Hashimoto’s Encephalopathy in a Pediatric Female: Time to Revisit This Diagnosis

  • Toyaja M Jadhav Department of Laboratory Sciences, 7 Airforce Hospital, Kanpur, Uttar Pradesh, India
  • Sunita Patil Department of Microbiology, Armed Forces Medical College, Pune, Maharashtra, India
Keywords: Hashimoto Disease, Thyroiditis, Autoimmune, Cytology

Abstract

Hashimoto’s encephalopathy (HE) is an infrequent encephalopathy of presumed autoimmune origin characterized by high titres of antithyroid antibodies. It is common in females, and although it is seen across all age groups, it is relatively uncommon among the paediatric population, with a prevalence of only about 1.2%. Hashimoto’s encephalopathy is a rare complication of Hashimoto’s thyroiditis and presents with a syndrome of persistent fluctuating neurologic and neuropsychologic deficits associated with elevated antithyroid antibodies, specifically thyroid peroxidase (TPO) antibodies. We present one such case of Hashimoto’s encephalopathy, diagnosed in an 11-year-old female, which mimicked brain death, along with a review of the available literature.

References

Mocellin R, Walterfang M, Velakoulis D. Hashimoto’s Encephalopathy Epidemiology, Pathogenesis and Management. CNS Drugs. 2007;21(10):799–811.

Chiarello P, Talarico V, Nicoletti A, Rodio B, Arcuri PP, Bosco D, et al. Hashimoto encephalopathy: A case report and a short revision of current literature. Acta Biomed. 2020 Sep 11;91(3):1–7.

Payer J, Petrovic T, Lisy L, Langer P. Hashimoto encephalopathy: A rare intricate syndrome. Int J Endocrinol Metab. 2012;10(2):506–14.

Kaulfers AMD, Bhowmick SK. Hashimoto Encephalopathy in Pediatrics: Report of 3 Cases. AACE Clin Case Rep. 2021 Jan 1;7(1):40–2.

Rubin DI. UpToDate. 2023. Hashimoto encephalopathy. Available from: https://www.wolterskluwer.com/en/know/clinical-

Brain L, Jellineck E, Ball K. Hashimoto’s disease and encephalopathy. Lancet. 1966;288(7462):512–4.

Shaw P, Walls T, Newman P, Cleland P, Cartlidge N. Hashimoto’s encephalopathy: A steroid-responsive disorder associated with high anti-thyroid antibody titers-report of 5 cases. Neurology. 1991;44:41–7.

Gayatri NA, Whitehouse WP. Pilot survey of Hashimoto’s encephalopathy in children. Dev Med Child Neurol. 2007 Feb 13;47(8):556–8.

Byrne OC, Zuberi SM, Madigan CA, King MD. Hashimoto’s thyroiditis - A rare but treatable cause of encephalopathy in children. Eur J Paediatr Neurol. 2000;4(6):279–82.

Watemberg N, Greenstein D, Levine A. Encephalopathy Associated With Hashimoto Thyroiditis: Pediatric Perspective. J Child Neurol. 2006;21(1):1–5.

Castillo P, Woodruff B, Caselli R, Vernino S, Lucchinetti C, Swanson J, et al. Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis. Arch Neurol [Internet]. 2006 Feb;63:197–202. Available from: http://archneur.jamanetwork.com/

Duffey P, Yee S, Reid IN, Bridges LR. Hashimoto’s encephalopathy: Postmortem findings after fatal status epilepticus. Neurology. 2003 Oct;61(8):1124–6.

Paulus W, Nolte K. Neuropathology of Hashimoto’s encephalopathy. J Neurol Neurosurg Psychiatry. 2003 Jul;74(7):1009.

Chaudhari A, Behan P. The clinical spectrum, diagnosis, pathogenesis and treatment of Hashimoto’s encephalopathy (recurrent acute disseminated encephalomyelitis). Curr Med Chem. 2003 Oct;10(19):1945–53.

