title: Clinical and MRI characterization of MS patients with a pure and severe cognitive onset.
abstract: OBJECTIVE
  Cognitive and behavioural symptoms are common in multiple sclerosis (MS), but they are rarely the inaugural and predominant manifestation of the disease. Our objective is to characterize the clinical and radiological features of cognitive-multiple sclerosis (cog-MS), defined as MS subjects who entered into the disease with cognitive symptoms, which subsequently remain the predominant manifestation.
  
  
  METHODS
  We describe the disease course, and clinical and radiological features of 18 subjects with a cognitive form of MS.
  
  
  RESULTS
  Memory loss and behavioural changes were the primary symptoms at disease onset. They remained prominent and led to severe cognitive impairment during disease course. The main associated manifestations were depression, pathological laughing and/or crying, urinary incontinence and gait disturbance suggestive of high-level gait disorder. Motor, sensory or cerebellar abnormalities were uncommon. During disease course, superimposed neurological relapses occurred in 61% of cases. Brain MRI revealed multiple periventricular lesions that were extensive and confluent in half of cases, and a severe atrophy measured as an increase in the third ventricular width compared to age-matched healthy controls. Gadolinium-enhancing lesions were common (72%). The mean diagnosis delay from disease onset was 2 years. A principal component analysis on the neuropsychological results revealed that verbal memory assessment is complementary to global cognitive functioning evaluation in these patients with severe cognitive deficit. Verbal memory deficit was associated with high EDSS.
  
  
  CONCLUSIONS
  cog-MS patients might represent a challenging diagnosis, which needs to be individualized for an early management.
authors.affiliation: AP-HP, Hôpital de la Salpêtrière, 47-83 boulevard de l'Hôpital, 75013 Paris, France.
authors.forename: Rana
authors.initials: R
authors.lastname: Assouad
authors.affiliation: AP-HP, Hôpital de la Salpêtrière, 47-83 boulevard de l'Hôpital, 75013 Paris, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1127, and Inserm U 1127, and CNRS UMR 7225, and ICM, F-75013 Paris, France.
authors.forename: Celine
authors.initials: C
authors.lastname: Louapre
authors.affiliation: AP-HP, Hôpital de la Salpêtrière, 47-83 boulevard de l'Hôpital, 75013 Paris, France; Centre Hospitalo-Universitaire de Reims, Université Champagne Ardennes Reims, France.
authors.forename: Ayman
authors.initials: A
authors.lastname: Tourbah
authors.affiliation: AP-HP, Hôpital de la Salpêtrière, 47-83 boulevard de l'Hôpital, 75013 Paris, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Papeix
authors.affiliation: AP-HP, Hôpital de la Salpêtrière, 47-83 boulevard de l'Hôpital, 75013 Paris, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1127, and Inserm U 1127, and CNRS UMR 7225, and ICM, F-75013 Paris, France.
authors.forename: Damien
authors.initials: D
authors.lastname: Galanaud
authors.affiliation: AP-HP, Hôpital de la Salpêtrière, 47-83 boulevard de l'Hôpital, 75013 Paris, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1127, and Inserm U 1127, and CNRS UMR 7225, and ICM, F-75013 Paris, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Lubetzki
authors.affiliation: Sorbonne Universités, UPMC Univ Paris 06, UMR S 1127, and Inserm U 1127, and CNRS UMR 7225, and ICM, F-75013 Paris, France; AP-HP, Hôpital Tenon, 4 rue de la Chine, 75020 Paris, France. Electronic address: bruno.stankoff@tnn.aphp.fr.
authors.forename: Bruno
authors.initials: B
authors.lastname: Stankoff
mesh_terms: D000328:Adult; D000342:Affective Symptoms / Q000503:physiopathology; D000368:Aged; D001284:Atrophy / Q000473:pathology; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D003072:Cognition Disorders / Q000473:pathology / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D020235:Gait Apraxia / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000145:classification / Q000473:pathology / Q000503:physiopathology*; D055815:Young Adult
keywords: Brain atrophy; Cognition; Gait apraxia; Multiple sclerosis; Pseudobulbar affect