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Published in: BMC Neurology 1/2023

Open Access 01-12-2023 | Encephalopathy | Research

Clinical presentations and treatment outcomes of Hashimoto encephalopathy at Siriraj Hospital – Thailand’s largest national tertiary referral center

Authors: Chaisak Dumrikarnlert, Smathorn Thakolwiboon, Vorapun Senanarong

Published in: BMC Neurology | Issue 1/2023

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Abstract

Background

Hashimoto encephalopathy has multiple clinical presentations, and other than the presence of thyroid antibody, laboratory and imaging investigations are all non-specific. Data specific to the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy in Thailand remain scarce.

Objectives

To retrospectively investigate the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy at Siriraj Hospital.

Methods

Patients who presented with acute encephalopathy at our center during July 2012-March 2017 were evaluated for eligibility. The inclusion criteria were positive anti-thyroperoxidase (anti-TPO) or anti-thyroglobulin (anti-Tg) in serum with negative neuronal antibody in serum or cerebral spinal fluid (CSF). Clinical presentations, symptom duration, laboratory results of thyroid status and thyroid autoantibody, CSF study, and clinical outcomes were collected.

Results

Of the 204 patients who presented with encephalopathy, 31 (15.2%) were positive for the anti-TPO or anti-Tg antibody. Of those, 13 patients met the diagnostic criteria for Hashimoto encephalopathy. Clinical presentations included cognitive impairment (76.9%), clouding of consciousness (46.2%), and behavior change (30.8%). The neuropsychiatric presentations were visual hallucination (30.8%), auditory hallucination (15.4%), delusion (7.7%), and mood disturbance (23.1%). Other clinical presentations included seizure (38.5%), abnormal movement (23.1%), sleep disturbance (38.5%), ataxia (46.2%), stroke-like episode (15.4%), and fever (15.4%). Most patients (76.9%) had onset within < 3 months. Regarding outcomes, 1 patient who did not receive corticosteroid died from status epilepticus and septic shock. Among the 12 patients who received corticosteroid, 9 (75%) had marked improvement, 1 (8.3%) had slight improvement, and 2 (16.6%) had no clinical improvement. Seven patients (53.9%) had normal thyroid function, 4 patients (30.8%) had subclinical hypothyroidism, and 2 patients (15.4%) had subclinical hyperthyroidism.

