Understanding Childhood Neuroimmune Diseases of the Central Nervous System.
Rasmussen encephalitis
acquired demyelinating syndromes
autoimmune encephalitis
children
hemophagocytic lymphohistiocytosis
immunogenetic diseases
interferonopathies
neuroimmune diseases
Journal
Frontiers in pediatrics
ISSN: 2296-2360
Titre abrégé: Front Pediatr
Pays: Switzerland
ID NLM: 101615492
Informations de publication
Date de publication:
2019
2019
Historique:
received:
28
05
2019
accepted:
25
11
2019
entrez:
11
1
2020
pubmed:
11
1
2020
medline:
11
1
2020
Statut:
epublish
Résumé
Immune-mediated diseases of the central nervous system (CNS) in childhood are a heterogeneous group of rare conditions sharing the inflammatory involvement of the CNS. This review highlights the growing knowledge of childhood neuroimmune diseases that primarily affect the CNS, outlining the clinical and diagnostic features, the pathobiological mechanisms and genetics, current treatment options, and emerging challenges. The clinical spectrum of these conditions is increasingly expanded, and the underlying mechanisms of dysregulation of the immune system could vary widely. Cell-mediated and antibody-mediated disorders, infection-triggered and paraneoplastic conditions, and genetically defined mechanisms can occur in previously healthy children and can contribute to different stages of the disease. The careful evaluation of the clinical presentation and temporal course of symptoms, the specific neuroimaging and immunological findings, and the exclusion of alternative causes are mandatory in clinical practice for the syndromic diagnosis. A common feature of these conditions is that immunotherapeutic agents could modulate the clinical course and outcomes of the disease. Furthermore, specific symptomatic treatments and comprehensive multidisciplinary care are needed in the overall management. We focus on recent advances on immune-mediated demyelinating CNS disorders, autoimmune encephalitis, interferonopathies, and possible neuroimmune disorders as Rasmussen encephalitis. Better knowledge of these conditions could allow prompt diagnosis and targeted immunotherapy, to decrease morbidity and mortality as well as to improve clinical outcomes, reducing the burden of the disease due to possible long-term neuropsychiatric sequelae. Persisting controversies remain in the rigorous characterization of each specific clinical entity because of the relative rarity in children; moreover, in a large proportion of suspected neuroimmune diseases, the immune "signature" remains unidentified; treatment guidelines are mostly based on retrospective cohort studies and expert opinions; then advances in specific molecular therapies are required. In the future, a better characterization of specific immunological biomarkers may provide a useful understanding of the underlying pathobiological mechanisms of these conditions in order to individualize more tailored therapeutic options and paradigms. Multicenter collaborative research on homogeneous groups of patients who may undergo immunological studies and therapeutic trials could improve the characterization of the underlying mechanisms, the specific phenotypes, and tailored management.
Identifiants
pubmed: 31921724
doi: 10.3389/fped.2019.00511
pmc: PMC6930888
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
511Informations de copyright
Copyright © 2019 Matricardi, Farello, Savasta and Verrotti.
