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Journal of Pediatric Neurology 2023; 21(06): 428-433
DOI: 10.1055/s-0043-1761485
Original Article

Determination of Clinical, Electrophysiological, and Radiological Characteristics of Pediatric Autoimmune Encephalopathy

Mesut Güngör 
1   Faculty of Medicine, Department of Child Neurology, Selçuk University, Konya, Türkiye
,
Merve Öztürk 
2   Department of Child Neurology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye
,
Adnan Deniz 
2   Department of Child Neurology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye
,
Defne Alikılıç 
2   Department of Child Neurology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye
,
Ömer Karaca 
2   Department of Child Neurology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye
,
Yonca Anık 
3   Department of Radiology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye
,
Bülent Kara 
2   Department of Child Neurology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye
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Abstract

Autoimmune encephalopathy (AE) is a group of diseases with subacute onset, that represents a wide clinical spectrum, manifested by complex neuropsychiatric symptoms and signs. In this study, the data of 27 patients diagnosed and followed up in our clinic with the diagnosis of AE between 2011 and 2021 were evaluated retrospectively. Out of 27 patients, 6 were definite seropositive AE, 2 of them met the diagnostic criteria for limbic encephalitis, and the remaining 19 were probable AE. Nowadays, we see AEs with increasing frequency. While there is a generally established approach in the diagnosis and treatment of seropositive patients, there are still hesitations and diagnostic difficulties in seronegative AEs. In this study, clinical, radiological, and prognostic features of definite and probable AE patients diagnosed in a tertiary pediatric neurology clinic were documented. It is thought that pediatric neurologists have an important responsibility to increase awareness about AE in pediatricians. In the future, it is predicted that AE will be diagnosed more frequently with new antibodies and one has to differentiate it from viral encephalitis and neuropsychiatric syndromes and diseases.

Keywords

autoimmune encephalopathy - limbic encephalitis - anti-NMDA antibody

Ethics Committee Approval

Approval for this study was obtained from the Local Ethics Committee of Kocaeli University Faculty of Medicine.