Conclusions

The results of this study revealed cognitive impairment, neuropsychiatric symptoms, seizure, ataxia, and sleep disturbance to be common manifestations of Hashimoto encephalopathy. This condition should always be considered in individuals with subacute onset of unexplained cognitive impairment or cerebellar ataxia. Laboratory and neuroimaging investigations were all found to be nonspecific in Hashimoto encephalopathy. Most patients responded well to treatment, so clinical suspicion and early diagnosis and treatment will lead to improved patient outcomes.
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Literature
1.
go back to reference Castillo P, Woodruff B, Caselli R, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch neurol. 2006;63:197–202.CrossRefPubMed Castillo P, Woodruff B, Caselli R, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch neurol. 2006;63:197–202.CrossRefPubMed
2.
go back to reference Kothbauer-Margreiter I, Sturzenegger M, Komor J, et al. Encephalopathy associated with Hashimoto thyroiditis: diagnosis and treatment. J Neurol. 1996;243:585–93.CrossRefPubMed Kothbauer-Margreiter I, Sturzenegger M, Komor J, et al. Encephalopathy associated with Hashimoto thyroiditis: diagnosis and treatment. J Neurol. 1996;243:585–93.CrossRefPubMed
3.
go back to reference Afshari M, Afshari ZS, Schuele SU. Pearls & oy-sters: Hashimoto encephalopathy. Neurology. 2012;78:e134–7.CrossRefPubMed Afshari M, Afshari ZS, Schuele SU. Pearls & oy-sters: Hashimoto encephalopathy. Neurology. 2012;78:e134–7.CrossRefPubMed
4.
go back to reference Matsunaga A, Ikawa M, FujiiA, et al. Hashimoto’s encephalopathy as a treatable adult-onset cerebellar ataxia mimicking spinocerebellar degeneration. Eur Neurol. 2013;69:14–20.CrossRefPubMed Matsunaga A, Ikawa M, FujiiA, et al. Hashimoto’s encephalopathy as a treatable adult-onset cerebellar ataxia mimicking spinocerebellar degeneration. Eur Neurol. 2013;69:14–20.CrossRefPubMed
6.
7.
go back to reference Waliszewska-Prosół M, Ejma M. Hashimoto Encephalopathy-Still more questions than answers. Cells 2022 Sep 14;11(18):2873. Waliszewska-Prosół M, Ejma M. Hashimoto Encephalopathy-Still more questions than answers. Cells 2022 Sep 14;11(18):2873.
8.
9.
go back to reference Laurent C, Capron J, Quillerou B, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): characteristics, treatment and outcome in 251 cases from the literature. Autoimmun Rev. 2016;15:1129–33.CrossRefPubMed Laurent C, Capron J, Quillerou B, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): characteristics, treatment and outcome in 251 cases from the literature. Autoimmun Rev. 2016;15:1129–33.CrossRefPubMed
10.
go back to reference Marshall GA, Doyle JJ. Long-term treatment of Hashimoto’s encephalopathy. J Neuropsychiatry Clin Neurosci. 2006;18:14–20.CrossRefPubMed Marshall GA, Doyle JJ. Long-term treatment of Hashimoto’s encephalopathy. J Neuropsychiatry Clin Neurosci. 2006;18:14–20.CrossRefPubMed
11.
go back to reference Chaudhuri A, Behan PO. The clinical spectrum, diagnosis, pathogenesis and treatment of Hashimoto’s encephalopathy (recurrent acute disseminated encephalomyelitis). Curr Med Chem. 2003;10:1945–53.PubMed Chaudhuri A, Behan PO. The clinical spectrum, diagnosis, pathogenesis and treatment of Hashimoto’s encephalopathy (recurrent acute disseminated encephalomyelitis). Curr Med Chem. 2003;10:1945–53.PubMed
12.
go back to reference Teasdale G, Jennett B. Assessment of coma and impaired consciousness. A practical scale. Lancet. 1974;2(7872):81–4.CrossRefPubMed Teasdale G, Jennett B. Assessment of coma and impaired consciousness. A practical scale. Lancet. 1974;2(7872):81–4.CrossRefPubMed
13.
15.
