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Published on: July 19, 2019
Fulminant inflammatory demyelination: long-term course and magnetic resonance imaging findings
C De Andrés1, J L Dobato, D Mateo
1Department of Neurology, Hospital General Universitario, Gregorio Marañón, Doctor Esquerdo 42, 28007-Madrid, SpainDepartment of Neuroradiology, Hospital General Universitario, Gregorio Marañón, Doctor Esquerdo 42, 28007-Madrid, Spain.
Abstract:
The course and prognosis of adult patients who recover from acute and extensive inflammatory demyelination, variously termed fulminant or Marburg's type of multiple sclerosis (MS), is poorly known. We report long-term magnetic resonance imaging (MRI) and neuropsychological findings in two patients who developed acute psychosis and a state resembling akinetic mutism, with CT scan evidence of extensive cerebral white matter damage. Both patients ultimately recovered, but were left with a severe, non-progressive dementia with prominent frontal lobe signs unassociated with motor impairment. Extensive residual MRI lesions with evidence of tissue destruction in the white matter and callosal atrophy were visible many years after the initial event One of the patients developed a single relapsing-remitting episode with multifocal neurological deficits several years after the onset, whereas the condition remained monophasic in the other patient during an 8 year follow-up. Our observations suggest that while patients with so-called "fulminant" demyelinating disorders may nowadays survive with supportive measures, the disorder may remain stabilized for many years following disease onset.
Insights
Adults recovering from severe demyelinating disease, like Marburg's multiple sclerosis (MS), may survive but often develop lasting dementia. Long-term follow-up reveals persistent brain damage and varied disease courses.
Area of Science:
- Neurology
- Neuroimmunology
Background:
- The long-term outcomes for adult patients with acute, extensive inflammatory demyelination, known as fulminant or Marburg's type of multiple sclerosis (MS), are not well understood.
- This condition presents with severe neurological symptoms and significant white matter damage.
Purpose of the Study:
- To report long-term magnetic resonance imaging (MRI) and neuropsychological findings in two patients with Marburg's MS.
- To characterize the course and prognosis of this rare and aggressive form of multiple sclerosis.
Main Methods:
- Long-term follow-up of two adult patients with Marburg's MS.
- Serial magnetic resonance imaging (MRI) and neuropsychological assessments.
- Clinical evaluation of neurological deficits and disease progression.
Main Results:
- Both patients survived the acute phase but developed severe, non-progressive dementia with frontal lobe signs.
- Persistent MRI lesions indicating white matter destruction and callosal atrophy were observed years after the initial event.
- One patient experienced a later relapsing-remitting episode, while the other had a monophasic course over an 8-year follow-up.
Conclusions:
- Adults with fulminant demyelinating disorders, including Marburg's MS, can survive with supportive care.
- These patients may experience long-term stabilization following the initial event, but often with residual cognitive impairment and visible brain damage.
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