title: Fulminant inflammatory demyelination: long-term course and magnetic resonance imaging findings.
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.
authors.affiliation: Department 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.
authors.forename: C
authors.initials: C
authors.lastname: De Andrés
authors.forename: J L
authors.initials: JL
authors.lastname: Dobato
authors.forename: D
authors.initials: D
authors.lastname: Mateo
authors.forename: C
authors.initials: C
authors.lastname: Benito
authors.forename: S
authors.initials: S
authors.lastname: Giménez-Roldàn