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Published in: BMC Neurology 1/2010

Open Access 01-12-2010 | Research article

Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes

Authors: Gianluca Tamagno, Yahya Celik, Rafael Simó, Marcel Dihné, Kazumi Kimura, Giorgio Gelosa, Byung I Lee, Caroline Hommet, Giovanni Murialdo

Published in: BMC Neurology | Issue 1/2010

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Abstract

Background

The encephalopathy associated with autoimmune thyroid disease (EAATD) is characterized by neurological/psychiatric symptoms, high levels of anti-thyroid antibodies, increased cerebrospinal fluid protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to the corticosteroid treatment in patients with an autoimmune thyroid disease. Almost all EAATD patients are affected by Hashimoto's thyroiditis (HT), although fourteen EAATD patients with Graves' disease (GD) have been also reported.

Methods

We have recorded and analyzed the clinical, biological, radiological, and electrophysiological findings and the data on the therapeutic management of all GD patients with EAATD reported so far as well as the clinical outcomes in those followed-up in the long term.

Results

Twelve of the fourteen patients with EAATD and GD were women. The majority of GD patients with EAATD presented with mild hyperthyroidism at EAATD onset or shortly before it. Active anti-thyroid autoimmunity was detected in all cases. Most of the patients dramatically responded to corticosteroids. The long term clinical outcome was benign but EAATD can relapse, especially at the time of corticosteroid dose tapering or withdrawal. GD and HT patients with EAATD present with a similar clinical, biological, radiological, and electrophysiological picture and require an unaffected EAATD management.

