ICNC Abstracts, ICNC 2018

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Spectrum of acquired White Matter Disorders in Children and their Outcomes
Vikas Chauhan, Arushi Gahlot Saini, Naveen Sankhyan, Sameer Vyas, Paramjit Singh, Jayashree Muralidharan

Last modified: 2018-09-09


Objective: To study hospital-based frequency, clinic-radiological features, and functional outcomes of acquired white-matter disorders in children

Methodology: Consecutive children from 1 month to 14 years of age with a probable/confirmed, acquired, predominant white matter disorder secondary to an infection, inflammation, toxin, metabolic insult or other acquired insult were enrolled over the past six months (January 2018-June 2018). Retrospective review of case sheets and prospective follow up was done. Functional outcomes was assessed after atleast 3 months of follow-up by expended disability status scale (EDSS).

Results: 8511 children were admitted in the hospital during the study period. Of these, 48 children with acquired white-matter disorders were identified. Hospital based frequency was 0.56%. Median age at presentation was 58.5 months (range 2-141). Majority were males 62.5% (n=30). Most prevalent disorders were acute encephalopathy with biphasic seizures and reduced diffusion (AESD) 18.8% (n=9), infectious encephalitis 18.8% (n=9), acute necrotizing encephalopathy of childhood 14.6% (n=7), acute disseminated encephalomyelitis 12.5% (n=6), cytomegalovirus infection 10.4% (n=5), acute transverse myelitis 10.4% (n=5), posterior reversible encephalopathy syndrome 6.3% (n=3), neuromyelitis optica spectrum disorder 4.2% (n=2) , optic neuritis 2% (n=1), and subacute sclerosing panencephalitis 2 (n=1).

EDSS was available in 43.8% (n=21). Mean EDSS overall was 3.3. Best EDSS scores were observed in the demyelinating group (mean 1.2, no disability or minimal signs in one functional domain) while the worst EDSS was observed with cytomegalovirus (mean 6.7) and AESD (mean 6.3).

Conclusion: Acquired white-matter disorders area significant cause of morbidity in children and need multidisciplinary care.


White-matter disorders; Children; Demyelinating; Encephalopathy; Outcome

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