Kothbauer-Margreiter I, Sturzenegger M, Komor J, Baumgartner R, Hess CW. Encephalopathy associated with Hashimoto thyroiditis: diagnosis and treatment. J Neurol. 1996;243(8):585–93.

Ishii K, Hayashi A, Tamaoka A, Usuki S, Mizusawa H, Shoji S. Case Report: Thyrotropin-Releasing Hormone-Induced Myoclonus and Tremor in a Patient With Hashimoto’s Encephalopathy. Am J Med Sci. 1995 Nov;310(5):202–5.

Waliszewska-Prosół M, Ejma M. Hashimoto Encephalopathy—Still More Questions than Answers. Cells. 2022 Sep 1;11(18):1–17.

Forchetti CM, Katsamakis G, Garron DC. Autoimmune thyroiditis and a rapidly progressive dementia: Global hypoperfusion on SPECT scanning suggests a possible mechanism. Neurology. 1997 Aug;49:623–6.

Chong JY, Rowland LP, Utiger RD. Hashimoto Encephalopathy Syndrome or Myth? [Internet]. Available from: http://archneur.jamanetwork.com/

Ferracci F, Moretto G, Candeago RM, Cimini N, Conte F, Gentile M, et al. Antithyroid antibodies in the CSF: Their role in the pathogenesis of Hashimoto’s encephalopathy. Neurology. 2003 Feb 25;60(4):712–4.

Doherty CP. Possibly, probably definitely, Hashimoto encephalopathy. J Neurol Sci. 2005;228:1–2.

Mayo Clinic. Creutzfeldt-Jakob disease. Mayo Clinic. 2023. p. 1–4.

World Health Organisation. Guillain–Barré syndrome. World Health Organisation. 2023. p. 1–6.

Mayo Clinic. Guillain-Barre syndrome. Mayo Clinic. 2023. p. 1–8.

Altabakhi IW, Babiker HM. Paraneoplastic Limbic Encephalitis. In: StatPearls. Arizona: National Library of Medicine; 2022. p. 1–10.

Vasan S, Kumar A. Wernicke Encephalopathy. In: StatPearls. National Library of Medicine; 2023. p. 1–10.

Rocke Z, Belyayeva M. Subacute Sclerosing Panencephalitis. In: StatPearls. National Library of Medicine; 2023. p. 1–10.

Chaudhuri J, Mukherjee A, Chakravarty A. Hashimoto’s Encephalopathy: Case Series and Literature Review. Curr Neurol Neurosci Rep. 2023;23:167–75.

Ram S. India’s contribution on guillain-barre syndrome: Mapping of 40 years research. Neurol India. 2013 Jul;61(4):375–82.

Khetsuriani N, Sanadze K, Abuladze M, Tatishvili N. High risk of subacute sclerosing panencephalitis following measles outbreaks in Georgia. Clin Microbiol Infect. 2020 Jun 1;26(6):737–42.

Chang JS, Chang TC. Hashimoto’s encephalopathy: Report of three cases. J Formos Med Assoc. 2014 Nov 1;113(11):862–6.

Yoshikawa H. Epidemiology of Guillain-Barré Syndrome. Brain Nerve. 2015 Nov 1;67(11):1–4.

Graus F. Paraneoplastic limbic encephalitis. Medlink Neurol. 2023. p. 1–6. Available from: https://www.medlink.com/articles/paraneoplastic-limbic-encephalitis

Cleveland Clinic. Wernicke-Korsakoff Syndrome. Cleveland Clinic. 2023. p. 1–5.

Tesini BL. Subacute Sclerosing Panencephalitis (SSPE). In: MSD MANUAL Consumer Version [Internet]. MSD; 2023. Available from: https://www.msdmanuals.com/en-in/home/children-s-health-issues/common-viral-infections-in-infants-and-children/subacute-sclerosing-panencephalitis

Cleveland Clinic. Creutzfeldt-Jakob Disease (CJD). Cleveland Clinic. 2022. p. 1–8.

Chandrakumar A, Bhardwaj A, T’Jong GW. Review of thiamine deficiency disorders: Wernicke encephalopathy and Korsakoff psychosis. J Basic Clin Physiol Pharmacol. 2019;30:153–62.

