title: Clinical-radiological-pathological spectrum of central nervous system-idiopathic inflammatory demyelinating disease in the elderly.
abstract: BACKGROUND
  The spectrum of central nervous system-idiopathic inflammatory demyelinating disease (CNS-IIDD) in the elderly is uncertain.
  
  
  OBJECTIVE
  To describe the clinical, radiological, and pathological features of a cohort of 30 pathologically proven CNS-IIDD patients ⩾65 years.
  
  
  METHODS
  Elderly multiple sclerosis (MS)/clinically isolated syndrome (CIS) patients were compared to a cohort of 125 patients with pathologically proven MS/CIS and symptom onset <65 years.
  
  
  RESULTS
  Median age at symptom onset was 69 years (interquartile range (IQR) = 68-75). Median follow-up was 1.9 years (IQR = 1.0-5.6). Diagnoses were MS (14/30), CIS (11/30), neuromyelitis optica (NMO; 4/30), and acute disseminated encephalomyelitis (ADEM; 1/30). Disability was higher in patients with MS/CIS ⩾65 compared to patients <65 (median Expanded Disability Status Scale (EDSS) 4 (IQR = 2.5-7) vs 2.5 (IQR = 1.5-4); p = 0.002). When compared to patients <65 years, there was no difference in the lesion size, number of patients fulfilling Barkhof's criteria, edema, or mass effect. Confluent demyelination was observed in 27 patients (MS/CIS (23/25), NMO (4)), 2 had a mixed perivenular/confluent pattern (MS (1), ADEM (1)), and 1 patient with MS had a mixed confluent/perivenular/coalescent pattern. Early active lesions were found in 19/30 patients ((MS (4), CIS (13), NMO (2); 53%). Cortical demyelination was present in 7/12 (58%) patients (MS (3), CIS(3), ADEM (1)).
  
  
  CONCLUSIONS
  A spectrum of CNS-IIDD can develop in the elderly, with presenting symptoms similar to younger patients. Early diagnosis of CNS demyelinating disease is essential to avoid invasive and disabling procedures.
authors.affiliation: Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: W Oliver
authors.initials: WO
authors.lastname: Tobin
authors.affiliation: Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Chiara
authors.initials: C
authors.lastname: Costanzi
authors.affiliation: Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Yong
authors.initials: Y
authors.lastname: Guo
authors.affiliation: Department of Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Parisi
authors.affiliation: Department of Health Sciences Research, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Weigand
authors.affiliation: Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Claudia F
authors.initials: CF
authors.lastname: Lucchinetti
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000375:Aging / Q000473:pathology / Q000502:physiology; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D003711:Demyelinating Diseases / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging
keywords: Demyelination; acute disseminated encephalomyelitis; autopsy; brain biopsy; elderly; neuromyelitis optica