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

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

Research output: Contribution to journalArticle

12 Citations (Scopus)

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 2011

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Demyelinating Diseases
Multiple Sclerosis
Dementia
Biopsy
Brain
Magnetic Resonance Imaging
faropenem medoxomil
Neurologic Examination
Nervous System
Cerebrospinal Fluid
Onset
Childhood
Therapeutics

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Childhood-onset multiple sclerosis with progressive dementia and pathological cortical demyelination. / Bunyan, Reem F.; Popescu, Bogdan F Gh; Carter, Jonathan L.; Caselli, Richard John; Parisi, Joseph E; Lucchinetti, Claudia F.

In: Archives of Neurology, Vol. 68, No. 4, 04.2011, p. 525-528.

Research output: Contribution to journalArticle

Bunyan, Reem F. ; Popescu, Bogdan F Gh ; Carter, Jonathan L. ; Caselli, Richard John ; Parisi, Joseph E ; Lucchinetti, Claudia F. / Childhood-onset multiple sclerosis with progressive dementia and pathological cortical demyelination. In: Archives of Neurology. 2011 ; Vol. 68, No. 4. pp. 525-528.
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