Conclusions

GD and HT equally represent the possible background condition for the development of EAATD, which should be considered in the differential diagnosis of all patients with encephalopathy of unknown origin and an autoimmune thyroid disease, regardless of the nature of the underlying autoimmune thyroid disease.
Literature
1.
go back to reference Brain L, Jellinek EH, Ball K: Hashimoto's disease and encephalopathy. Lancet. 1966, 2: 512-514. 10.1016/S0140-6736(66)92876-5.CrossRefPubMed Brain L, Jellinek EH, Ball K: Hashimoto's disease and encephalopathy. Lancet. 1966, 2: 512-514. 10.1016/S0140-6736(66)92876-5.CrossRefPubMed
2.
go back to reference Shaw PJ, Walls TJ, Newman PK, Cleland PG, Cartlidge NE: Hashimoto's encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers - report of five cases. Neurology. 1991, 41: 228-233.CrossRefPubMed Shaw PJ, Walls TJ, Newman PK, Cleland PG, Cartlidge NE: Hashimoto's encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers - report of five cases. Neurology. 1991, 41: 228-233.CrossRefPubMed
3.
go back to reference Cantón A, deFàbregas O, Tintoré M, Mesa J, Codina A, Simó R: Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition?. J Neurol Sci. 2000, 176: 65-69. 10.1016/S0022-510X(00)00302-6.CrossRefPubMed Cantón A, deFàbregas O, Tintoré M, Mesa J, Codina A, Simó R: Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition?. J Neurol Sci. 2000, 176: 65-69. 10.1016/S0022-510X(00)00302-6.CrossRefPubMed
4.
go back to reference Sawka AM, Fatourechi V, Boeve BF, Mokri B: Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996. Thyroid. 2002, 12: 393-398. 10.1089/105072502760043477.CrossRefPubMed Sawka AM, Fatourechi V, Boeve BF, Mokri B: Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996. Thyroid. 2002, 12: 393-398. 10.1089/105072502760043477.CrossRefPubMed
5.
go back to reference Forchetti CM, Katsamakis G, Garron DC: Autoimmune thyroiditis and a rapidly progressive dementia: global hypoperfusion on SPECT scanning suggests a possible mechanism. Neurology. 1997, 49: 623-626.CrossRefPubMed Forchetti CM, Katsamakis G, Garron DC: Autoimmune thyroiditis and a rapidly progressive dementia: global hypoperfusion on SPECT scanning suggests a possible mechanism. Neurology. 1997, 49: 623-626.CrossRefPubMed
6.
go back to reference Nolte KW, Unbehaun A, Sieker H, Kloss TM, Paulus W: Hashimoto encephalopathy: a brainstem vasculitis?. Neurology. 2000, 54: 769-770.CrossRefPubMed Nolte KW, Unbehaun A, Sieker H, Kloss TM, Paulus W: Hashimoto encephalopathy: a brainstem vasculitis?. Neurology. 2000, 54: 769-770.CrossRefPubMed
7.
go back to reference Zettinig G, Asenbaum S, Fueger BJ, Hofmann A, Diemling M, Mittlboeck M, Dudczak R: Increased prevalence of subclinical brain perfusion abnormalities in patients with autoimmune thyroiditis: evidence of Hashimoto's encephalitis?. Clin Endocrinol (Oxford). 2003, 59: 637-643. 10.1046/j.1365-2265.2003.01901.x.CrossRef Zettinig G, Asenbaum S, Fueger BJ, Hofmann A, Diemling M, Mittlboeck M, Dudczak R: Increased prevalence of subclinical brain perfusion abnormalities in patients with autoimmune thyroiditis: evidence of Hashimoto's encephalitis?. Clin Endocrinol (Oxford). 2003, 59: 637-643. 10.1046/j.1365-2265.2003.01901.x.CrossRef
8.
go back to reference Ferracci F, Bertiato G, Moretto G: Hashimoto's encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci. 2004, 217: 165-168. 10.1016/j.jns.2003.09.007.CrossRefPubMed Ferracci F, Bertiato G, Moretto G: Hashimoto's encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci. 2004, 217: 165-168. 10.1016/j.jns.2003.09.007.CrossRefPubMed
9.
go back to reference Ochi H, Horiuchi I, Araki N, Toda T, Araki T, Sato K, Murai H, Osoegawa M, Yamada T, Okamura K, Ogino T, Mizumoto K, Yamashita H, Saya H, Kira J: Proteomic analysis of human brain identifies alpha-enolase as a novel autoantigen in Hashimoto's encephalopathy. FEBS Lett. 2002, 528: 197-202. 10.1016/S0014-5793(02)03307-0.CrossRefPubMed Ochi H, Horiuchi I, Araki N, Toda T, Araki T, Sato K, Murai H, Osoegawa M, Yamada T, Okamura K, Ogino T, Mizumoto K, Yamashita H, Saya H, Kira J: Proteomic analysis of human brain identifies alpha-enolase as a novel autoantigen in Hashimoto's encephalopathy. FEBS Lett. 2002, 528: 197-202. 10.1016/S0014-5793(02)03307-0.CrossRefPubMed
