Acute Disseminated Encephalomyelitis in North Indian Children: Clinical Profile and Follow-Up

Abstract

Acute disseminated encephalomyelitis in children is not uncommon in developing countries, yet there is little systematic documentation of its clinical profile and follow-up. We studied the clinical and neuroradiologic features of acute disseminated encephalomyelitis in 52 consecutive children. Clinical details, magnetic resonance imaging (MRI) findings, and the results of other investigations were recorded, and children were followed up from 6 to 48 months. A repeat MRI was done after 3 to 4 months, and in those with persistent lesions, another MRI was done after 6 to 7 months of discharge. The mean age at presentation was 6.14 +/- 3.17 years, 73.1% were male, and 17 children had a history of antecedent infectious illness or vaccination. Most children had a meningoencephalitic presentation, with sudden-onset motor weakness in 76.9% and seizures in 36.5%. Altered sensorium and pyramidal signs were seen in 55.8% and 80.7% of children, respectively. On MRI, scattered T(2)-weighted hyperintense lesions were seen, mainly in the subcortical white matter, especially in the parietal (53.8%) and frontal (30.17%) regions. Thalamic, basal ganglia, and callosal lesions were seen in 30.76%, 17.3%, and 13.46% of cases, respectively. Variable contrast enhancement was seen in 48% of those who had contrast MRIs. The response to methylprednisolone was good, with dramatic recovery in 26.9% and marked improvement in 51.9% at discharge. On follow-up, of 44 children, residual smaller MRI lesions were seen in 30. The MRI was repeated at 6 months in children with residual lesions, and it was found that the lesions either disappeared or were significantly reduced after 6 months in 75% of cases. Four children had relapse of acute disseminated encephalomyelitis with new lesions on MRI. All of them responded to methylprednisolone. None of the clinical or neuroradiologic factors at presentation had any significant correlation with relapse. Six months after discharge, no deficits could be found in 61.3% of cases; 15.9% and 4.5% had motor and cognitive deficits, and 9% had multiple deficits. The presentation of pediatric acute disseminated encephalomyelitis in developing countries is similar to that in developed countries. In spite of an aggressive presentation, most children respond well to corticosteroids. MRI lesions disappear or are significantly reduced at 6 months in the majority of cases.

Clinical profile of acute disseminated encephalomyelitis in children

“…[245816] One difference noticed was the absence of optic neuritis in the present sample. Optic neuritis is reported to occur in 3-35% of cases in various reports.…”

“…Studies from India and abroad have reported that ADEM is more common in boys,[2–489] while multiple sclerosis, the other demyelinating disorder with many similarities, is more common in women. [1011] The reason for this female preponderance in the present sample should be evaluated in view of the disproportionate increase in the incidence of multiple sclerosis in women in recent studies.…”

The corpus callosum in the diagnosis of multiple sclerosis and other CNS demyelinating and inflammatory diseases

“…It is characteristically monophasic and, when present, impaired consciousness is a helpful feature to help discriminate it from other demyelinating diseases such as MS. CC and other periventricular lesions in ADEM may be less common than that seen in MS or NMO, although the reported frequency is highly variable ranging from 5% to 80% in case series 36–42. When the CC is involved in ADEM, there is often a high overall lesion load and lesions may be large and extend into the CC from the adjacent white matter 42. Among 12 patients with ADEM and CC involvement, the CC was involved bilaterally in all, with the splenium affected in 9 of the patients 43.…”

The Accuracy of Prevalence Rates of Multiple Sclerosis: A Critical Review

“…Other recent publications also mention relapses of DEM [22][23][24] . A curious logical hiatus occurs when changing the diagnosis from DEM to MS after a recurrence, since the MRI lesion(s) is/are still atypical and similar to those of DEM.…”