Références
Lancet Neurol. 2013 Feb;12(2):157-65
pubmed: 23290630
Neurology. 2013 Sep 3;81(10):882-7
pubmed: 23925760
Lancet Neurol. 2011 May;10(5):436-45
pubmed: 21459044
Neurology. 2019 Jan 1;92(1):26-33
pubmed: 30381369
Neurotherapeutics. 2016 Oct;13(4):824-832
pubmed: 27215218
Eur J Paediatr Neurol. 2018 Mar;22(2):301-307
pubmed: 29396169
Arch Dis Child. 2011 Feb;96(2):186-91
pubmed: 20959359
Curr Treat Options Neurol. 2012 Jun;14(3):264-75
pubmed: 22476745
Brain. 2014 Aug;137(Pt 8):2178-92
pubmed: 24951641
Seizure. 2015 Apr;27:16-8
pubmed: 25891922
Eur J Neurol. 2018 Dec;25(12):e125-e126
pubmed: 30403325
Neurology. 2016 Aug 30;87(9 Suppl 2):S110-6
pubmed: 27572855
Mult Scler. 2011 Oct;17(10):1258-61
pubmed: 20876155
Brain. 2010 Feb;133(Pt 2):333-48
pubmed: 20129932
J Neurol. 2016 Apr;263(4):765-71
pubmed: 26886205
Neurology. 2008 Jan 29;70(5):374-7
pubmed: 18227418
Pediatr Neurol. 2009 Jan;40(1):31-3
pubmed: 19068251
Dev Med Child Neurol. 2015 Jan;57(1):95-9
pubmed: 25040285
Dev Med Child Neurol. 2019 Sep;61(9):1101-1107
pubmed: 31175679
Neurology. 2016 Aug 30;87(9 Suppl 2):S74-81
pubmed: 27572865
Epilepsy Res. 2010 Mar;89(1):104-12
pubmed: 19883995
Mult Scler. 2013 Nov;19(13):1749-59
pubmed: 23579092
Neurology. 2016 Aug 30;87(9 Suppl 2):S38-45
pubmed: 27572859
Neurol Neuroimmunol Neuroinflamm. 2015 Sep 24;2(5):e150
pubmed: 26445726
Ann Neurol. 2011 May;69(5):892-900
pubmed: 21416487
Epilepsia. 2012 Sep;53(9):1539-45
pubmed: 22738156
Mult Scler Relat Disord. 2018 Apr;21:84-87
pubmed: 29499442
N Engl J Med. 2007 Jun 21;356(25):2603-13
pubmed: 17582070
Epidemiology. 2014 Nov;25(6):928-9
pubmed: 25265138
Expert Rev Neurother. 2016 Aug;16(8):937-49
pubmed: 27171736
Pediatr Neurol. 2016 Jul;60:13-23
pubmed: 27343023
Ann Neurol. 2009 Jul;66(1):11-8
pubmed: 19670433
J Neurol Neurosurg Psychiatry. 2018 Feb;89(2):138-146
pubmed: 28951498
Epilepsia. 1999 Feb;40(2):242-7
pubmed: 9952274
Epilepsy Res. 2014 Aug;108(6):1106-19
pubmed: 24815913
Mult Scler Int. 2013;2013:398259
pubmed: 23431447
J Neurol Neurosurg Psychiatry. 2015 Mar;86(3):265-72
pubmed: 25121570
Neuropediatrics. 2014 Oct;45(5):336-40
pubmed: 25062271
J Pediatr. 2013 Apr;162(4):850-856.e2
pubmed: 23164315
Neurology. 2015 Mar 24;84(12):1233-41
pubmed: 25636713
Dev Med Child Neurol. 2011 Nov;53(11):1058-60
pubmed: 21883173
Ann Neurol. 2018 Jan;83(1):40-51
pubmed: 29244234
J Neurol Neurosurg Psychiatry. 2015 Jan;86(1):20-5
pubmed: 25248365
Lancet Neurol. 2014 Sep;13(9):936-48
pubmed: 25142460
J Neuroimmunol. 2018 Aug 15;321:157-163
pubmed: 29793728
Ann Neurol. 2009 Dec;66(6):833-42
pubmed: 20033986
Biochem Pharmacol. 2018 Nov;157:51-66
pubmed: 30171835
Dev Med Child Neurol. 2016 Apr;58(4):376-84
pubmed: 26330176
Lancet Neurol. 2018 Feb;17(2):162-173
pubmed: 29275977
Epilepsy Behav. 2015 May;46:249-51
pubmed: 25960424
Expert Rev Neurother. 2015;15(12):1391-419
pubmed: 26559389
Pediatrics. 2007 Sep;120(3):e622-8
