title: Diffuse reduction of cerebral grey matter volumes in Erdheim-Chester disease.
abstract: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis characterized by systemic inflammation and granulomatous infiltration of multiple organs including the central nervous system (CNS), bones, and retroperitoneum. CNS infiltration occurs in one third of patients, but cognitive changes are common in patients without CNS disease. Here we investigate whether there is a neuroanatomic basis to observed cognitive deficits, even in absence of CNS disease.
  
  
  
  We present a volumetric analysis of eleven ECD patients without CNS tumors or prior neurotoxic treatments.
  
  
  
  Compared to age-matched controls, ECD patients have diffuse, bihemispheric reduction in cortical thickness and subcortical gray matter.
  
  
  
  These findings provide the first corroborating evidence for neurologic disease in ECD patients without direct CNS infiltration.
authors.affiliation: Department of Neurology, Memorial Sloan Kettering Cancer Center, 160 E. 53rd. St. Second Floor Neurology, New York, NY, 10022, USA. diamone1@mskcc.org.
authors.forename: Eli L
authors.initials: EL
authors.lastname: Diamond
authors.affiliation: Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, USA.
authors.forename: Vaios
authors.initials: V
authors.lastname: Hatzoglou
authors.affiliation: Department of Radiology, Well Cornell Medical College, New York, USA.
authors.forename: Sneha
authors.initials: S
authors.lastname: Patel
authors.affiliation: Human Oncology and Pathogenesis Program, Department of Medicine, Memorial Sloan Kettering CancerCenter, New York, USA.
authors.forename: Omar
authors.initials: O
authors.lastname: Abdel-Wahab
authors.affiliation: Human Oncology and Pathogenesis Program, Department of Medicine, Memorial Sloan Kettering CancerCenter, New York, USA.
authors.forename: Raajit
authors.initials: R
authors.lastname: Rampal
authors.affiliation: Human Oncology and Pathogenesis Program, Department of Medicine, Memorial Sloan Kettering CancerCenter, New York, USA.
authors.forename: David M
authors.initials: DM
authors.lastname: Hyman
authors.affiliation: Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, USA.
authors.forename: Andrei I
authors.initials: AI
authors.lastname: Holodny
authors.affiliation: Department of Radiology, Well Cornell Medical College, New York, USA.
authors.forename: Ashish
authors.initials: A
authors.lastname: Raj
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology*; D031249:Erdheim-Chester Disease / Q000473:pathology*; D066128:Gray Matter / Q000473:pathology*; D015614:Histiocytosis / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged
keywords: Brain; Erdheim-Chester disease; Histiocytosis; MRI