10.
go back to reference Oide T, Tokuda T, Yazaki M, Watarai M, Mitsuhashi S, Kaneko K, Hashimoto T, Ohara S, Ikeda S: Anti-neuronal autoantibody in Hashimoto's encephalopathy: neuropathological, immunohistochemical, and biochemical analysis of two patients. J Neurol Sci. 2004, 217: 7-12. 10.1016/j.jns.2003.08.005.CrossRefPubMed Oide T, Tokuda T, Yazaki M, Watarai M, Mitsuhashi S, Kaneko K, Hashimoto T, Ohara S, Ikeda S: Anti-neuronal autoantibody in Hashimoto's encephalopathy: neuropathological, immunohistochemical, and biochemical analysis of two patients. J Neurol Sci. 2004, 217: 7-12. 10.1016/j.jns.2003.08.005.CrossRefPubMed
11.
go back to reference Chong JY, Rowland LP, Utiger RD: Hashimoto encephalopathy. Syndrome or myth?. Arch Neurol. 2003, 60: 164-171. 10.1001/archneur.60.2.164.CrossRefPubMed Chong JY, Rowland LP, Utiger RD: Hashimoto encephalopathy. Syndrome or myth?. Arch Neurol. 2003, 60: 164-171. 10.1001/archneur.60.2.164.CrossRefPubMed
12.
go back to reference Castillo P, Woodruff B, Caselli R, Vernino S, Lucchinetti C, Swanson J, Noseworthy J, Aksamit A, Carter J, Sirven J, Hunder G, Fatourechi V, Mokri B, Drubach D, Pittock S, Lennon V, Boeve B: Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol. 2006, 63: 197-202. 10.1001/archneur.63.2.197.CrossRefPubMed Castillo P, Woodruff B, Caselli R, Vernino S, Lucchinetti C, Swanson J, Noseworthy J, Aksamit A, Carter J, Sirven J, Hunder G, Fatourechi V, Mokri B, Drubach D, Pittock S, Lennon V, Boeve B: Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol. 2006, 63: 197-202. 10.1001/archneur.63.2.197.CrossRefPubMed
13.
go back to reference Saito H, Fujita N, Miyakoshi M, Arai A, Nagai H: A case of Hashimoto's encephalopathy associated with Graves' disease. Rinsho Shinkeigaku. 2002, 42: 619-622.PubMed Saito H, Fujita N, Miyakoshi M, Arai A, Nagai H: A case of Hashimoto's encephalopathy associated with Graves' disease. Rinsho Shinkeigaku. 2002, 42: 619-622.PubMed
14.
go back to reference Seo SW, Lee BI, Lee JD, Park SA, Kim KS, Kim SH, Yun MJ: Thyrotoxic autoimmune encephalopathy: a repeat positron emission tomography study. J Neurol Neurosurg Psychiatry. 2003, 74: 504-506. 10.1136/jnnp.74.4.504.CrossRefPubMedPubMedCentral Seo SW, Lee BI, Lee JD, Park SA, Kim KS, Kim SH, Yun MJ: Thyrotoxic autoimmune encephalopathy: a repeat positron emission tomography study. J Neurol Neurosurg Psychiatry. 2003, 74: 504-506. 10.1136/jnnp.74.4.504.CrossRefPubMedPubMedCentral
15.
go back to reference Utku U, Asil T, Celik Y, Tucer D: Reversible MR angiographic findings in a patient with autoimmune Graves disease. Am J Neuroradiol. 2004, 25: 1541-543.PubMed Utku U, Asil T, Celik Y, Tucer D: Reversible MR angiographic findings in a patient with autoimmune Graves disease. Am J Neuroradiol. 2004, 25: 1541-543.PubMed
16.
go back to reference Tamagno G, Federspil G, Murialdo G: Clinical and diagnostic aspects of Encephalopathy Associated to Autoimmune Thyroid Disease (or Hashimoto's Encephalopathy). Intern Emerg Med. 2006, 1: 15-23. 10.1007/BF02934715.CrossRefPubMed Tamagno G, Federspil G, Murialdo G: Clinical and diagnostic aspects of Encephalopathy Associated to Autoimmune Thyroid Disease (or Hashimoto's Encephalopathy). Intern Emerg Med. 2006, 1: 15-23. 10.1007/BF02934715.CrossRefPubMed
17.
go back to reference Blanchin S, Coffin C, Viader F, Ruf J, Carayon P, Potier F, Portier E, Comby E, Allouche S, Ollivier Y, Reznik Y, Ballet JJ: Anti-thyroperoxidase antibodies from patients with Hashimoto's encephalopathy bind to cerebellar astrocytes. J Neuroimmunol. 2007, 192: 13-20. 10.1016/j.jneuroim.2007.08.012.CrossRefPubMed Blanchin S, Coffin C, Viader F, Ruf J, Carayon P, Potier F, Portier E, Comby E, Allouche S, Ollivier Y, Reznik Y, Ballet JJ: Anti-thyroperoxidase antibodies from patients with Hashimoto's encephalopathy bind to cerebellar astrocytes. J Neuroimmunol. 2007, 192: 13-20. 10.1016/j.jneuroim.2007.08.012.CrossRefPubMed