pubmed: 17698967
Neurology. 2015 Jul 14;85(2):177-89
pubmed: 26092914
Curr Rheumatol Rep. 2018 Nov 8;20(12):83
pubmed: 30406861
Hematology Am Soc Hematol Educ Program. 2013;2013:605-11
pubmed: 24319239
Mult Scler. 2013 Jan;19(1):76-86
pubmed: 22516794
Neurology. 2012 Apr 10;78(15):1150-6
pubmed: 22422896
JAMA Neurol. 2016 Oct 1;73(10):1251-1253
pubmed: 27533122
Ann Neurol. 2011 Feb;69(2):292-302
pubmed: 21387374
Eur J Pediatr. 2007 May;166(5):405-12
pubmed: 17219129
Expert Opin Drug Metab Toxicol. 2019 Mar;15(3):199-211
pubmed: 30689454
J Clin Exp Neuropsychol. 2018 Apr;40(3):234-252
pubmed: 28585453
Nat Rev Neurol. 2017 Sep;13(9):567-572
pubmed: 28799551
Neurology. 2017 Mar 14;88(11):1012-1020
pubmed: 28202703
Neurology. 2003 Dec 23;61(12):1807-10
pubmed: 14694056
Handb Clin Neurol. 2013;111:511-9
pubmed: 23622199
Neurol Neuroimmunol Neuroinflamm. 2015 Mar 12;2(2):e81
pubmed: 25798445
Mult Scler. 2012 Jan;18(1):45-54
pubmed: 21865412
Br J Haematol. 2008 Feb;140(3):327-35
pubmed: 18076710
Neurology. 1958 Jun;8(6):435-45
pubmed: 13566382
Neurology. 2001 Jan 23;56(2):248-50
pubmed: 11160964
Epilepsy Behav. 2015 Dec;53:51-7
pubmed: 26519666
Arch Iran Med. 2012 Jun;15(6):381-3
pubmed: 22642251
J Neuroimmunol. 2019 Oct 15;335:577008
pubmed: 31352183
Neurology. 2016 Aug 30;87(9 Suppl 2):S28-37
pubmed: 27572858
Mult Scler. 2011 Apr;17(4):441-8
pubmed: 21148017
Lancet Neurol. 2011 Jan;10(1):63-74
pubmed: 21163445
Neurology. 2009 Jan 20;72(3):232-9
pubmed: 19153370
Neurology. 2016 Aug 30;87(9 Suppl 2):S97-S102
pubmed: 27572869
Ann Neurol. 2007 Jan;61(1):25-36
pubmed: 17262855
Curr Opin Neurol. 2002 Apr;15(2):197-200
pubmed: 11923635
JAMA Neurol. 2016 Nov 1;73(11):1297-1307
pubmed: 27618707
Lancet Neurol. 2010 Jan;9(1):67-76
pubmed: 19962348
Autoimmun Rev. 2014 Mar;13(3):215-24
pubmed: 24514081
Eur J Paediatr Neurol. 2017 Mar;21(2):414-417
pubmed: 27653852
Ann N Y Acad Sci. 2015 Mar;1338:94-114
pubmed: 25315420
Eur J Paediatr Neurol. 2018 Jan;22(1):182-185
pubmed: 29203057
Lancet Neurol. 2007 Sep;6(9):805-15
pubmed: 17706564
Eur J Neurol. 2014 Apr;21(4):654-9
pubmed: 24471864
Expert Opin Pharmacother. 2017 Oct;18(14):1491-1498
pubmed: 28847180
Neurology. 2007 Apr 17;68(16 Suppl 2):S23-36
pubmed: 17438235
Pharmacol Res. 2016 May;107:426-429
pubmed: 27080588
Eur J Paediatr Neurol. 2018 Jan;22(1):190-193
pubmed: 28919330
Neurology. 2011 Apr 5;76(14):1252-5
pubmed: 21464429
Acta Neurol Scand. 2019 Sep;140(3):184-193
pubmed: 31102535
Autoimmun Rev. 2017 Aug;16(8):803-810
pubmed: 28572051
Neurology. 2002 Oct 8;59(7):1006-10
pubmed: 12370453
Epilepsia. 2014 May;55(5):e38-43
pubmed: 24702622
Lancet Neurol. 2014 Mar;13(3):276-86
pubmed: 24462240
Neurol Sci. 2014 Jul;35(7):983-93
pubmed: 24619070
Neurology. 2002 Aug 27;59(4):499-505
pubmed: 12236201
Lancet Neurol. 2016 Apr;15(4):391-404
pubmed: 26906964
J Clin Pathol. 2017 Jan;70(1):1-8
pubmed: 27646526
Epilepsia. 2006;47 Suppl 5:41-6
