Abstract
Aim: Steroid responsive encephalopathy with autoimmune thyroiditis (SREAT) is a clinically and electrographically heterogeneous steroid-responsive encephalopathy associated with thyroid autoantibodies. We report an adolescent with SREAT and review PubMed literature relating to childhood.
Methods: A 14-year-old boy, without any preceding history of trauma, meningoencephalitis or seizures, was admitted in a comatose state. A similar episode of loss of consciousness 2 months prior with normal neuroimaging and electroencephalogram (EEG) had been followed by behavioral alterations. A year previously, during evaluation for increased appetite and poor weight gain, he was noted to have small goitre with thyroid-stimulating hormone (TSH) 7.26 mIU/L, T3 1.232 nmol/L, and T4 117.63 nmol/L. Routine hemogram, blood biochemistry, thyroid function tests including free hormone levels, ultrasonography thyroid and magnetic resonance imaging were normal. EEG showed diffuse slowing of all waves. Cerebrospinal fluid showed no pleocytosis and electrophoresis showed oligoclonal band. Viral studies and serum N-methyl-D-aspartate receptor antibody levels were negative. Anti-thyroid peroxidase (Anti-TPO) antibodies were raised. Intervention was with intravenous dexamethasone 4 mg every 6 h for 1 week followed by tapering schedule of oral prednisolone over 6 months.
Results: He regained consciousness after the second dose of dexamethasone and was discharged on day 7 in a fully conscious and ambulant state on a tapering course of low dose prednisolone for 6 months. He remains euthyroid with normal sensorium and behavior at 18 months follow-up. Only 50 cases below 18 years age were identified amongst 300 PubMed articles up to 31 July 2013.
Conclusion: Prompt steroid therapy following early recognition by high clinical suspicion and measurement of antithyroid antibody titers can lead to a favorable prognosis in SREAT.
References
1. Chong JY, Rowland LP. What’s in a NAIM? Hashimoto encephalopathy, steroid-responsive encephalopathy associated with autoimmune thyroiditis, or nonvasculitic autoimmune meningoencephalitis? Arch Neurol 2006;63:175–6.10.1001/archneur.63.2.175Search in Google Scholar
2. Zuliani L, Graus F, Giometto B, Bien C, Vincent A. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry 2012;83:638–45.10.1136/jnnp-2011-301237Search in Google Scholar
3. Brain L, Jellinek EH, Ball K. Hashimoto’s disease and encephalopathy. Lancet 1966;7462:512–4.10.1016/S0140-6736(66)92876-5Search in Google Scholar
4. de Holanda NC, de Lima DD, Cavalcanti TB, Lucena CS, Bandeira F. Hashimoto’s encephalopathy: systematic review of the literature and an additional case. J Neuropsychiatry Clin Neurosci 2011;23:384–90.10.1176/jnp.23.4.jnp384Search in Google Scholar PubMed
5. Dinkar K, Sinha S, Taly AB, Bindu PS, Bharath RD. Steroid-responsive encephalopathy in autoimmune thyroiditis: clinical spectrum and MRI observations in three cases. Ann Indian Acad Neurol 2011;14:205–7.10.4103/0972-2327.85897Search in Google Scholar PubMed PubMed Central
6. Ferracci F, Bertiato G, Moretto G. Hashimoto’s encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci 2004;217:165–8.10.1016/j.jns.2003.09.007Search in Google Scholar PubMed
7. Sawka AM, Fatourechi V, Boeve BF, Mokri B. Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996. Thyroid 2002;12:393–8.10.1089/105072502760043477Search in Google Scholar PubMed
8. Maydell BV, Kopp M, Komorski GV, Jeugling FD, Korinthenberg R. Hashimoto encephalopathy – is it underdiagnosed in pediatric patients? Neuropediatrics 2002;33:86–9.10.1055/s-2002-32364Search in Google Scholar PubMed
9. Castillo P, Woodruff B, Caselli R, Vernino S, Lucchinetti C, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 2006;63:197–202.10.1001/archneur.63.2.197Search in Google Scholar PubMed
10. Mahmud FH, Lteif AN, Renaud DL, Reed AM, Brands CK. Steroid-responsive encephalopathy associated with Hashimoto’s thyroiditis in an adolescent with chronic hallucinations and depression: case report and review. Pediatrics 2003;112: 686–90.10.1542/peds.112.3.686Search in Google Scholar PubMed
11. Boban M, Malojcic B. Rumination syndrome in a patient with Hashimoto’s encephalopathy. J Neuropsychiatry Clin Neurosci 2011;23:E11–2.10.1176/jnp.23.4.jnpe11Search in Google Scholar PubMed