18.
go back to reference Mitchell RS, Yager JY, Marks SD: Childhood onset demyelination and Graves' disease: shared antigen or autoimmune clustering?. J Pediatr Endocrinol Metab. 2007, 20: 233-236.CrossRef Mitchell RS, Yager JY, Marks SD: Childhood onset demyelination and Graves' disease: shared antigen or autoimmune clustering?. J Pediatr Endocrinol Metab. 2007, 20: 233-236.CrossRef
19.
go back to reference Hommet C, Mondon K, Huc M, Jeffredo S, Constans T, deToffol B, Camus V: Atypical neuropsychiatric symptoms revealing Hashimoto's encephalopathy. J Am Geriatr Soc. 2007, 55: 1144-1146. 10.1111/j.1532-5415.2007.01219.x.CrossRefPubMed Hommet C, Mondon K, Huc M, Jeffredo S, Constans T, deToffol B, Camus V: Atypical neuropsychiatric symptoms revealing Hashimoto's encephalopathy. J Am Geriatr Soc. 2007, 55: 1144-1146. 10.1111/j.1532-5415.2007.01219.x.CrossRefPubMed
20.
go back to reference Dihné M, Schuier FJ, Schuier M, Cordes J, Hartung HP, Knehans A, Mueller S: Hashimoto encephalopathy following iodine 131 (131I) radiotherapy of Graves disease. Arch Neurol. 2008, 65: 282-283. 10.1001/archneurol.2007.49.CrossRefPubMed Dihné M, Schuier FJ, Schuier M, Cordes J, Hartung HP, Knehans A, Mueller S: Hashimoto encephalopathy following iodine 131 (131I) radiotherapy of Graves disease. Arch Neurol. 2008, 65: 282-283. 10.1001/archneurol.2007.49.CrossRefPubMed
21.
go back to reference Wilcox RA, To T, Koukourou A, Frasca J: Hashimoto's encephalopathy masquerading as acute psychosis. J Clin Neurosci. 2008, 15: 1301-1304. 10.1016/j.jocn.2006.10.019.CrossRefPubMed Wilcox RA, To T, Koukourou A, Frasca J: Hashimoto's encephalopathy masquerading as acute psychosis. J Clin Neurosci. 2008, 15: 1301-1304. 10.1016/j.jocn.2006.10.019.CrossRefPubMed
22.
go back to reference Tateishi Y, Iguchi Y, Kimura K, Aoki J, Uemura J, Shibazaki K: A case of autoimmune thyroid disease presenting posterior reversible encephalopathy syndrome. J Neurol Sci. 2008, 271: 203-206. 10.1016/j.jns.2008.03.011.CrossRefPubMed Tateishi Y, Iguchi Y, Kimura K, Aoki J, Uemura J, Shibazaki K: A case of autoimmune thyroid disease presenting posterior reversible encephalopathy syndrome. J Neurol Sci. 2008, 271: 203-206. 10.1016/j.jns.2008.03.011.CrossRefPubMed
23.
go back to reference Sakurai T, Tanaka Y, Koumura A, Hayashi Y, Kimura A, Hozumi I, Yoneda M, Inuzuka T: Case report of a patient with Hashimoto's encephalopathy associated with Basedow's disease mimicking Creutzfeldt-Jakob disease. Brain Nerve. 2008, 60: 559-565.PubMed Sakurai T, Tanaka Y, Koumura A, Hayashi Y, Kimura A, Hozumi I, Yoneda M, Inuzuka T: Case report of a patient with Hashimoto's encephalopathy associated with Basedow's disease mimicking Creutzfeldt-Jakob disease. Brain Nerve. 2008, 60: 559-565.PubMed
24.
go back to reference Gelosa G, DiFrancesco JC, Tremolizzo L, Lanzani F, Rota S, Colombo M, Perego P, Massimini D, Marzorati L, Curtò NA, Ferrarese C: Autoimmune encephalopathy in Graves' disease: remission after total thyroidectomy. J Neurol Neurosurg Psychiatry. 2009, 80: 698-699. 10.1136/jnnp.2008.152413.CrossRefPubMed Gelosa G, DiFrancesco JC, Tremolizzo L, Lanzani F, Rota S, Colombo M, Perego P, Massimini D, Marzorati L, Curtò NA, Ferrarese C: Autoimmune encephalopathy in Graves' disease: remission after total thyroidectomy. J Neurol Neurosurg Psychiatry. 2009, 80: 698-699. 10.1136/jnnp.2008.152413.CrossRefPubMed
25.
go back to reference Rocha MS, Brucki SM, Ferraz AC: Cerebral vasculitis and Basedow-Graves disease: report of two cases. Arq Neuropsiquiatr. 2001, 59: 948-953.CrossRefPubMed Rocha MS, Brucki SM, Ferraz AC: Cerebral vasculitis and Basedow-Graves disease: report of two cases. Arq Neuropsiquiatr. 2001, 59: 948-953.CrossRefPubMed
