title: Late-onset multiple sclerosis presenting with cognitive dysfunction and severe cortical/infratentorial atrophy.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences calabresem@hotmail.it.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Calabrese
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences.
authors.forename: Alberto
authors.initials: A
authors.lastname: Gajofatto
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences.
authors.forename: Francesca
authors.initials: F
authors.lastname: Gobbin
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences.
authors.forename: Giulia
authors.initials: G
authors.lastname: Turri
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences.
authors.forename: Silvia
authors.initials: S
authors.lastname: Richelli
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences.
authors.forename: Angela
authors.initials: A
authors.lastname: Matinella
authors.affiliation: Institute of Radiology, Department of Pathology and Diagnostics, University of Verona, Italy.
authors.forename: Eugenio Simone
authors.initials: ES
authors.lastname: Oliboni
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences.
authors.forename: Maria Donata
authors.initials: MD
authors.lastname: Benedetti
authors.affiliation: Neurology Section, Department of Neurological and Movement Sciences.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Monaco
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D001284:Atrophy; D001921:Brain / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology*; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000473:pathology* / Q000523:psychology*; D009483:Neuropsychological Tests; D012189:Retrospective Studies; D013494:Supranuclear Palsy, Progressive / Q000473:pathology / Q000523:psychology
keywords: Cognitive dysfunction; brain and brainstem atrophy; multiple sclerosis