; P. Langer
Authors‘ workplace:
5. interná klinika Lekárskej fakulty UK a FNsP Ružinov, Bratislava, Slovenská republika, prednosta prof. MUDr. Juraj Payer, CSc.
1; Neurologická klinika Slovenskej zdravotníckej univerzity, Bratislava, Slovenská republika, prednosta prof. MUDr. Ľubomír Lisý, DrSc.
2; Laboratórium diabetu a výživy Ústavu experimentálnej endokrinológie SAV, Bratislava, Slovenská republika, riaditeľ MUDr. Vladimír Štrbák, DrSc.
3
Published in:
Vnitř Lék 2007; 53(3): 300-306
Category:
Case Reports
Overview
Hashimoto's encephalopathy (HE) is a brain disease associated with autoimmune thyroid disease. Over 100 articles have been published on the topic, especially in connection with hypothyroidism. In addition to an overview of the relevant literature, we also report a case of a female patient with a rare association of HE with thyreotoxicosis. The patient presented with varied clinicalneurological symptoms: she had convulsions, psychotic symptoms, altered consciousness Examinations (laboratory tests, CT, MRI, EEG, CSL exams) detected increased level of proteins in the liquor, episodes of rhythmic δ activity on EEG, increased antithyreoidal antibody titre (TOOab, TGAb, TRAb) in serum. After initial treatment with carbimazole and hydrocortizone, the patient’s condition dramatically improved and she was disconnected from artificial lung ventilation, conscious and convulsion-free. During the following 30 days, the patient would get worse after attempts to withdraw glucocorticoids. In spite of thyreotoxicosis, we classified the condition as HE and the patient was fully stabilised after pulse treatment with methylprednisolone. Clinically, the patient was subject to further outpatient follow up, without symptoms of encephalopathy; glucocorticoids were gradually withdrawn and were discontinued completely after another four months. The authors conclude that HE, even though rare, may pass unnoticed due to its symptoms which are similar to many other and more frequent diseases. HE should be considered in patients with potential or known autoimmune thyroidism and atypical neuropsychiatric manifestation responding to corticoid treatment.
Key words:
Hashimoto’s encephalopathy – autoimmune thyreoiditis – hypothyreodism – hyperthyreosis – glucocorticoids – carbimazole
Sources
1. Archambeaud F, Galinat S, Regouby Y et al. Encéphalopathie de Hashimoto. Analyse de quatre observations. Rev Méd Interne 2001; 22: 653-659.
2. Asher R. Myxedema madness. Brit Med J 1949; 2: 555-562.
3. Azdin-Ozemir Z, Tuzun E, Baykan B et al. Autoimune encephalopathy presenting with epilepsia partialis continua. Clin EEG Neurosci 2006; 37: 204-209.
4. Barker R, Zajicek J, Wilkinson I. Thyrotoxic Hashimoto’s encephalpathy. J Neurol Neurosurg Psychiatry 1996; 60: 234.
5. Berio A, Piazzi A. A case of Kearns-Sayre syndrome with autoimmune thyroiditis and possible Hashimoto encephalopathy. Panminerva Med 2002; 44: 265-269.
6. Brain L, Jellinek EH, Ball K. Hashimoto’s disease and encephalopathy. Lancet 1966; ii: 512-514.
7. Boers PM, Colebatch JG. Hashimotos encephalopathy responding to plasmapheresis. J Neurol Neurosurg Psychiatry 2002; 73: 601.
8. Canton A, Fabregas O, Tintore M et al. Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition? J Neurol Sci 2000; 176: 65-69.
9. Castillo P, Woodruff B, Caselli R et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 2006; 63: 175-176.
10. Chen HC, Masherani U. Hashimoto’s encephalopathy. South Med J 2000; 93: 504-506.
11. Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol 2003; 60: 164-171.
12. Deutsch M, Koskinas J, Tzannos K et al. Hashimoto encephalopathy with pegylated interferon α-2b and ribavirin. Ann Pharmacother 2005; 39: 1745-1748.
13. Ferracci F, Bertiato G, Moretto G. Hashimoto’s encephalopathy: Epidemiologic data and pathogenetic considerations. J Neurol Sci 2004; 217: 165-168.
14. Ferlazzo E, Raffaele M, Mazzu I et al. Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy. Epilepsy Behav 2006; 8: 328-330.
15. Günther P, Kopf A. Hashimoto encephalopathy. Psychiatr Prax 2004; 31: 310-312.
16. Henchey R, Cibula J, Helveston W et al. Electroencephalographic findings in Hashimoto encephalopathy. Neurology 1995; 45: 977-981.
17. Janes SE, Santosh B, Thomas D et al. Hashimoto‘s encephalopathy: an unusual case of seizures in the intensive care unit. Pediatr Crit Care Med 2004; 5: 578-581.
18. Leigh H, Kramer SI. The psychiatric manifestations of endocrinne disorders. Adv Intern Med 1984; 29: 413-445.
19. Mahmud FH, Renald DL, Reed AM et al. Steroid-responsive encephalopathy associated with Hashimoto’s thyroiditis in an adolescent with chronic hallucinations and depression: Case report and review. Pediatrics 2003; 112: 686-690.
20. Maydell B, Kopp M, Komorowski G et al. Hashimoto encephalopathy - is it underdiagnosed in pediatric patients? Neuropediatrics 2002; 33: 86-89.
21. Nagpal T, Pande S. Hashimoto's encephalopathy: Response to plasma exchange. Neurol India 2004; 52: 245-247.
22. Ochi H, Horiuchi I, Araki N et al. Proteomic analysis of human brain identifies α-enolase as a novel autoantigen in Hashimoto encephalopathy. FEBS Letters 2002; 528: 197-202.
23. Peschen-Rosin R, Schabet M, Dichgans J. Manifestation of Hashimoto’s encephalopathy years before onset of thyroid disease. Eur Neurol 1999; 41: 79-84.
24. Rodriguez AJ, Jicha GA, Steeves TD et al. EEG changes in a patient with steroid-responsive encephalopathy associated with antibodies to thyroperoxidase (SREAT), Hashimoto encephalopathy. J Clin Naurophysiol 2006; 23: 371-373.
25. Seo SW, Lee BI, Lee JD et al. Thyrotoxic autoimmune encephalopathy: a repeat positron emission tomography study. J Neurol Neurosurg Psychiatry 2003; 74: 504-506.
26. Shaw PJ, Walls TJ, Newman PK et al. Hashimoto’s encephalopathy, a steroid-responsive disorder associated with high anti-thyroid antibody titers (report of five cases). Neurology 1991; 41: 228-233.
27. Song ZM, Seo DW, Chang GY. MR findings in Hashimoto encephalopathy. AINR Am J Neuroradiol 2004; 25: 807-808.
28. Vasconcellos E, Pina-Garza JE, Fakhoury T et al. Pediatric manifestations of Hashimoto’s encephalopathy. Ped Neurol 1999; 20: 394-398.
29. Watemberg N, Greenstein D, Levine A. Encephalopathy associated with Hashimoto thyroiditis pediatric perspective. J Child Neurol 2006; 21: 1-5.
30. Zettinig G, Asenbaum S, Fueger BJ et al. Increased prevalence of subclinical brain perfusion abnormalities in patients with autoimmune thyroiditis: evidence of Hashimoto’s encephalitis? Clin Endocrinol 2003; 59: 637-643.
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Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
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