Shen K, Xu Y, Guan H, Zhong W, Chen M, Zhao J, et al. Paraneoplastic limbic encephalitis associated with lung cancer. Sci Rep. 2018 Dec 1;8(1).

Papetti L, Amodeo ME, Sabatini L, Baggieri M, Capuano A, Graziola F, et al. Subacute Sclerosing Panencephalitis in Children: The Archetype of Non-Vaccination. Viruses. 2022 Apr 1;14(4).

Sitammagari K, Masood W. Creutzfeldt Jakob Disease. In: StatPearls. National Library of Medicine; 2022.

Van Den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, Van Doorn PA. Guillain-Barré syndrome: Pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014;10:469–82.

Vogrig A, Muñiz-Castrillo S, Desestret V, Joubert B, Honnorat J. Pathophysiology of paraneoplastic and autoimmune encephalitis: genes, infections, and checkpoint inhibitors. Ther Adv Neurol Disord. 2020;13.

Ota Y, Capizzano AA, Moritani T, Naganawa S, Kurokawa R, Srinivasan A. Comprehensive review of Wernicke encephalopathy: pathophysiology, clinical symptoms and imaging findings. Jpn J Radiol. 2020;38:809–20.

Kelemen K, Kövecsi A, Banias L, Klára I, Mihály I, Forró C, et al. A Rare Case of Histopathologically Confirmed Creutzfeldt–Jakob Disease from Romania, Long Route to Diagnosis—Case Report and an Overview of the Romanian CJD Situation. J Clin Med. 2022 Aug 1;11(16).

Medscape. Guillian-Barre syndrome - Medscape Reference. Medscape. 2024. p. 1–5.

Nisarga P, Gyure K. CNS nontumor: Toxic and metabolic disorders - Wernicke-Korsakoff syndrome. Pathology Outlines. 2022. p. 1–5.

Pernick N. CNS nontumor: Infections - Chronic / subacute infections: Subacute sclerosing panencephalitis (SSPE). Pathology Outlines. 2020. p. 1–5.

Betancor OL, Cali I, Baiardi S, Dafou D, Collins SJ. Diagnostic performance of CSF biomarkers in a well-characterized Australian cohort of sporadic Creutzfeldt-Jakob disease.

Zanusso G, Fiorini M, Ferrari S, Gajofatto A, Cagnin A, Galassi A, et al. Cerebrospinal fluid markers in sporadic Creutzfeldt-Jakob disease. Int J Mol Sci. 2011 Sep;12(9):6281–92.

Pritchard J. Guillain-Barré syndrome. Clin Med [Internet]. 2010;10:399–401. Available from: www.gbs.org.uk

Rocke Z, Belyayeva M. Subacute Sclerosing Panencephalitis. Stat Pearls. 2023. p. 1–5.

Knight R. Potential treatments for Creutzfeldt-Jakob disease. 2006.

Altabakhi IW, Babiker HM. Paraneoplastic Limbic Encephalitis. Stat Pearls. 2022. p. 1–5.

Samia P, Oyieke K, Tunje D, Udwadia-Hegde A, Feemster K, Oncel I, et al. Options in the Treatment of Subacute Sclerosing Panencephalitis: Implications for Low Resource Areas. Curr Treat Options Neurol. 2022;24:99–110.

Dumrikarnlert C, Thakolwiboon S, Senanarong V. Clinical presentations and treatment outcomes of Hashimoto encephalopathy at Siriraj Hospital – Thailand’s largest national tertiary referral center. BMC Neurol. 2023 Dec 1;23(1).

Netto AB, Taly AB, Kulkarni GB, Umamaheswara Rao GS, Rao S. Mortality in mechanically ventilated patients of Guillain Barr Syndrome. Ann Indian Acad Neurol. 2011 Oct;14(4):262–6.

Lindberg M, Oyler R. Wernicke’s encephalopathy. Am Fam Physician. 1990 Apr;41(4):1205–9.

Published
26-05-2024
Section
Review Article