Childhood-Onset Multiple Sclerosis With Progressive Dementia and Pathological Cortical Demyelination

Authors:
  • BPBogdan F. Gh. Popescu
    Bogdan F. Gh. Popescu
  • JCJonathan L. Carter
    Jonathan L. Carter
  • RCRichard J. Caselli
    Richard J. Caselli
  • JPJoseph E. Parisi
    Joseph E. Parisi
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Abstract
Objective To describe a case of childhood-onset progressive multiple sclerosis with dementia and evidence of extensive cortical demyelination from brain biopsy specimen. Design Case report. Setting Mayo Clinic, Rochester, Minnesota. Patient A 26-year-old man with a history of behavioral changes starting at the age of 13 years followed by progressive dementia. Interventions Neurological examination, magnetic resonance imaging, cerebrospinal fluid studies, neuropsychological testing, and brain biopsy. Results Magnetic resonance imaging scans showed numerous T2-weighted hyperintensities throughout the central nervous system not associated with contrast enhancement. Brain biopsy specimens showed cortical and subcortical demyelination. All 3 types of cortical demyelinating lesions were observed: leukocortical, intracortical, and subpial. Lesions were associated with profound microglial activation. The patient continued to progress despite attempts to treat with multiple sclerosis disease–modifying therapies. Conclusions Multiple sclerosis should be considered in the diagnosis of progressive dementia in children and young adults. Cortical demyelination may contribute to cognitive decline in patients with dementia due to multiple sclerosis.

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