go back to reference Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol. 2003;60(2):164–71.CrossRefPubMed Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol. 2003;60(2):164–71.CrossRefPubMed
16.
go back to reference Mattozzi S, Sabater L, Escudero D, et al. Hashimoto encephalopathy in the 21st century. Neurology. 2020;94:e1–e8.CrossRef Mattozzi S, Sabater L, Escudero D, et al. Hashimoto encephalopathy in the 21st century. Neurology. 2020;94:e1–e8.CrossRef
17.
go back to reference Sliwinska A, Fumuso P, Stringer B, et al. Hashimoto Encephalopathy With Status Epilepticus Cureus. 2020;12(12):e11857.PubMed Sliwinska A, Fumuso P, Stringer B, et al. Hashimoto Encephalopathy With Status Epilepticus Cureus. 2020;12(12):e11857.PubMed
18.
go back to reference Guasp M, Giné-Servén E, Maudes E, et al. Clinical, neuroimmunologic, and CSF investigations in First Episode Psychosis. Neurology. 2021;97(1):e61–e75.CrossRefPubMed Guasp M, Giné-Servén E, Maudes E, et al. Clinical, neuroimmunologic, and CSF investigations in First Episode Psychosis. Neurology. 2021;97(1):e61–e75.CrossRefPubMed
19.
go back to reference Kayser MS, Titulaer MJ, Gresa-Arribas N, et al. Frequency and characteristics of isolated psychiatric episodes in anti-N-methyl-d-aspartate receptor encephalitis. JAMA Neurol. 2013;70(9):1133–9.CrossRefPubMed Kayser MS, Titulaer MJ, Gresa-Arribas N, et al. Frequency and characteristics of isolated psychiatric episodes in anti-N-methyl-d-aspartate receptor encephalitis. JAMA Neurol. 2013;70(9):1133–9.CrossRefPubMed
20.
go back to reference Höftberger R, van Sonderen A, Leypoldt F, et al. Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology. 2015;84(24):2403–12.CrossRefPubMedPubMedCentral Höftberger R, van Sonderen A, Leypoldt F, et al. Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology. 2015;84(24):2403–12.CrossRefPubMedPubMedCentral
21.
go back to reference Lopez-Chiriboga AS, Komorowski L, Kümpfel T, et al. Metabotropic glutamate receptor type 1 autoimmunity: clinical features and treatment outcomes. Neurology. 2016;86(11):1009–13.CrossRefPubMedPubMedCentral Lopez-Chiriboga AS, Komorowski L, Kümpfel T, et al. Metabotropic glutamate receptor type 1 autoimmunity: clinical features and treatment outcomes. Neurology. 2016;86(11):1009–13.CrossRefPubMedPubMedCentral
22.
go back to reference Shaw PJ, Walls TJ, Newman PK, et al. Hashimoto’s encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers—report of 5 cases. Neurology. 1991;41(2pt1):228–33.CrossRefPubMed Shaw PJ, Walls TJ, Newman PK, et al. Hashimoto’s encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers—report of 5 cases. Neurology. 1991;41(2pt1):228–33.CrossRefPubMed
23.
go back to reference Alazzeh A, Jaroudi S, Gooch M et al. Focal neurological presentation in Hashimoto’s encephalopathy mimicking a vascular occlusion of the middle cerebral artery.BMJ Case Rep 2017; 2017:bcr-2017-219933. Alazzeh A, Jaroudi S, Gooch M et al. Focal neurological presentation in Hashimoto’s encephalopathy mimicking a vascular occlusion of the middle cerebral artery.BMJ Case Rep 2017; 2017:bcr-2017-219933.
24.
go back to reference Graham BR, Shiff N, Nour M, et al. Hashimoto Encephalopathy presenting with Stroke-Like episodes in an adolescent female: a case report and literature review. Pediatr Neurol. 2016;59:62–70.CrossRefPubMed Graham BR, Shiff N, Nour M, et al. Hashimoto Encephalopathy presenting with Stroke-Like episodes in an adolescent female: a case report and literature review. Pediatr Neurol. 2016;59:62–70.CrossRefPubMed
25.
