Childhood-onset multiple sclerosis with progressive dementia and pathological cortical demyelination

Reem F. Bunyan, Bogdan F.Gh Popescu, Jonathan L. Carter, Richard J. Caselli, Joseph E. Parisi, Claudia F. Lucchinetti

Research output: Contribution to journalArticle

12 Scopus citations

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 numerousT2- weightedhyperintensitiesthroughoutthecentral nervous system not associated with contrast enhancement. Brain biopsy specimensshowedcorticalandsubcortical demyelination. All3typesofcorticaldemyelinatinglesionswere 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 progressivedementiain childrenandyoung adults. Cortical demyelinationmay contribute to cognitive decline in patients with dementia due to multiple sclerosis.

Original languageEnglish (US)
Pages (from-to)525-528
Number of pages4
JournalArchives of neurology
Volume68
Issue number4
DOIs
StatePublished - Apr 1 2011

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ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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