Hashimoto's encephalopathy presenting with unusual clinical findings in pediatric population: revision of the diagnostic criteria
Abstract
Aim: Hashimoto's encephalopathy is a rare progressive and relapsing disease of presumed
autoimmune origin associated with high titers of thyroid antibodies. Also named steroid-responsive
encephalopathy, the disease can be cured with early treatment. Hashimoto's encephalopathy may
occur with various clinical manifestations, but it often presents with symptoms such as seizures,
confusion, hallucination, sleep abnormalities, and behavioural problems. Patients are mostly euthyroid
or mildly hypothyroid. Hashimoto encephalopathy is a controversial diagnosis for misdiagnosis and
overdiagnosis because of the high prevalence of thyroid antibodies in the population. Therefore, this
diagnosis requires strict criteria that can highly indicate the diagnosis. We aimed to present the
diagnosis and treatment processes from unusual symptoms of patients who are considered to have
Hashimoto's encephalopathy with very strict criteria.
Materials and Methods: Here we present five pediatric patients diagnosed with Hashimoto's
encephalopathy between 2013 and 2023. The clinical signs and symptoms, laboratory findings,
treatment options and response to treatment were obtained from patient records retrospectively.
Results: On admission, patients had striking behavioural changes such as hallucinations, insomnia,
purposeless laughing as well as signs of encephalopathy such as status epilepticus and confusion;
neurological sequelae; history of surgery, and fever. All patients had high levels of thyroid antibodies
and they responded perfectly to steroid treatment in a short period of time.
Conclusion: Hashimoto’s encephalopathy should be considered in all patients presenting with
encephalopathy in the pediatric age group. These patients may have unusual and clinical
manifestations.
References
- Kothbauer-Margreiter I, Sturzenegger M, Komor J, et al. Encephalopathy associated with Hashimoto thyroiditis: diagnosis and treatment. J Neurol 1996;243:585–93.
- Tang Y, Xing Y, Lin MT, et al. Hashimoto's encephalopathy cases: The Chinese experience. BMC Neurol 2012;12:60.
- 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;41:228–33.
- Vasconcellos E, Piña-Garza JE, Fakhoury T, et al. Pediatric manifestations of Hashimoto's encephalopathy. Pediatr Neurol 1999;20(5):394–8.
- Brain L, Jellinek EH, Ball K. Hashimoto's disease and encephalopathy. Lancet 1966;2:512-4.
- Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol 2003;60:164–71.
- 7. Gayatri NA, Whitehouse WP. A pilot survey of Hashimoto's encephalopathy in children. Dev Med Child Neurol. 2005;47:556–8.
- Ferracci F, Bertiato G, Moretto G. Hashimoto's encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci. 2004;217:165–8.
- Gul Mert G, Horoz OO, Herguner MO, et al. Hashimoto's encephalopathy: four cases and review of the literature. Int J Neurosci. 2014;124:302–6.
- Kutluk MG, Haznedar P, Bektas O, et al. Hashimoto's encephalopathy in children: different manifestations of five cases. Acta Neurol Belg. 2019;119:595–9.
- Ferlazzo E, Raffaele M, Mazzù I, et al. Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy. Epilepsy Behav. 2006;8:328–30.
- Chiarello P, Talarico V, Nicoletti A, et al. Hashimoto encephalopathy: a case report and a short revision of current literature. Acta Biomed. 2020;91:e2020087.
- Ghika-Schmid F, Ghika J, Regli F, et al. Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition? Mov Disord. 1996;11:555–62.
- Sawka AM, Fatourechi V, Boeve BF, et al. Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996. Thyroid. 2002;12:393–8.
- Bohnen NI, Parnell KJ, Harper CM. Reversible MRI findings in a patient with Hashimoto's encephalopathy. Neurology. 1997;49:246–7.
- Schäuble B, Castillo PR, Boeve BF, et al. EEG findings in steroid-responsive encephalopathy associated with autoimmune thyroiditis. Clin Neurophysiol Off J Int Fed Clin Neurophysiol. 2003;114:32–7.
- Marshall GA, Doyle JJ. Long-term treatment of Hashimoto's encephalopathy. J Neuropsychiatry Clin Neurosci. 2006;18:14–20.
Pediyatrik popülasyonda alışılmamış klinik bulgularla bulunan Hashimoto ensefalopatisi: tanı kriterlerinin gözden geçirilmesi
Abstract
Amaç: Hashimoto ensefalopatisi, yüksek antikor titrelerinde tiroid antikorları ile ilişkili varsayılan
otoimmün kökenli, nadir görülen, ilerleyici ve tekrarlayan bir hastalıktır. Steroide duyarlı ensefalopati
olarak da adlandırılan bu hastalık, erken tedavi ile tedavi edilebilir.
