Introduction: Acquired demyelinating disorders(ADS) constitute treatable cause of acute neurological illness. National data regarding spectrum and outcome of pediatric demyelinating diseases is limited, hence the study ¹.

Methods: Retrospective chart review of patients with ADS at pediatric-neurology division between January 2010 to 2018 was done. Neuroradiologist reviewed MRI. Diagnosis revised as per IPMSSG definitions².

Results: Forty-three patients, mean age 7.75 years, 27 boys, 16 girls included. Eleven had acute disseminated encephalomyelitis (ADEM), 10 neuromyelitis-optica-spectrum disease (NMOSD), 22 clinically-isolated-syndrome (CIS) including 6 optic-neuritis(ON), 5 brainstem-demyelination, 3 transverse-myelitis (TM), 2 each tumefactive-demyelination/others and 4 polyfocal. White-matter lesions seen in (46.5%), brainstem lesions (45%), basal ganglia and thalami (30%), spinal cord lesions (22.5%), corpus callosum (13.9%), optic nerve and area postrema (11.6%). At median follow-up of 10.6 months complete recovery occurred in 67.4%, abnormal neuroexam without functional limitation (ataxia, scoliosis, amblyopia) in 11.60%, neurodeficits in 9.3%. All with ADEM were normal, 70 % with NMOSD had no significant neurodeficits and 59% of CIS had complete recovery with neurologic signs in 36.3%. Relapse was seen in 5, all within 6 months of first episode; 4 had NMOSD. Follow- up scans reviewed in 10; 5 were normal and 5 showed resolving lesions.

Conclusion: Higher proportion of CIS than ADEM, relatively more NMOSD seen. Brainstem lesions common. Good outcome with few early relapses in NMOSD.

References

1. Gulati S. Ann Indian Acad Neurol 2015 Sep;18: S48-55

2. Tenembaum S. Neurology; 87: S59-66