go back to reference Valencia-Sanchez C, Pittock SJ, Mead-Harvey C, et al. Brain dysfunction and thyroid antibodies: autoimmune diagnosis and misdiagnosis. Brain Commun. 2021;3(2):fcaa233.CrossRefPubMedPubMedCentral Valencia-Sanchez C, Pittock SJ, Mead-Harvey C, et al. Brain dysfunction and thyroid antibodies: autoimmune diagnosis and misdiagnosis. Brain Commun. 2021;3(2):fcaa233.CrossRefPubMedPubMedCentral
26.
go back to reference Ferracci F, Carnevale A. The neurological disorder associated with thyroid autoimmunity. J Neurol. 2006;253(8):975–84.CrossRefPubMed Ferracci F, Carnevale A. The neurological disorder associated with thyroid autoimmunity. J Neurol. 2006;253(8):975–84.CrossRefPubMed
27.
go back to reference Zhu Y, Yang H, Xiao F. Hashimoto’s encephalopathy: a report of three cases and relevant literature reviews. Int J Clin Exp Med. 2015;8(9):16817–26.PubMedPubMedCentral Zhu Y, Yang H, Xiao F. Hashimoto’s encephalopathy: a report of three cases and relevant literature reviews. Int J Clin Exp Med. 2015;8(9):16817–26.PubMedPubMedCentral
28.
go back to reference Kelley BP, Patel SC, Marin HL, et al. Autoimmune encephalitis: pathophysiology and imaging review of an overlooked diagnosis. AJNR Am J Neuroradiol. 2017;38:1070–8.CrossRefPubMedPubMedCentral Kelley BP, Patel SC, Marin HL, et al. Autoimmune encephalitis: pathophysiology and imaging review of an overlooked diagnosis. AJNR Am J Neuroradiol. 2017;38:1070–8.CrossRefPubMedPubMedCentral
29.
go back to reference Mocellin R, Walterfang M, Velakoulis D, et al. Hashimoto’s encephalopathy: epidemiology, pathogenesis and management. CNS Drugs. 2007;21(10):799–811.CrossRefPubMed Mocellin R, Walterfang M, Velakoulis D, et al. Hashimoto’s encephalopathy: epidemiology, pathogenesis and management. CNS Drugs. 2007;21(10):799–811.CrossRefPubMed
30.
go back to reference Jegatheeswaran V, Chan M, Chen YA. MRI findings of two patients with Hashimoto Encephalopathy. Cureus. 2021;13(6):e15697.PubMedPubMedCentral Jegatheeswaran V, Chan M, Chen YA. MRI findings of two patients with Hashimoto Encephalopathy. Cureus. 2021;13(6):e15697.PubMedPubMedCentral
31.
go back to reference Ochi H, Horiuchi I, Araki N, et al. Proteomic analysis of human brain identifies alpha-enolase as a novel autoantigen in Hashimoto encephalopathy. FEBS Lett. 2002;528:197–202.CrossRefPubMed Ochi H, Horiuchi I, Araki N, et al. Proteomic analysis of human brain identifies alpha-enolase as a novel autoantigen in Hashimoto encephalopathy. FEBS Lett. 2002;528:197–202.CrossRefPubMed
32.
go back to reference Fujii A, Yoneda M, Ito T, et al. Autoantibodies against the amino terminal of α-enolase are a useful diagnostic marker of Hashimoto encephalopathy. J Neuroimmunol. 2005;162:130–6.CrossRefPubMed Fujii A, Yoneda M, Ito T, et al. Autoantibodies against the amino terminal of α-enolase are a useful diagnostic marker of Hashimoto encephalopathy. J Neuroimmunol. 2005;162:130–6.CrossRefPubMed
33.
go back to reference Yoneda M, Fujii A, Ito A, et al. High prevalence of serum autoantibodies against the amino terminal of alpha-enolase in Hashimoto encephalopathy. J Neuroimmunol. 2007;185(1–2):195–200.CrossRefPubMed Yoneda M, Fujii A, Ito A, et al. High prevalence of serum autoantibodies against the amino terminal of alpha-enolase in Hashimoto encephalopathy. J Neuroimmunol. 2007;185(1–2):195–200.CrossRefPubMed
34.
go back to reference Terrier B, Degand N, Guilpain P, et al. Alpha-enolase: a target of antibodies in infectious and autoimmune diseases. Autoimmun Rev. 2007;6(3):176–82.CrossRefPubMed Terrier B, Degand N, Guilpain P, et al. Alpha-enolase: a target of antibodies in infectious and autoimmune diseases. Autoimmun Rev. 2007;6(3):176–82.CrossRefPubMed
35.
