Steroid response encephalopathy associated with autoimmune thyroiditis
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Alma Mater Europae, Campus College Rezonanca, Privat Bearer of Higher Education, Pristina, Kosovo
University Clinical Center of Kosovo, Clinic of Neurology, Pristina, Kosovo
Medical Faculty Skopje 'Ss Cyril and Methodius,' North Macedonia
Submission date: 2023-12-28
Final revision date: 2024-03-04
Acceptance date: 2024-03-04
Online publication date: 2024-04-16
Corresponding author
Dren Boshnjaku   

Medical Faculty Skopje 'Ss Cyril and Methodius', 50 divizija, 1000, Skopje, North Macedonia
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare autoimmune disorder affecting the central nervous system, characterized by a spectrum of neurological and psychiatric symptoms.

This case study aims to highlight the diagnostic challenges and the successful management of SREAT syndrome in a young woman with autoimmune thyroiditis.

Case study:
A 21-year-old woman with documented Hashimoto's thyroiditis and polycystic ovary syndrome was admitted to the Clinic of Neurology following a 5-minute tonic seizure and subsequent confusion state lasting several hours. Initial brain MRI showed no abnormalities, and EEG revealed generalized slowness. Comprehensive laboratory assessments, including a complete blood count, biochemical analysis, and electrolyte panels, all yielded normal results. Further investigation revealed a significantly elevated anti-thyroid peroxidase antibody (anti-TPO) titer exceeding 1000 IU/mL. The suspicion of SREAT syndrome was considered. Pulse therapy with methylprednisolone was associated with rapid recovery. The patient was discharged from the hospital with an oral corticosteroid tapering regimen.

Results and discussion:
The administration of pulse therapy with methylprednisolone resulted in a rapid and very good response in the patient, evidenced by the resolution of seizure activity and improvement in confusion. Laboratory investigations, particularly the markedly elevated anti-TPO titer, supported the diagnosis of SREAT syndrome. The subsequent management with an oral corticosteroid tapering regimen maintained the patient's clinical stability.

This case highlights the importance of considering autoimmune encephalopathy in patients with a history of autoimmune thyroiditis presenting with neurological and psychiatric symptoms. Further research is warranted to better understand the underlying pathomechanisms.

Ercoli T, Defazio G, Muroni A. Status epilepticus in Hashimoto's encephalopathy. Seizure. 2019;70:1–5.
Brain L, Jellinek EH, Ball K. Hashimoto’s disease and encephalopathy. Lancet. 1966;2(7462):512–514.
Castillo P, Woodruff B, Caselli R, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol. 2006;63(2):197–202.
Payer J, Petrovic T, Lisy L, Langer P. Hashimoto encephalopathy: A rare intricate syndrome. Int J Endocrinol Metab. 2012;10(2):506–514.
Tamagno G, Celik Y, Simó R, et al. Encephalopathy associated with autoimmune thyroid disease in patients with Graves’ disease: Clinical manifestations, follow-up, and outcomes. BMC Neurol. 2010;10:27.
Tamagno G, Federspil G, Murialdo G. Clinical and diagnostic aspects of encephalopathy associated with autoimmune thyroid disease (or Hashimoto’s encephalopathy). Intern Emerg Med. 2006;1(1):15–23.
Watemberg N, Greenstein D, Levine A. Encephalopathy associated with Hashimoto thyroiditis: Pediatric perspective. J Child Neurol. 2006;21(1):1–5.
Shein M, Apter A, Dickerman Z, et al. Encephalopathy in compensated Hashimoto thyroiditis: A clinical expression of autoimmune cerebral vasculitis. Brain Dev 1986;8(1):60–64.
Chaudhuri A, Behan PO. The clinical spectrum, diagnosis, pathogenesis, and treatment of Hashimoto’s encephalopathy (recurrent acute disseminated encephalomyelitis). Curr Med Chem. 2003;10(19):1945–1953.
Mocellin R, Walterfang M, Velakoulis D. Hashimoto's encephalopathy: epidemiology, pathogenesis and management. CNS Drugs. 2007;21:799–811. 10.2165/00023210-200721100- 00002.
Jacob S, Rajabally YA. Hashimoto’s encephalopathy: steroid resistance and response to intravenous immunoglobulins. J Neurol Neurosurg Psychiatry. 2005, 76(3):455–456.
Drulović J, Andrejević S, Bonaci-Nikolić B, Mijailović V. Hashimoto’s encephalopathy: a long lasting remission induced by intravenous immunoglobulins. Vojnosanit Pregl. 2011, 68(5):452–454. 1
Titulaer MJ, McCracken L, Gabilonda I, et al. Treatment and prognostic factors for long-term outcome in patients with anti NMDA receptor encephalitis: an obstervational cohort study. Lancet Neurol 2013;12(2):157–165.
Philip R, Saran S, Gutch M, Gupta K. An unusual presentation of Hashimoto’s encephalopathy. Indian J Endocrinol Metab. 2014;18(1):113–115.
Jacob S, Rajabally YA. Hashimoto’s encephalopathy: steroid resistance and response to intravenous immunoglobulins. J Neurol Neurosurg Psychiatry. 2005;76(3):455–456.
Wiśniewski K, Bełej M, Wojtkiewicz J. Associations between serum levels of thyroid stimulating hormone (TSH), free thyroxine (fT4), free triiodothyronine (fT3) and clinical symptoms in patients diagnosed with Hashimoto’s thyroiditis. Pol Ann Med. 2020;27(2):96–102.
Chaudhuri J, Mukherjee A, Chakravarty A. Hashimoto's Encephalopathy: Case Series and Literature Review. Curr Neurol Neurosci Rep. 2023;23(4):167–175.
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