title: Mapping the progression of progranulin-associated frontotemporal lobar degeneration.
abstract: BACKGROUND
  A 55-year-old woman was followed over a 13-year period as part of a longitudinal study of people at risk for familial dementia. She was a member of a family with an autosomal dominant familial dementia that fulfilled consensus criteria for frontotemporal lobar degeneration. The patient was initially asymptomatic but developed progressive behavioral and cognitive decline characterized by apathy, impaired emotion recognition, mixed aphasia and parietal lobe dysfunction.
  
  
  METHODS
  Clinical assessments, neuropsychometry, volumetric brain MRI, and genetic mutation screening.
  
  
  METHODS
  Progranulin-associated frontotemporal lobar degeneration.
  
  
  RESULTS
  Explanation of the patient's condition and genetic counseling for her family.
authors.affiliation: Dementia Research Centre, Institute of Neurology, University College London, London, UK.
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Rohrer
authors.forename: Jason D
authors.initials: JD
authors.lastname: Warren
authors.forename: Josephine
authors.initials: J
authors.lastname: Barnes
authors.forename: Simon
authors.initials: S
authors.lastname: Mead
authors.forename: Jonathan
authors.initials: J
authors.lastname: Beck
authors.forename: Tracey
authors.initials: T
authors.lastname: Pepple
authors.forename: Richard
authors.initials: R
authors.lastname: Boyes
authors.forename: Rohani
authors.initials: R
authors.lastname: Omar
authors.forename: John
authors.initials: J
authors.lastname: Collinge
authors.forename: John M
authors.initials: JM
authors.lastname: Stevens
authors.forename: Elizabeth K
authors.initials: EK
authors.lastname: Warrington
authors.forename: Martin N
authors.initials: MN
authors.lastname: Rossor
authors.forename: Nick C
authors.initials: NC
authors.lastname: Fox
mesh_terms: D001931:Brain Mapping / Q000379:methods*; D003072:Cognition Disorders / Q000175:diagnosis / Q000235:genetics / Q000523:psychology / Q000628:therapy; D003704:Dementia / Q000175:diagnosis* / Q000235:genetics* / Q000523:psychology / Q000628:therapy; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics*; D008875:Middle Aged; D000077153:Progranulins; D011498:Protein Precursors / Q000235:genetics*
chemical_list: C492314:GRN protein, human; D036341:Intercellular Signaling Peptides and Proteins; D000077153:Progranulins; D011498:Protein Precursors