title: Survival profiles of patients with frontotemporal dementia and motor neuron disease.
abstract: BACKGROUND
  Frontotemporal dementia and amyotrophic lateral sclerosis are neurodegenerative diseases associated with TAR DNA-binding protein 43- and ubiquitin-immunoreactive pathologic lesions.
  
  
  OBJECTIVE
  To determine whether survival is influenced by symptom of onset in patients with frontotemporal dementia and amyotrophic lateral sclerosis.
  
  
  METHODS
  Retrospective review of patients with both cognitive impairment and motor neuron disease consecutively evaluated at 4 academic medical centers in 2 countries.
  
  
  METHODS
  Clinical phenotypes and survival patterns of patients.
  
  
  RESULTS
  A total of 87 patients were identified, including 60 who developed cognitive symptoms first, 19 who developed motor symptoms first, and 8 who had simultaneous onset of cognitive and motor symptoms. Among the 59 deceased patients, we identified 2 distinct subgroups of patients according to survival. Long-term survivors had cognitive onset and delayed emergence of motor symptoms after a long monosymptomatic phase and had significantly longer survival than the typical survivors (mean, 67.5 months vs 28.2 months, respectively; P < .001). Typical survivors can have simultaneous or discrete onset of cognitive and motor symptoms, and the simultaneous-onset patients had shorter survival (mean, 19.2 months) than those with distinct cognitive or motor onset (mean, 28.6 months) (P = .005).
  
  
  CONCLUSIONS
  Distinct patterns of survival profiles exist in patients with frontotemporal dementia and motor neuron disease, and overall survival may depend on the relative timing of the emergence of secondary symptoms.
authors.affiliation: Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA. huw@uphs.upenn.edu
authors.forename: William T
authors.initials: WT
authors.lastname: Hu
authors.forename: Harro
authors.initials: H
authors.lastname: Seelaar
authors.forename: Keith A
authors.initials: KA
authors.lastname: Josephs
authors.forename: David S
authors.initials: DS
authors.lastname: Knopman
authors.forename: Bradley F
authors.initials: BF
authors.lastname: Boeve
authors.forename: Eric J
authors.initials: EJ
authors.lastname: Sorenson
authors.forename: Leo
authors.initials: L
authors.lastname: McCluskey
authors.forename: Lauren
authors.initials: L
authors.lastname: Elman
authors.forename: Helenius J
authors.initials: HJ
authors.lastname: Schelhaas
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Parisi
authors.forename: Benno
authors.initials: B
authors.lastname: Kuesters
authors.forename: Virginia M-Y
authors.initials: VM
authors.lastname: Lee
authors.forename: John Q
authors.initials: JQ
authors.lastname: Trojanowski
authors.forename: Ronald C
authors.initials: RC
authors.lastname: Petersen
authors.forename: John C
authors.initials: JC
authors.lastname: van Swieten
authors.forename: Murray
authors.initials: M
authors.lastname: Grossman
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000473:pathology; D005260:Female; D057174:Frontotemporal Lobar Degeneration / Q000150:complications* / Q000401:mortality* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000401:mortality* / Q000503:physiopathology; D012189:Retrospective Studies