Clinical features of children with acute disseminated encephalomyelitis and related recurrence factors
RUAN Jin, CHENG Min, LI Xiu-Juan
Department of Neurology, Children's Hospital of Chongqing Medical University/Ministry of Education Key Laboratory of Child Development and Disorders/China International Science and Technology Cooperation Base of Child Development and Critical Disorders/Chongqing Key Laboratory of Pediatrics, Chongqing 400014, China
Abstract Objective To study the clinical features of children with acute disseminated encephalomyelitis (ADEM) and related recurrence factors. Methods A retrospective analysis was performed for the clinical data and prognosis of 73 children with ADEM who were hospitalized from November 2011 to January 2017. Results Among the 73 children, 41 (56%) had a history of infection before onset and 7 (10%) had a history of vaccination. All children had the symptoms of encephalopathy, including disturbance of consciousness in 47 children (64%) and mental and behavioral disorders in 54 children (74%). Pyrexia was observed in 53 children (73%), dyskinesia in 47 children (64%), headache in 47 children (64%) and vomiting in 40 children (55%). Brain MRI was performed for 65 children and the results showed involvement of the subcortical white matter (83%, 54/65), the deep nuclei (60%, 39/65), the brain stem (58%, 38/65) and the cerebellum (42%, 27/65). Spinal cord involvement was observed in 20 children (20/43, 47%). A total of 15 children experienced recurrence during follow-up. Compared with the non-recurrence group, the recurrence group had signifcantly higher percentages of children with deep nucleus involvement (P < 0.05), with injury in ≥ 3 spinal segments (P < 0.01) and with a time from disease onset to gamma-globulin/hormone treatment of >2 weeks (P < 0.05). Conclusions ADEM in children have various clinical manifestations. A small number of children may experience recurrence. Deep nucleus involvement on MRI, long spinal segmental injury (≥ 3 segments) and late treatment with gamma-globulin/hormone (>2 weeks) may be associated with the recurrence of ADEM.
RUAN Jin,CHENG Min,LI Xiu-Juan. Clinical features of children with acute disseminated encephalomyelitis and related recurrence factors[J]. CJCP, 2019, 21(3): 223-228.
RUAN Jin,CHENG Min,LI Xiu-Juan. Clinical features of children with acute disseminated encephalomyelitis and related recurrence factors[J]. CJCP, 2019, 21(3): 223-228.
Suppiej A, Vittorini R, Fontanin M, et al. Acute disseminated encephalomyelitis in children:focus on relapsing patients[J]. Pediatr Neurol, 2008, 39(1):12-17.
[2]
Krupp LB, Tardieu M, Amato MP, et al. International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders:revisions to the 2007 definitions[J]. Mult Scler, 2013, 19(10):1261-1267.
[3]
Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders[J]. Neurology, 2015, 85(2):177-189.
[4]
Xiong CH, Yan Y, Liao Z, et al. Epidemiological characteristics of acute disseminated encephalomyelitis in Nanchang, China:a retrospective study[J]. BMC Public Health, 2014, 14:111.
[5]
Giri PP, Bhattyacharya S, Das D, et al. Acute disseminated encephalomyelitis:a clinical and neuroradiological profile of pediatric patients[J]. Neurol India, 2016, 64(6):1187-1192.
[6]
Pohl D, Alper G, Van Haren K, et al. Acute disseminated encephalomyelitis:updates on an inflammatory CNS syndrome[J]. Neurology, 2016, 87(9 Suppl 2):S38-S45.
[7]
Wender M. Acute disseminated encephalomyelitis (ADEM)[J]. J Neuroimmunol, 2011, 231(1-2):92-99.
[8]
Pohl D. Epidemiology, immunopathogenesis and management of pediatric central nervous system inflammatory demyelinating conditions[J]. Curr Opin Neurol, 2008, 21(3):366-372.
[9]
Karussis D, Petrou P. The spectrum of post-vaccination inflammatory CNS demyelinating syndromes[J]. Autoimmun Rev, 2014, 13(3):215-224.
[10]
Young NP, Weinshenker BG, Lucchinetti CF. Acute disseminated encephalomyelitis:current understanding and controversies[J]. Semin Neurol, 2008, 28(1):84-94.
[11]
Steiner I, Kennedy PG. Acute disseminated encephalomyelitis:current knowledge and open questions[J]. J Neurovirol, 2015, 21(5):473-479.
[12]
Koelman DL, Chahin S, Mar SS, et al. Acute disseminated encephalomyelitis in 228 patients:a retrospective, multicenter US study[J]. Neurology, 2016, 86(22):2085-2093.
[13]
Hardy TA. How should we diagnose acute disseminated encephalomyelitis?[J]. Dev Med Child Neurol, 2018, 60(11):1070.
[14]
Boesen MS, Blinkenberg M, Koch-Henriksen N, et al. Implications of the International Paediatric Multiple Sclerosis Study Group consensus criteria for paediatric acute disseminated encephalomyelitis:a nationwide validation study[J]. Dev Med Child Neurol, 2018, 60(11):1123-1131.
[15]
Javed A, Khan O. Acute disseminated encephalomyelitis[J]. Handb Clin Neurol, 2014, 123:705-717.
[16]
Mikaeloff Y, Adamsbaum C, Husson B, et al. MRI prognostic factors for relapse after acute CNS inflammatory demyelination in childhood[J]. Brain, 2004, 127(Pt 9):1942-1947.
Kariyawasam S, Singh RR, Gadian J, et al. Clinical and radiological features of recurrent demyelination following acute disseminated encephalomyelitis (ADEM)[J]. Mult Scler Relat Disord, 2015, 4(5):451-456.
[19]
Verhey LH, Branson HM, Shroff MM, et al. MRI parameters for prediction of multiple sclerosis diagnosis in children with acute CNS demyelination:a prospective national cohort study[J]. Lancet Neurol, 2011, 10(12):1065-1073.
[20]
Duignan S, Wright S, Rossor T, et al. Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specifc in children with acquired demyelinating syndromes[J]. Dev Med Child Neurol, 2018, 60(9):958-962.