12. Erol I, Saygi S, Alehan F. Hashimoto’s encephalopathy in children and adolescents. Pediatr Neurol 2011;45:420–2.10.1016/j.pediatrneurol.2011.09.010Search in Google Scholar PubMed
13. Tsai SL, Lewis EC, Sell E, Whiting S. Central nervous system vasculitis with positive antithyroid antibodies in an adolescent boy. Pediatr Neurol 2011;45:189–92.10.1016/j.pediatrneurol.2011.04.012Search in Google Scholar PubMed
14. Park J, Kim JG, Park SP, Lee HW. Asymmetric chorea as presenting symptom in Graves’ disease. Neurol Sci 2012;33:343–5.10.1007/s10072-011-0679-0Search in Google Scholar PubMed
15. Gayatri NA, Whitehouse WP. Pilot survey of Hashimoto’s encephalopathy in children. Dev Med Child Neurol 2005;47:556–8.10.1017/S0012162205001088Search in Google Scholar
16. Balestri P, Grosso S, Garibaldi G. Alternating hemiplegia of childhood or Hashimoto’s encephalopathy? J Neurol Neurosurg Psychiatry 1999;66:548–9.10.1136/jnnp.66.4.548Search in Google Scholar PubMed PubMed Central
17. Mamoudjy N, Korff C, Maurey H, Blanchard G, Steshenko D, et al. Hashimoto’s encephalopathy: identification and long-term outcome in children. Eur J Paediatr Neurol 2013;17:280–7.10.1016/j.ejpn.2012.11.003Search in Google Scholar PubMed
18. Mitchell RS, Yager JY, Marks SD. Childhood onset demyelination and Graves’ disease: shared antigen or autoimmune clustering? J Pediatr Endocrinol Metab 2007;20:1233–6.10.1515/JPEM.2007.20.11.1233Search in Google Scholar PubMed
19. Lopez-Giovaneli J, Moreaud O, Faure P, Debaty I, Chabre O, et al. Cortico-responsive encephalopathy associated with autoimmune thyroiditis (SREAT):about two case reports characterized by a gap between the diagnosis of autoimmune thyroiditis and neurological disorders. Ann Endocrinol (Paris) 2007;68:173–6.10.1016/j.ando.2007.05.001Search in Google Scholar PubMed
20. Matsunaga A, Yoneda M. Anti-NAE autoantibodies and clinical spectrum in Hashimoto’s encephalopathy. Rinsho Byori 2009;57:271–8.Search in Google Scholar
21. Takanashi J, Takahashi Y, Imamura A, Kodama K, Watanabe A, et al. Late delirious behavior with 2009 H1N1 influenza: mild autoimmune-mediated encephalitis? Pediatrics 2012;129:e1068–71.10.1542/peds.2010-3221Search in Google Scholar PubMed
22. Mahad DJ, Staugaitis S, Ruggieri P, Parisi J, Kleinschmidt-Demasters BK, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis and primary CNS demyelination. J Neurol Sci 2005;228:3–5.10.1016/j.jns.2004.08.015Search in Google Scholar PubMed
23. Santra G, De D, Phaujdar S, Rudra A, Dutta PS. Hashimoto’s encephalopathy. J Assoc Physicians India 2012;60:48–50.Search in Google Scholar
24. Kivity S, Agmon-Levin N, Zisappl M, Shapira Y, Nagy EV, et al. Vitamin D and autoimmune thyroid diseases. Cell Mol Immunol 2011;8:243–7.10.1038/cmi.2010.73Search in Google Scholar PubMed PubMed Central
25. Giovanella L, Pedrazzi P, Jandus P, Marone C. Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) with negative thyroperoxidase antibodies. Eur J Clin Invest 2008;38:693–4.10.1111/j.1365-2362.2008.01997.xSearch in Google Scholar PubMed
26. Rodriguez AJ, Jicha GA, Steeves TD, Benarroch EE, Westmoreland BF. EEG changes in a patient with steroid-responsive encephalopathy associated with antibodies to thyroperoxidase (SREAT, Hashimoto’s encephalopathy). J Clin Neurophysiol 2006;23:371–3.10.1097/01.wnp.0000214542.21735.49Search in Google Scholar PubMed
27. Grommes C, Griffin C, Downes KA, Lerner AJ. Steroid-responsive encephalopathy associated with autoimmune thyroiditis presenting with diffusion MR imaging changes. AJNR Am J Neuroradiol 2008;29:1550–1.10.3174/ajnr.A1113Search in Google Scholar PubMed PubMed Central
28. Mancardi MM, Fazzini F, Rossi A, Gaggero R. Hashimoto’s encephalopathy with selective involvement of the nucleus accumbens: a case report. Neuropediatrics 2005;36:218–20.10.1055/s-2005-865712Search in Google Scholar PubMed
29. Bektas Ö, Yılmaz A, Kendirli T, Sıklar Z, Deda G. Hashimoto encephalopathy causing drug-resistant status epilepticus treated with plasmapheresis. Pediatr Neurol 2012;46:132–5.10.1016/j.pediatrneurol.2011.11.009Search in Google Scholar PubMed
30. Olmez I, Moses H, Sriram S, Kirshner H, Lagrange AH, et al. Diagnostic and therapeutic aspects of Hashimoto’s encephalopathy. J Neurol Sci 2013;331:67–71.10.1016/j.jns.2013.05.009Search in Google Scholar PubMed
