4.3 Article

Late-onset multiple sclerosis presenting with cognitive dysfunction and severe cortical/infratentorial atrophy

Journal

MULTIPLE SCLEROSIS JOURNAL
Volume 21, Issue 5, Pages 580-589

Publisher

SAGE PUBLICATIONS LTD
DOI: 10.1177/1352458514542363

Keywords

Cognitive dysfunction; multiple sclerosis; brain and brainstem atrophy

Funding

  1. Sanofi-Genzyme
  2. Merck Serono
  3. Biogen Idec
  4. Teva
  5. Bayer
  6. Novartis Pharma

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Objective: Although cognitive dysfunction is a relevant aspect of multiple sclerosis (MS) from the earliest disease phase, cognitive onset is unusual thus jeopardizing early and accurate diagnosis. Here we describe 12 patients presenting with cognitive dysfunction as primary manifestation of MS with either mild or no impairment in non-cognitive neurological domains. Methods: Twelve patients with cognitive onset who were subsequently diagnosed with MS (CI-MS) were included in this retrospective study. Twelve cognitively normal MS patients (CN-MS), 12 healthy controls and four patients having progressive supranuclear palsy (PSP) served as the reference population. Results: Ten CI-MS patients had progressive clinical course and all patients had late disease onset (median age = 49 years; range = 40-58 years). Among cognitive functions, frontal domains were the most involved. Compared to CN-MS and healthy controls, significant cortical and infratentorial atrophy characterized CI-MS patients. Selective atrophy of midbrain tegmentum with relative sparing of pons, known as The Hummingbird sign, was observed in eight CI-MS and in three PSP patients. Discussion: Our observation suggests that MS diagnosis should be taken into consideration in case of cognitive dysfunction, particularly when associated with slowly progressive disease course and severe cortical, cerebellar and brainstem atrophy even in the absence of other major neurological symptoms and signs.

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