go back to reference Sechi E, Flanagan EP. Antibody-mediated Autoimmune Diseases of the CNS: Challenges and Approaches to diagnosis and management. Front Neurol. 2021;12:673339.CrossRefPubMedPubMedCentral Sechi E, Flanagan EP. Antibody-mediated Autoimmune Diseases of the CNS: Challenges and Approaches to diagnosis and management. Front Neurol. 2021;12:673339.CrossRefPubMedPubMedCentral
36.
go back to reference Dupont S. Non convulsive status epilepticus in the elderly. Geriatr Psychol Neuropsychiatr Vieil. 2019;17(S1):25–30.PubMed Dupont S. Non convulsive status epilepticus in the elderly. Geriatr Psychol Neuropsychiatr Vieil. 2019;17(S1):25–30.PubMed
37.
go back to reference Flanagan EP, Geschwind MD, Lopez-Chiriboga AS, et al. Autoimmune encephalitis misdiagnosis in adults. JAMA Neurol. 2023;80(1):30–9.CrossRefPubMed Flanagan EP, Geschwind MD, Lopez-Chiriboga AS, et al. Autoimmune encephalitis misdiagnosis in adults. JAMA Neurol. 2023;80(1):30–9.CrossRefPubMed
38.
go back to reference Valencia-Sanchez C, Pittock SJ, Mead-Harvey C, et al. Brain dysfunction and thyroid antibodies: autoimmune diagnosis and misdiagnosis. Brain Commun. 2021;3(2):fcaa233.CrossRefPubMedPubMedCentral Valencia-Sanchez C, Pittock SJ, Mead-Harvey C, et al. Brain dysfunction and thyroid antibodies: autoimmune diagnosis and misdiagnosis. Brain Commun. 2021;3(2):fcaa233.CrossRefPubMedPubMedCentral
39.
go back to reference Litmeier S, Pruss H, Witsch E, et al. Initial serum thyroid peroxidase antibodies and long-term outcomes in SREAT. Acta Neurol Scand. 2016;134:452–7.CrossRefPubMed Litmeier S, Pruss H, Witsch E, et al. Initial serum thyroid peroxidase antibodies and long-term outcomes in SREAT. Acta Neurol Scand. 2016;134:452–7.CrossRefPubMed
40.
go back to reference Riangwiwat T, Sangtian J, Sriphrapradang C. Steroid-responsive encephalopathy: an under recognized aspect of Hashimoto’s thyroiditis. Case Reports 2015; 2015: bcr2014208969. Riangwiwat T, Sangtian J, Sriphrapradang C. Steroid-responsive encephalopathy: an under recognized aspect of Hashimoto’s thyroiditis. Case Reports 2015; 2015: bcr2014208969.
41.
go back to reference Termsarasab P, Pitakpatapee Y, Frucht SJ et al. Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) presenting with pure cerebellar Ataxia. Tremor Other Hyperkinet Mov (N Y) 2018; 8: 585. Termsarasab P, Pitakpatapee Y, Frucht SJ et al. Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) presenting with pure cerebellar Ataxia. Tremor Other Hyperkinet Mov (N Y) 2018; 8: 585.
42.
go back to reference Charoensri A, Tunlayadechanont S, Apiwattanakul M, et al. Autoimmune antibody in encephalopathic patients: a pilot study. J Med Assoc Thai. 2021;104:943–50.CrossRef Charoensri A, Tunlayadechanont S, Apiwattanakul M, et al. Autoimmune antibody in encephalopathic patients: a pilot study. J Med Assoc Thai. 2021;104:943–50.CrossRef
43.
go back to reference Sharma PM, Javali M, Mahale R, et al. Hashimoto encephalopathy: a study of the clinical profile, radiological and electrophysiological correlation in a Tertiary Care Center in South India. J Neurosci Rural Pract. 2015;6(3):309–14.CrossRefPubMedPubMedCentral Sharma PM, Javali M, Mahale R, et al. Hashimoto encephalopathy: a study of the clinical profile, radiological and electrophysiological correlation in a Tertiary Care Center in South India. J Neurosci Rural Pract. 2015;6(3):309–14.CrossRefPubMedPubMedCentral
Metadata
Title
Clinical presentations and treatment outcomes of Hashimoto encephalopathy at Siriraj Hospital – Thailand’s largest national tertiary referral center
Authors
Chaisak Dumrikarnlert
Smathorn Thakolwiboon
Vorapun Senanarong
Publication date
01-12-2023
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2023
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-023-03305-4

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