Abstract
Introduction
Hashimoto’s encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP).
Case presentation
A 14-year-old male was admitted with right-sided weakness, dysphagia, speech disorder, and aggressiveness. Brain magnetic resonance imaging showed increased intensity in bilateral temporal, insular cortex, amygdala, and parahippocampal area on T2-weighted and fluid-attenuated inversion recovery images. Autoimmune encephalitis was considered as the patient had subacute onset of psychiatric and motor disturbances with normal findings for cerebrospinal fluid. N-methyl-D-aspartate receptor, anti-glutamate-type α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 and 2, anti-contactin-associated protein-like 2, anti-gamma-aminobutyric acid receptor, anti-Leucine-rich, and glioma-inactivated 1 antibodies were negative but the anti-thyroperoxidase (antiTPO) level was greater than 998 IU/ML (n:0–9). Steroid therapy was initiated as pulse therapy and maintained with 2-mg/kg/day dose with the diagnosis of HE. He was symptom free for 6 months. In the follow-up period, he had two recurrences which responded to steroid therapy.
Conclusion
The common causes of PSP are demyelinating, vascular, and motor neuron diseases and congenital malformations of the opercular or insular cortex. However, there are no cases of PSP developing after any autoimmune encephalitis. This case highlights the importance of early detection of antiTPO antibodies with the findings of PSP due to autoimmune encephalitis.
Similar content being viewed by others
References
Brain L, Jellinek EH, Ball K (1966) Hashimoto’s disease and encephalopathy. Lancet 2(7462):512–514
Chong JY, Rowland LP, Utiger RD (2003) Hashimoto encephalopathy: syndrome or myth? Arch Neurol 60(2):164–171. https://doi.org/10.1001/archneur.60.2.164
Sharma PM, Javali M, Mahale R, Madhusudhan BK, Majeed AA, Srinivasa R (2015) Hashimoto encephalopathy: a study of the clinical profile, radiological and electrophysiological correlation in a tertiary care center in South India. J Neurosci Rural Pract 6(3):309–314. https://doi.org/10.4103/0976-3147.158753
Galluzzi S, Geroldi C, Zanetti O, Frisoni GB (2002) Hashimoto’s encephalopathy in the elderly: relationship to cognitive impairment. J Geriatr Psychiatry Neurol 15(3):175–179. https://doi.org/10.1177/089198870201500309
Montagna G, Imperiali M, Agazzi P, D’Aurizio F, Tozzoli R, Feldt-Rasmussen U, Giovanella L (2016) Hashimoto’s encephalopathy: a rare proteiform disorder. Autoimmun Rev 15(5):466–476. https://doi.org/10.1016/j.autrev.2016.01.014
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 (2006) Steroid responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 63(2):197–202. https://doi.org/10.1001/archneur.63.2.197
Doherty CP (2005) Possibly, probably definitely, Hashimoto encephalopathy. J Neurol Sci 228(1):1–2. https://doi.org/10.1016/j.jns.2004.09.025
Tamagno G, Federspil G, Murialdo G (2006) Clinical and diagnostic aspects of encephalopathy associated with autoimmune thyroid disease (or Hashimoto’s encephalopathy). Intern Emerg Med 1(1):15–23. https://doi.org/10.1007/BF02934715
Tamagno G, Celik Y, Simó R, Dihné M, Kimura K, Gelosa G, Lee BI, Hommet C, Murialdo G (2010) Encephalopathy associated with autoimmune thyroid disease in patients with Graves’ disease: clinical manifestations, follow-up, and outcomes. BMC Neurol 10(1):27. https://doi.org/10.1186/1471-2377-10-27
Chen N, Qin W, Wei C, Wang X, Li K (2011) Time course of Hashimoto’s encephalopathy revealed by MRI: report of two cases. J Neurol Sci 300(1–2):169–172. https://doi.org/10.1016/j.jns.2010.09.019
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 (2006) Steroidresponsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 63(2):197–202. https://doi.org/10.1001/archneur.63.2.197
Ferracci F, Bertiato G, Moretto G (2004) Hashimoto’s encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci 217(2):165–168. https://doi.org/10.1016/j.jns.2003.09.007
Mamoudjy N, Korff C, Maurey H, Blanchard G, Steshenko D, Loiseau-Corvez MN, Husson B, Brauner R, Tardieu M, Deiva K (2013) Hashimoto’s encephalopathy: identification and long-term outcome in children. Eur J Paediatr Neurol 17(3):280–287. https://doi.org/10.1016/j.ejpn.2012.11.003
Bohnen NI, Parnell KJ, Harper CM (1997) Reversible MRI findings in a patient with Hashimoto’s encephalopathy. Neurology 49(1):246–247. https://doi.org/10.1212/WNL.49.1.246
Miller A, Pratt H, Schiffer RB (2011) Pseudobulbar affect: the spectrum of clinical presentations, etiologies and treatments. Expert Rev Neurother 11(7):1077–1088. https://doi.org/10.1586/ern.11.68
Van der Poel JC, Haenggeli CA, Overweg-Plandsoen WCG (1995) Operculum syndrome: unusual feature of herpes simplex encephalitis. Pediatr Neurol 12(3):246–249. https://doi.org/10.1016/0887-8994(95)00005-Z
Mc Grath NM, Anderson NE, Hope JKA, Croxson MC, Powell KF (1997) Anterior opercular syndrome, caused by herpes simplex encephalitis. Neurology 49(2):494–497. https://doi.org/10.1212/WNL.49.2.494
Almekhlafi MA, Couillard PL, Patry DG, Jetté N (2010) Herpes encephalitis presenting with an opercular syndrome and epilepsia partialis continua. Neurologist 16(3):208–210. https://doi.org/10.1097/NRL.0b013e3181aa3791
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
Rights and permissions
About this article
Cite this article
Oz Tuncer, G., Teber, S., Kutluk, M.G. et al. Hashimoto’s encephalopathy presenting as pseudobulbar palsy. Childs Nerv Syst 34, 1251–1254 (2018). https://doi.org/10.1007/s00381-018-3720-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-018-3720-2