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Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children
R C Dale1, C de Sousa, W K Chong
1Departments of Neurology, Radiology and Histopathology, Great Ormond Street Hospital for Children and Institute of Child Health, University College London, London, UK. russ@russdale.fsnet.co.uk
Insights
Acute disseminated encephalomyelitis (ADEM) and multiphasic disseminated encephalomyelitis (MDEM) present differently from multiple sclerosis (MS) in children. These distinct clinical and MRI findings may aid in predicting long-term outcomes and the potential development of MS.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases of the Central Nervous System
Background:
- Differentiating acute disseminated encephalomyelitis (ADEM) and multiphasic disseminated encephalomyelitis (MDEM) from multiple sclerosis (MS) in children is crucial for prognosis.
- Early identification of these conditions impacts patient management and family counseling.
Purpose of the Study:
- To compare the presenting clinical and magnetic resonance imaging (MRI) features of children with ADEM/MDEM versus those with MS.
- To evaluate the potential of these initial differences in predicting long-term outcomes and the risk of developing MS.
Main Methods:
- Retrospective study of 48 children with disseminated CNS demyelination (28 ADEM, 7 MDEM, 13 MS) over a mean follow-up of 5.64 years.
- Comparison of presenting symptoms, neurological signs, cerebrospinal fluid (CSF) analysis, and MRI findings between ADEM/MDEM and MS groups.
- Assessment of clinical outcomes and disease course, including relapses and recovery, in both groups.
Main Results:
- ADEM/MDEM patients more frequently presented with preceding infections, polysymptomatic onset, encephalopathy, and pyramidal signs compared to MS patients.
- Seizures were exclusive to the ADEM/MDEM group, while unilateral optic neuritis was specific to MS.
- MRI revealed periventricular sparing in ADEM/MDEM, contrasting with more common periventricular lesions in MS. ADEM/MDEM lesions showed resolution, whereas MS patients developed new lesions.
- Complete recovery occurred in 57% of ADEM patients, while MS patients exhibited relapsing-remitting or progressive courses.
Conclusions:
- Distinct clinical and MRI characteristics at presentation differentiate ADEM/MDEM from MS in children.
- These early differences are valuable for prognosis and counseling families about the potential for future MS development.
- The findings support the utility of early diagnostic markers for guiding therapeutic strategies and managing expectations.
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.