Hashimoto ensefalopatisi çeşitli klinik belirtilerle ortaya çıkabilir, ancak çoğu zaman nöbetler,
konfüzyon, halüsinasyon, uyku anormallikleri ve davranış sorunları gibi semptomlarla da kendini
gösterir. Hastaların birçoğu ötiroid veya hafif hipotiroiddir. Genel popülasyonda tiroid antikorlarının
yüksek görülme oranları nedeniyle Hashimoto ensefalopatisi tanısı tartışmalıdır. Bu nedenle, oldukça
katı kriterler ile tanı koymak uygun olacaktır. Bu çalışmada, bu kriterler ile saptadığımız Hashimoto
ensefalopatili olgularımızdan olağandışı prezente olanların tanı ve tedavi süreçlerini sunmayı
amaçladık.
Yöntem: Bu çalışmada, 2013 ile 2023 yılları arasında Hashimoto ensefalopatisi tanısı konan beş
pediatrik hasta dikkate alındı. Klinik belirti ve bulguları, laboratuvar bulguları, tedavi seçenekleri ve
tedaviye yanıtları retrospektif olarak hasta kayıtlarından elde edildi.
Bulgular: Hastaların başvurularında halüsinasyonlar, uykusuzluk, amaçsız gülme gibi çarpıcı davranış
değişikliklerinin yanı sıra status epileptikus ve konfüzyon gibi ensefalopati bulgular gözlemlendi. Tüm
hastalarda yüksek tiroid antikorları seviyeleri gözlemlendi ve steroid tedavisine hastalardan kısa
sürede çok iyi yanıt alındığı görüldü.
Sonuç: Ensefalopati ile başvuran pediatrik yaş grubundaki tüm hastalarda Hashimoto ensefalopatisi
düşünülmelidir. Bu hastalarda alışılmadık, olağandışı ve klinik belirtileri olabilir.
References
- Kothbauer-Margreiter I, Sturzenegger M, Komor J, et al. Encephalopathy associated with Hashimoto thyroiditis: diagnosis and treatment. J Neurol 1996;243:585–93.
- Tang Y, Xing Y, Lin MT, et al. Hashimoto's encephalopathy cases: The Chinese experience. BMC Neurol 2012;12:60.
- 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;41:228–33.
- Vasconcellos E, Piña-Garza JE, Fakhoury T, et al. Pediatric manifestations of Hashimoto's encephalopathy. Pediatr Neurol 1999;20(5):394–8.
- Brain L, Jellinek EH, Ball K. Hashimoto's disease and encephalopathy. Lancet 1966;2:512-4.
- Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol 2003;60:164–71.
- 7. Gayatri NA, Whitehouse WP. A pilot survey of Hashimoto's encephalopathy in children. Dev Med Child Neurol. 2005;47:556–8.
- Ferracci F, Bertiato G, Moretto G. Hashimoto's encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci. 2004;217:165–8.
- Gul Mert G, Horoz OO, Herguner MO, et al. Hashimoto's encephalopathy: four cases and review of the literature. Int J Neurosci. 2014;124:302–6.
- Kutluk MG, Haznedar P, Bektas O, et al. Hashimoto's encephalopathy in children: different manifestations of five cases. Acta Neurol Belg. 2019;119:595–9.
- Ferlazzo E, Raffaele M, Mazzù I, et al. Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy. Epilepsy Behav. 2006;8:328–30.
- Chiarello P, Talarico V, Nicoletti A, et al. Hashimoto encephalopathy: a case report and a short revision of current literature. Acta Biomed. 2020;91:e2020087.
- Ghika-Schmid F, Ghika J, Regli F, et al. Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition? Mov Disord. 1996;11:555–62.
- Sawka AM, Fatourechi V, Boeve BF, et al. Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996. Thyroid. 2002;12:393–8.
- Bohnen NI, Parnell KJ, Harper CM. Reversible MRI findings in a patient with Hashimoto's encephalopathy. Neurology. 1997;49:246–7.
- Schäuble B, Castillo PR, Boeve BF, et al. EEG findings in steroid-responsive encephalopathy associated with autoimmune thyroiditis. Clin Neurophysiol Off J Int Fed Clin Neurophysiol. 2003;114:32–7.
- Marshall GA, Doyle JJ. Long-term treatment of Hashimoto's encephalopathy. J Neuropsychiatry Clin Neurosci. 2006;18:14–20.
Details
| Primary Language | English |
|---|---|
| Subjects | Pediatric Neurology |
| Journal Section | Research Articles |
| Authors | |
| Publication Date | September 18, 2023 |
| Submission Date | February 15, 2023 |
| Published in Issue | Year 2023 |