pubmed: 17239105
Best Pract Res Clin Rheumatol. 2017 Aug;31(4):441-459
pubmed: 29773266
Neurology. 2017 Jul 18;89(3):269-278
pubmed: 28615429
Mult Scler. 2013 Sep;19(10):1320-2
pubmed: 23413296
Ann Neurol. 2007 Jul;62(1):67-80
pubmed: 17503512
Neurology. 2011 Sep 20;77(12):1143-8
pubmed: 21865580
JAMA Neurol. 2013 Apr;70(4):498-501
pubmed: 23380884
Neuroimmunomodulation. 2017;24(2):113-119
pubmed: 28922662
Expert Opin Pharmacother. 2017 Jun;18(8):789-798
pubmed: 28481700
Epilepsia. 2016 Jun;57(6):956-66
pubmed: 27106864
Lancet Neurol. 2016 Aug;15(9):967-981
pubmed: 27478954
Neurology. 2016 Aug 30;87(9 Suppl 2):S53-8
pubmed: 27572862
Pediatr Neurol. 2014 Mar;50(3):205-12
pubmed: 24530152
Nat Rev Neurol. 2018 Jul;14(7):433-445
pubmed: 29925924
Neuroradiology. 2003 Mar;45(3):171-83
pubmed: 12684722
Neurology. 2013 Oct 22;81(17):1500-6
pubmed: 24068784
Curr Opin Neurol. 2017 Jun;30(3):334-344
pubmed: 28234797
Curr Neuropharmacol. 2010 Jun;8(2):135-48
pubmed: 21119885
Neurology. 2007 Apr 17;68(16 Suppl 2):S7-12
pubmed: 17438241
Mult Scler. 2013 Sep;19(10):1261-7
pubmed: 23572237
Ann Clin Transl Neurol. 2014 Mar 1;1(3):180-189
pubmed: 24707504
Pediatr Neurosurg. 1999 May;30(5):278-82
pubmed: 10461077
J Clin Invest. 2018 Jul 2;128(7):2760-2762
pubmed: 29889100
Brain. 2005 Mar;128(Pt 3):454-71
pubmed: 15689357
Brain. 2012 Nov;135(Pt 11):3453-68
pubmed: 23065479
Neurology. 2012 Sep 11;79(11):1094-100
pubmed: 22933737
Clin Infect Dis. 2012 Apr;54(7):899-904
pubmed: 22281844
J Clin Neurosci. 2016 Jun;28:175-8
pubmed: 26775150
Neurology. 2013 Jul 23;81(4):395-7
pubmed: 23794679
Front Immunol. 2016 Feb 23;7:64
pubmed: 26941743
Nat Rev Neurol. 2009 Aug;5(8):458-62
pubmed: 19657347
Pediatr Neurol. 2011 Apr;44(4):275-81
pubmed: 21397169
J Neurol. 2012 Sep;259(9):1929-35
pubmed: 22349866
Neurology. 2015 Sep 15;85(11):967-75
pubmed: 26296514
Lancet Neurol. 2008 Dec;7(12):1091-8
pubmed: 18851928
Pediatr Neurol. 2014 Apr;50(4):363-7
pubmed: 24630282
Seizure. 2017 Jan;44:74-80
pubmed: 28029552
Epilepsia. 2012 Apr;53(4):613-21
pubmed: 22309137
N Engl J Med. 2018 Mar 1;378(9):840-851
pubmed: 29490181
Expert Rev Clin Immunol. 2017 Oct;13(10):975-989
pubmed: 28738749
Dev Med Child Neurol. 2017 Aug;59(8):796-805
pubmed: 28439890
Neurology. 2014 Feb 11;82(6):474-81
pubmed: 24415568
Epilepsia. 2014 Aug;55 Suppl 3:29-33
pubmed: 25209083
Lancet Infect Dis. 2010 Dec;10(12):835-44
pubmed: 20952256
Epilepsia. 2004 May;45(5):516-26
pubmed: 15101833
Ann Neurol. 2018 Jan;83(1):166-177
pubmed: 29293273
Neurology. 2003 Feb 11;60(3):422-5
pubmed: 12578922
Ann Neurol. 2018 Sep;84(3):473-480
pubmed: 30076629
Lancet Neurol. 2014 Feb;13(2):167-77
pubmed: 24360484
Neurology. 2016 Aug 30;87(9 Suppl 2):S46-52
pubmed: 27572861
Paediatr Drugs. 2018 Oct;20(5):429-453
pubmed: 30003498
Pediatr Neurol. 2017 Jan;66:104-107
pubmed: 27913085
Lancet Neurol. 2013 Dec;12(12):1159-69
pubmed: 24183309