31. Brooks BL, Barlow KM. A methodology for assessing treatment response in Hashimoto’s encephalopathy: a case study demonstrating repeated computerized neuropsychological testing. J Child Neurol 2011;26:786–91.10.1177/0883073810391532Search in Google Scholar PubMed
©2014 by Walter de Gruyter Berlin/Boston
Articles in the same Issue
- Frontmatter
- Editorial
- Welcome to Assistant Editors at JPEM
- Review article
- Common approach to childhood obesity in Japan
- Images in pediatric endocrinology
- Aromatase deficiency in an adolescent girl misdiagnosed as congenital adrenal hyperplasia in infancy and childhood
- Original articles
- Prevalence of vitamin D deficiency among Iranian adolescents
- Analysis of body composition among children and adolescents – a cross-sectional study of the Polish population and comparison of body fat measurement methods
- Association of rs8066560 variant in the sterol regulatory element-binding protein 1 (SREBP-1) and miR-33b genes with hyperglycemia and insulin resistance
- Differences in anxiety and depression between male and female caregivers of children with a disorder of sex development
- Fasting and postprandial levels of a novel anorexigenic peptide nesfatin in childhood obesity
- The effect of growth hormone treatment on height in children with idiopathic short stature
- Clinical characteristics of type 1 diabetes over a 40 year period in Turkey: secular trend towards earlier age of onset
- Relationships of gamma-glutamyltransferase and beta 2-microglobulin on high sensitivity C-reactive protein among Japanese elementary school children
- Evaluation of insulin resistance and metabolic syndrome in a group of obese Czech children
- Circulating intact parathyroid hormone is suppressed at 25-hydroxyvitamin D concentrations >25 nmol/L in children
- Association between vitamin D level and cardiovascular risk in obese children and adolescents
- Serum paraoxonase activity and oxidative stress and their relationship with obesity-related metabolic syndrome and non-alcoholic fatty liver disease in obese children and adolescents
- Clinical features at diagnosis and responses to antithyroid drugs in younger children with Graves’ disease compared with adolescent patients
- Influence of hormonal parameters, bone mineral density and bone turnover on fracture risk in healthy male adolescents: a case control study
- Evaluation of lipid and glucose metabolism and cortisol and thyroid hormone levels in obese appropriate for gestational age (AGA) born and non-obese small for gestational age (SGA) born prepubertal Slovak children
- Chitotriosidase as a possible marker of clinically evidenced atherosclerosis in dyslipidemic children
- Exhaled carbon monoxide in adolescents with diabetic cardiovascular autonomic neuropathy
- Gut hormone profiles in preterm and term infants during the first 2 months of life
- Outcome analysis of aromatase inhibitor therapy to increase adult height in males with predicted short adult stature and/or rapid pubertal progress: a retrospective chart review
- A deletion including exon 2 of the TSHR gene is associated with thyroid dysgenesis and severe congenital hypothyroidism
- Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in childhood
- Patient Reports
- Neonatal diabetes in an infant of diabetic mother: same novel INS missense mutation in the mother and her offspring
- Atypical neurologic presentations of new onset type 1 diabetes mellitus in pediatric age group: a report of five unusual cases and review of the literature
- Importance of dietary calcium and vitamin D in the treatment of hypercalcaemia in Williams-Beuren syndrome
- Vitamin D intoxication in two brothers: be careful with dietary supplements
- Transient diabetes insipidus in a preterm neonate and the challenge of desmopressin dosing
- A rare case of hypoglycaemia due to insulinoma in an adolescent with acutely altered mental status
- Holoprosencephaly: ZIC2 mutation in a case with panhypopituitarism
- An association of hypochondroplasia and immune deficiency
- Hypophosphatemic rickets caused by a novel PHEX gene mutation in an Indian girl
- Psoriasis vulgaris and autoimmune polyendocrine syndrome type I: a case report
- Identification of a combined missense/splice-site mutation in FAH causing tyrosinemia type 1
- Letter to the Editor
- Vitamin D intake and premature infants with intraventricular hemorrhage: how advisable is it?
