Related Experiment Video
Updated: Jun 24, 2025
Dynamic Visual Tests to Identify and Quantify Visual Damage and Repair Following Demyelination in Optic Neuritis Patients
Published on: April 14, 2014
Long-term outcomes of ADEM-like and tumefactive presentations of CNS demyelination: a case-comparison analysis
Simon V Arnett1,2,3, Kerri Prain4, Sudarshini Ramanathan5,6
1School of Medicine, Menzies Health Institute Queensland, Gold Coast Campus, Griffith University, Gold Coast, QLD, 4222, Australia. Simon.arnett@health.qld.gov.au.
Abstract:
A minority of initial multiple sclerosis (MS) presentations clinically or radiologically resemble other central nervous system (CNS) pathologies, acute disseminated encephalomyelitis (ADEM) or tumefactive demyelination (atypical demyelination presentations). With the aim of better defining the long-term outcomes of this group we have performed a retrospective cohort comparison of atypical demyelination versus 'typical' MS presentations. Twenty-seven cases with atypical presentations (both first and subsequent demyelinating events) were identified and compared with typical MS cases. Disease features analysed included relapse rates, disability severity, whole brain and lesion volumes, lesion number and distribution. Atypical cases represented 3.9% of all MS cases. There was considerable overlap in the magnetic resonance imaging (MRI) features of ADEM-like and tumefactive demyelination cases. ADEM-like cases tended to be younger but not significantly so. Atypical cases showed a trend towards higher peak expanded disability severity score (EDSS) score at the time of their atypical presentation. Motor, cranial nerve, cerebellar, cerebral and multifocal presentations were all more common in atypical cases, and less likely to present with optic neuritis. Cerebrospinal fluid (CSF) white cell counts were higher in atypical cases (p = 0.002). One atypical case was associated with peripheral blood myelin oligodendrocyte glycoprotein (MOG) antibodies, but subsequent clinical and radiological course was in keeping with MS. There was no difference in long-term clinical outcomes including annualised relapse rates (ARR), brain volume, lesion numbers or lesion distributions. Atypical demyelination cases were more likely to receive high potency disease modifying therapy early in the course of their illness. Despite the severity of initial illness, our cohort analysis suggests that atypical demyelination presentations do not confer a higher risk of long-term adverse outcomes.
Insights
A minority of initial multiple sclerosis (MS) presentations can mimic other central nervous system (CNS) conditions. However, atypical demyelination presentations do not lead to worse long-term outcomes compared to typical MS cases.
Area of Science:
- Neurology
- Neuroimmunology
- Neuroimaging
Background:
- A subset of initial multiple sclerosis (MS) presentations exhibit clinical or radiological features overlapping with other central nervous system (CNS) pathologies, such as acute disseminated encephalomyelitis (ADEM) or tumefactive demyelination.
- These atypical presentations pose diagnostic challenges and necessitate understanding their long-term prognosis.
Purpose of the Study:
- To compare the long-term clinical outcomes of patients with atypical demyelination presentations versus those with typical MS.
- To identify specific characteristics and differences between these two groups.
Main Methods:
- Retrospective cohort study comparing 27 cases of atypical demyelination with typical MS cases.
- Analysis of disease features including relapse rates, disability severity (EDSS), MRI metrics (lesion/brain volumes), and cerebrospinal fluid (CSF) analysis.
- Evaluation of treatment patterns and long-term clinical outcomes.
Main Results:
- Atypical presentations constituted 3.9% of all MS cases, with overlap in MRI features between ADEM-like and tumefactive demyelination.
- Atypical cases showed a trend towards higher peak EDSS scores and more frequent motor, cranial nerve, cerebellar, cerebral, and multifocal presentations, with less frequent optic neuritis.
- CSF white cell counts were significantly higher in atypical cases (p=0.002).
- Despite initial severity, no significant differences were observed in long-term outcomes such as annualised relapse rates (ARR), brain volume, or lesion distribution.
- Atypical cases were more likely to receive early high-potency disease-modifying therapy.
Conclusions:
- Initial atypical demyelination presentations in MS, while potentially severe, do not appear to confer a higher risk of adverse long-term outcomes.
- The findings support that MS should be considered even in the presence of atypical initial presentations.
- Early aggressive treatment may be a factor in achieving similar long-term outcomes in atypical MS cases.
Related Concept Videos
01:35Long-term Potentiation
01:26Alzheimer's Disease: Overview
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ...
01:03Long-term Depression
Calcium Ion Concentration Mechanism
If over...