Publication History

Received: 13 September 2022

Accepted: 02 January 2023

Article published online:
07 February 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Graus F, Titulaer MJ, Balu R. et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016; 15 (04) 391-404
    CrossrefPubMedGoogle Scholar
  • 2 Armangue T, Petit-Pedrol M, Dalmau J. Autoimmune encephalitis in children. J Child Neurol 2012; 27 (11) 1460-1469
    CrossrefPubMedGoogle Scholar
  • 3 de Bruijn MAAM, Bruijstens AL, Bastiaansen AEM. et al; CHANCE Study Group. Pediatric autoimmune encephalitis: recognition and diagnosis. Neurol Neuroimmunol Neuroinflamm 2020; 7 (03) e682
    CrossrefPubMedGoogle Scholar
  • 4 Gadian J, Eyre M, Konstantoulaki E. et al. Neurological and cognitive outcomes after antibody-negative autoimmune encephalitis in children. Dev Med Child Neurol 2022; 64 (05) 649-653
    CrossrefPubMedGoogle Scholar
  • 5 Brenton JN, Goodkin HP. Antibody-mediated autoimmune encephalitis in childhood. Pediatr Neurol 2016; 60: 13-23
    CrossrefPubMedGoogle Scholar
  • 6 Hacohen Y, Wright S, Waters P. et al. Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens. J Neurol Neurosurg Psychiatry 2013; 84 (07) 748-755
    CrossrefPubMedGoogle Scholar
  • 7 Dalmau J, Gleichman AJ, Hughes EG. et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008; 7 (12) 1091-1098
    CrossrefPubMedGoogle Scholar
  • 8 Shepherd R, Cheung AS, Pang K, Saffery R, Novakovic B. Sexual dimorphism in innate immunity: the role of sex hormones and epigenetics. Front Immunol 2021; 11: 604000
    CrossrefPubMedGoogle Scholar
  • 9 Zhang J, Ji T, Chen Q. et al. Pediatric autoimmune encephalitis: case series from two Chinese tertiary pediatric neurology centers. Front Neurol 2019; 10: 906
    CrossrefPubMedGoogle Scholar
  • 10 Basheer S, Nagappa M, Mahadevan A, Bindu PS, Taly AB, Girimaji SC. Neuropsychiatric manifestations of pediatric NMDA receptor autoimmune encephalitis: a case series from a tertiary care center in India. Prim Care Companion CNS Disord 2017; 19 (04) 17
    CrossrefPubMedGoogle Scholar
  • 11 Incecik F, Herguner OM, Besen S, Yılmaz M. Autoimmune encephalitis associated with glutamic acid decarboxylase antibodies: a case series. Acta Neurol Belg 2018; 118 (03) 411-414
    CrossrefPubMedGoogle Scholar
  • 12 Ren C, Ren H, Ren X. et al. Case report: autoimmune encephalitis associated with anti-glutamic acid decarboxylase antibodies: a pediatric case series. Front Neurol 2021; 12: 641024
    CrossrefPubMedGoogle Scholar
  • 13 Mattozzi S, Sabater L, Escudero D. et al. Hashimoto encephalopathy in the 21st century. Neurology 2020; 94 (02) e217-e224
    CrossrefPubMedGoogle Scholar
  • 14 Dalmau J, Armangué T, Planagumà J. et al. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. Lancet Neurol 2019; 18 (11) 1045-1057
    CrossrefPubMedGoogle Scholar
  • 15 Armangue T, Spatola M, Vlagea A. et al; Spanish Herpes Simplex Encephalitis Study Group. Frequency, symptoms, risk factors, and outcomes of autoimmune encephalitis after herpes simplex encephalitis: a prospective observational study and retrospective analysis. Lancet Neurol 2018; 17 (09) 760-772
    CrossrefPubMedGoogle Scholar
  • 16 Suleiman J, Dale RC. The recognition and treatment of autoimmune epilepsy in children. Dev Med Child Neurol 2015; 57 (05) 431-440
    CrossrefPubMedGoogle Scholar
  • 17 Moise AM, Karakis I, Herlopian A. et al. Continuous EEG findings in autoimmune encephalitis. J Clin Neurophysiol 2021; 38 (02) 124-129
    CrossrefPubMedGoogle Scholar
  • 18 Garg D, Mohammad SS, Sharma S. Autoimmune encephalitis in children: an update. Indian Pediatr 2020; 57 (07) 662-670
    CrossrefPubMedGoogle Scholar
  • 19 Aneja S, Sharma S. Diagnosis and management of acute encephalitis in children. Indian J Pediatr 2019; 86 (01) 70-75
    CrossrefPubMedGoogle Scholar
  • 20 Etemadifar M, Ashourizadeh H, Nouri H. et al. MRI signs of CNS demyelinating diseases. Mult Scler Relat Disord 2021; 47: 102665
    CrossrefPubMedGoogle Scholar
  • 21 Rutatangwa A, Mittal N, Francisco C, Nash K, Waubant E. Autoimmune encephalitis in children: a case series at a tertiary care center. J Child Neurol 2020; 35 (09) 591-599
    CrossrefPubMedGoogle Scholar
  • 22 Abboud H, Probasco JC, Irani S. et al; Autoimmune Encephalitis Alliance Clinicians Network. Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry 2021; 92 (07) 757-768
    CrossrefPubMedGoogle Scholar
  • 23 Zuliani L, Nosadini M, Gastaldi M. et al. Management of antibody-mediated autoimmune encephalitis in adults and children: literature review and consensus-based practical recommendations. Neurol Sci 2019; 40 (10) 2017-2030
    CrossrefPubMedGoogle Scholar
  • 24 Dale RC, Brilot F, Duffy LV. et al. Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease. Neurology 2014; 83 (02) 142-150
    CrossrefPubMedGoogle Scholar
  • 25 Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis 2012; 54 (07) 899-904
    CrossrefPubMedGoogle Scholar