title: Diffuse Encephalopathy Associated with Isolated Cerebral Langerhans Cell Histiocytosis.
abstract: Langerhans cell histiocytosis is a rare disease of the monocyte-macrophage system. Abnormalities of the hypothalamic-pituitary region are common in individuals with central nervous system involvement.
  
  
  
  This six-year-old boy developed rapidly progressive aggressive behavior, central diabetes insipidus, and repeated complex partial seizures. Magnetic resonance imaging revealed a diffuse leukoencephalopathy-like pattern and numerous infratentorial and supratentorial granulomatous nodules in the brain parenchyma along with infundibular and hypothalamic mass lesions. Stereotactic serial biopsies enabled histopathologic and immunohistochemical diagnosis of Langerhans cell histiocytosis.
  
  
  
  Similar MRI findings have rarely been described in the literature. These findings represent part of the broad neuroradiological spectrum of Langerhans cell histiocytosis of the nervous system in children.
authors.affiliation: Department of Radiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany. Electronic address: oliver.rompel@uk-erlangen.de.
authors.forename: Oliver
authors.initials: O
authors.lastname: Rompel
authors.affiliation: Institute of Neuropathology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: Rolf
authors.initials: R
authors.lastname: Buslei
authors.affiliation: Department of Radiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Hammon
authors.affiliation: Department of Pediatrics, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: Helmuth-Günther
authors.initials: HG
authors.lastname: Dörr
authors.affiliation: Department of Pediatrics, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Chada
authors.affiliation: Department of Neurosurgery, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: Guido
authors.initials: G
authors.lastname: Nikkhah
authors.affiliation: Department of Radiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Uder
authors.affiliation: Department of Pediatrics, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: Regina
authors.initials: R
authors.lastname: Trollmann
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology*; D001927:Brain Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D002648:Child; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008297:Male
keywords: Langerhans cell histiocytosis; MRI findings; biopsy; brain parenchyma; multiple granulomas