26.
go back to reference Kurne A, Aydin OF, Karabudak R: White matter alteration in a patient with Graves' disease. J Child Neurol. 2007, 22: 1128-1131. 10.1177/0883073807306250.CrossRefPubMed Kurne A, Aydin OF, Karabudak R: White matter alteration in a patient with Graves' disease. J Child Neurol. 2007, 22: 1128-1131. 10.1177/0883073807306250.CrossRefPubMed
27.
go back to reference Fujiki F, Tsuboi Y, Saito N, Yamada T: Thyrotoxic encephalopathy showing reversible diffusion-weighted imaging abnormalities. No To Shinkei. 2004, 56: 1017-1023.PubMed Fujiki F, Tsuboi Y, Saito N, Yamada T: Thyrotoxic encephalopathy showing reversible diffusion-weighted imaging abnormalities. No To Shinkei. 2004, 56: 1017-1023.PubMed
28.
go back to reference Payer J, Lisy L, Baqi L, Petrovic T, Langer P: Hashimoto's encepahlopthy - a rare and unusual syndrome. Vnitr Lek. 2007, 53: 300-306.PubMed Payer J, Lisy L, Baqi L, Petrovic T, Langer P: Hashimoto's encepahlopthy - a rare and unusual syndrome. Vnitr Lek. 2007, 53: 300-306.PubMed
29.
go back to reference Peschen-Rosin R, Schabet M, Dichgans J: Manifestation of Hashimoto's encephalopathy years before onset of thyroid disease. Eur Neurol. 1999, 41: 79-84. 10.1159/000008007.CrossRefPubMed Peschen-Rosin R, Schabet M, Dichgans J: Manifestation of Hashimoto's encephalopathy years before onset of thyroid disease. Eur Neurol. 1999, 41: 79-84. 10.1159/000008007.CrossRefPubMed
30.
31.
go back to reference Yuceyar N, Karadeniz M, Erdogan M, Copur A, Akgun A, Kumral E, Ozgen G: Thyrotoxic autoimmune encephalopathy in a female patient: only partial response to typical immunosuppressant treatment and remission after thyroidectomy. Clin Neurol Neurosurg. 2007, 109: 458-462. 10.1016/j.clineuro.2007.02.006.CrossRefPubMed Yuceyar N, Karadeniz M, Erdogan M, Copur A, Akgun A, Kumral E, Ozgen G: Thyrotoxic autoimmune encephalopathy in a female patient: only partial response to typical immunosuppressant treatment and remission after thyroidectomy. Clin Neurol Neurosurg. 2007, 109: 458-462. 10.1016/j.clineuro.2007.02.006.CrossRefPubMed
32.
go back to reference Tsai MH, Lee LH, Chen SD, Lu CH, Chen MT, Chuang YC: Complex partial status epilepticus as a manifestation of Hashimoto's encephalopathy. Seizure. 2007, 16: 713-716. 10.1016/j.seizure.2007.05.018.CrossRefPubMed Tsai MH, Lee LH, Chen SD, Lu CH, Chen MT, Chuang YC: Complex partial status epilepticus as a manifestation of Hashimoto's encephalopathy. Seizure. 2007, 16: 713-716. 10.1016/j.seizure.2007.05.018.CrossRefPubMed
33.
go back to reference Yoneda M, Fujii A, Ito A, Yokoyama H, Nakagawa H, Kuriyama M: High prevalence of serum autoantibodies against the amino terminal of alpha-enolase in Hashimoto's encephalopathy. J Neuroimmunol. 2007, 185: 195-200. 10.1016/j.jneuroim.2007.01.018.CrossRefPubMed Yoneda M, Fujii A, Ito A, Yokoyama H, Nakagawa H, Kuriyama M: High prevalence of serum autoantibodies against the amino terminal of alpha-enolase in Hashimoto's encephalopathy. J Neuroimmunol. 2007, 185: 195-200. 10.1016/j.jneuroim.2007.01.018.CrossRefPubMed
34.
go back to reference Pozo-Rosich P, Villoslada P, Canton A, Simo R, Rovira A, Montalban X: Reversible white matter alterations in encephalopathy associated with autoimmune thyroid disease. J Neurol. 2002, 249: 1063-1065. 10.1007/s00415-002-0788-z.CrossRefPubMed Pozo-Rosich P, Villoslada P, Canton A, Simo R, Rovira A, Montalban X: Reversible white matter alterations in encephalopathy associated with autoimmune thyroid disease. J Neurol. 2002, 249: 1063-1065. 10.1007/s00415-002-0788-z.CrossRefPubMed
Metadata
Title
Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes
Authors
Gianluca Tamagno
Yahya Celik
Rafael Simó
Marcel Dihné
Kazumi Kimura
Giorgio Gelosa
Byung I Lee
Caroline Hommet
Giovanni Murialdo
Publication date
01-12-2010
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2010
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/1471-2377-10-27

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