Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children

Abstract

Forty-eight children with disseminated demyelination of the CNS, 28 with acute disseminated encephalomyelitis (ADEM), seven with multiphasic disseminated encephalomyelitis (MDEM) and 13 with multiple sclerosis were studied for a mean follow-up period of 5.64 years. The presentation findings of the ADEM/MDEM group were compared with those of the multiple sclerosis group. The following findings were more commonly seen in ADEM/MDEM presentation compared with the multiple sclerosis presentations: predemyelinating infectious disease (74 versus 38%, P: < 0.05); polysymptomatic presentation (91 versus 38%, P: < 0.002); pyramidal signs (71 versus 23%, P: < 0.01); encephalopathy (69 versus 15%, P: < 0.002); and bilateral optic neuritis (23 versus 8%, not significant). Seizures occurred only in the ADEM/MDEM group (17 versus 0%, not significant). Unilateral optic neuritis occurred only in the multiple sclerosis patients (23 versus 0%, P: < 0.01). There were no differences in the frequencies of transverse myelitis, brainstem involvement, cerebellar signs and sensory disturbance between the two groups. ADEM/MDEM patients were more likely to have blood leucocytosis (64 versus 22%, P: < 0.05), CSF lymphocytosis (64 versus 42%, not significant) and CSF protein elevation (60 versus 33%, not significant). Patients presenting with multiple sclerosis were more likely to have intrathecal synthesis of oligoclonal bands on presentation (64 versus 29%, not significant). MRI showed that subcortical white matter lesions were almost universal in both groups, though periventricular lesions were more common in multiple sclerosis (92 versus 44%, P: < 0.01). By contrast, in ADEM/MDEM there was absolute and relative periventricular sparing in 56 and 78% of patients, respectively. Follow-up MRI revealed complete or partial lesion resolution in 90% and no new lesions in the ADEM/MDEM group. All of the multiple sclerosis patients had new lesions on repeat MRI (five during relapse and six during asymptomatic convalescent phases). The outcome in the ADEM patients was mixed; 57% of patients made a complete recovery. The mean follow-up for the 35 ADEM/MDEM patients was 5.78 years (range 1.0-15.4 years). Eight of the 13 multiple sclerosis patients relapsed within the first year; 11 had a relapsing-remitting course, one a primary progressive course and one a secondary progressive course. These differences in the presentation of ADEM/MDEM compared with multiple sclerosis may help in the prognosis given to families regarding the possibility of later development of multiple sclerosis.

Clinical characteristics of optic neuritis in Taiwanese children

“…In addition, MS is rarely associated with a preceding viral infection. 32 MRI is the imaging of choice in diagnosis of ADEM, and is beneficial in the distinction from MS. 33 In our study, the visual outcome in the ADEM group was not different from that in the MS group, which might be due to too small a number of patients in these two groups to achieve a statistically significant difference. Further investigation for collecting more patient number in these two groups and a prospective data collection for a considerable length of time should be needed.…”

Use of neuron-specific enolase for assessing the severity and outcome of neurological disorders in patients

“…However, the estimate in a single patient of the severity of neurological status as well as outcome based exclusively on cNSE should be considered with caution. be due to the fact that acute disseminated encephalomyelitis predominantly affects white matter (39), which contains lower concentrations of NSE. An alternative explanation is that NSE had been elevated at the onset of symptoms but had returned to normal levels at the time of lumbar puncture.…”

Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report

“…The diagnosis of multiple sclerosis, the most common among the demyelinating diseases, requires the presence of relapsing or progressive neurological disturbances with spatial dispersion [11][12][13] . More agressive variants of multiple sclerosis, such as Schilder's disease and Balo's concentric sclerosis, usually take months or years but rarely weeks to develop.…”