Articles in the same Issue
- Frontmatter
- Editorial
- Welcome to Assistant Editors at JPEM
- Review article
- Common approach to childhood obesity in Japan
- Images in pediatric endocrinology
- Aromatase deficiency in an adolescent girl misdiagnosed as congenital adrenal hyperplasia in infancy and childhood
- Original articles
- Prevalence of vitamin D deficiency among Iranian adolescents
- Analysis of body composition among children and adolescents – a cross-sectional study of the Polish population and comparison of body fat measurement methods
- Association of rs8066560 variant in the sterol regulatory element-binding protein 1 (SREBP-1) and miR-33b genes with hyperglycemia and insulin resistance
- Differences in anxiety and depression between male and female caregivers of children with a disorder of sex development
- Fasting and postprandial levels of a novel anorexigenic peptide nesfatin in childhood obesity
- The effect of growth hormone treatment on height in children with idiopathic short stature
- Clinical characteristics of type 1 diabetes over a 40 year period in Turkey: secular trend towards earlier age of onset
- Relationships of gamma-glutamyltransferase and beta 2-microglobulin on high sensitivity C-reactive protein among Japanese elementary school children
- Evaluation of insulin resistance and metabolic syndrome in a group of obese Czech children
- Circulating intact parathyroid hormone is suppressed at 25-hydroxyvitamin D concentrations >25 nmol/L in children
- Association between vitamin D level and cardiovascular risk in obese children and adolescents
- Serum paraoxonase activity and oxidative stress and their relationship with obesity-related metabolic syndrome and non-alcoholic fatty liver disease in obese children and adolescents
- Clinical features at diagnosis and responses to antithyroid drugs in younger children with Graves’ disease compared with adolescent patients
- Influence of hormonal parameters, bone mineral density and bone turnover on fracture risk in healthy male adolescents: a case control study
- Evaluation of lipid and glucose metabolism and cortisol and thyroid hormone levels in obese appropriate for gestational age (AGA) born and non-obese small for gestational age (SGA) born prepubertal Slovak children
- Chitotriosidase as a possible marker of clinically evidenced atherosclerosis in dyslipidemic children
- Exhaled carbon monoxide in adolescents with diabetic cardiovascular autonomic neuropathy
- Gut hormone profiles in preterm and term infants during the first 2 months of life
- Outcome analysis of aromatase inhibitor therapy to increase adult height in males with predicted short adult stature and/or rapid pubertal progress: a retrospective chart review
- A deletion including exon 2 of the TSHR gene is associated with thyroid dysgenesis and severe congenital hypothyroidism
- Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in childhood
- Patient Reports
- Neonatal diabetes in an infant of diabetic mother: same novel INS missense mutation in the mother and her offspring
- Atypical neurologic presentations of new onset type 1 diabetes mellitus in pediatric age group: a report of five unusual cases and review of the literature
- Importance of dietary calcium and vitamin D in the treatment of hypercalcaemia in Williams-Beuren syndrome
- Vitamin D intoxication in two brothers: be careful with dietary supplements
- Transient diabetes insipidus in a preterm neonate and the challenge of desmopressin dosing
- A rare case of hypoglycaemia due to insulinoma in an adolescent with acutely altered mental status
- Holoprosencephaly: ZIC2 mutation in a case with panhypopituitarism
- An association of hypochondroplasia and immune deficiency
- Hypophosphatemic rickets caused by a novel PHEX gene mutation in an Indian girl
- Psoriasis vulgaris and autoimmune polyendocrine syndrome type I: a case report
- Identification of a combined missense/splice-site mutation in FAH causing tyrosinemia type 1
- Letter to the Editor
- Vitamin D intake and premature infants with intraventricular hemorrhage: how advisable is it?