A retrospective study on acquired demyelinating diseases in children
Keywords:Pediatric demyelinating disorders, ADEM, Transverse myelitis, MOG
Background: Acquired demyelinating diseases (ADS) соnstitute а heterogeneous grоuр of central nervous system disorders of autoimmune origin that causes significant physical and cognitive disabilities. Early recognition and prompt management causes significant improvement in acute episodes of demyelinating disorders.
Methods: 33 children diagnosed with demyelinating disorders at Lokmanya Tilal municipal medical college and hospital in Mumbai were enrolled. The study was conducted between January 2013 and November 2022. Demographic data, clinical profile, CSF study, serum antibody, radiological findings were collected and results were analyzed. Statistical Data was analysed using statistical software GraphPad in Stat.V3.0. Data were presented in tables and figures whenever needed. P value <0.05 considered as significant.
Results: Of 33 patients, 21 (63%) were cases of acute disseminated encephalomyelitis (ADEM), 6 (18.1%) of transverse myelitis (TM), 1 case of ADEM + TM (3%), 3 (9%) of neuromyelitis optica, 1 (3%) of Optic neuritis and 1 (3%) of multiple sclerosis. ADEM patients presented with encephalopathy and multifocal neurological deficits, 40% were MOG positive. Two patients were of multiphasic ADEM. Patients of transverse myelitis had paraparesis or quadriparesis and sensory + bladder involvement. Patients with NMO presented with bilateral visual impairment with limb weakness and bladder involvement. Steroids were the primary treatment, 3 patients (9%) required intravenous immunoglobulin (IVIG) and 1 (3%) patient received plasma exchange therapy.
Conclusions: ADEM is the most common ADS. Early diagnosis and management with steroids therapy improves outcome in most of the patients. Non response to steroids warrants second line treatment options like IV Immunoglobulin (IVIG) or Therapeutic plasma exchange.
Cañellas AR, Gols AR, Izquierdo JR, Subirana MT, Gairin XM. Idiopathic inflammatory- demyelinating diseases of the central nervous system. Neuroradiology. 2007;49(5):393-409.
Petzold A. Isolated, relapsing and progressive demyelinating diseases of the central nervous system. J Neurol. 2008;255(6):69-76.
Bar-Or A, Hintzen RQ, Dale RC, Rostasy K, Brück W, Chitnis T. Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases. Neurology. 2016;87(9): S12-9.
Krupp LB, Tardieu M, Amato MP, Banwell B, Chitnis T, Dale RC et al. International Pediatric Multiple Sclerosis Study Group. International pediatric multiple sclerosis study group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: Revisions to the 2007 definitions. Mult Scler. 2013;19:1261-7.
Gulati S, Chakrabarty B, Kumar A, Jain P, Patel H, Sani L. Acquired demyelinating disorders of central nervous system: A pediatric cohort. Ann Indian Acad Neurol. 2015;18:S48-55.
Torisu H, Kira R, Ishizaki Y, Sanefuji M, Yamaguchi Y, Yasumoto S et al. Clinical study of childhood acute disseminated encephalomyelitis, multiple sclerosis, and acute transverse myelitis in Fukuoka Prefecture, Japan. Brain Dev. 2010;32(6):454-62.
Singhi PD, Ray M, Singhi S, Khandelwal NK. Acute disseminated encephalomyelitis in North Indian children: Clinical profile and follow-up. J Child Neurol. 2006;21(10):851-7.
Gowda V, Shetty D, Madivala B, Benakappa N, Benakappa A. Clinical and radiological profiles, treatment, and outcome of pediatric acquired demyelinating disorders of the central nervous system. J Pediatr Neurosci. 2019;14(2):76-81.
Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology. 2002;59(8):1224-31.
Richer LP, Sinclair DB, Bhargava R. Neuroimaging features of acute disseminated encephalomyelitis in childhood. Pediatr Neurol. 2005;32(1):30-6.
Defresne P, Hollenberg H, Husson B, Tabarki B, Landrieu P, Huault G. Acute transverse myelitis in children: clinical course and prognostic factors. J Child Neurol. 2003;18(6):401-6.
Pidcock FS, Krishnan C, Crawford TO, Salorio CF, Trovato M, Kerr DA. Acute transverse myelitis in childhood: center-based analysis of 47 cases. Neurology. 2007;68(18):1474-80.
Lotze TE, Northrop JL, Hutton GJ, Ross B, Schiffman JS, Hunter JV. Spectrum of pediatric neuromyelitis optica. Pediatrics. 2008;122(5):e1039-47.
Collongues N, Marignier R, Zéphir H, Papeix C, Fontaine B, Blanc F et al. Long-term follow- up of neuromyelitis optica with a pediatric onset. Neurology. 2010;75(12):1084-8.