title: Chiari type 1 malformation in Neurofibromatosis type 1: experience of a center and review of the literature.
abstract: OBJECTIVE
  To evaluate the possible correlation and the true incidence between Neurofibromatosis type 1 and Arnold-Chiari malformation type I.
  
  
  METHODS
  We reviewed all clinical charts, neurological consultations and MRI scans of 428 NF1 patients followed by 1994 to 2014 in our Department. NF1 patients in our clinic are seen usually every year by both the dermatologist and the neurologist. All patients also undergo a brain and spinal cord with the same 1.5 Tesla MRI scan.
  
  
  RESULTS
  We found a diagnosis of Arnold Chiari malformation type I in 9 of the 428 NF1 cases (2%).
  
  
  CONCLUSIONS
  This frequency is higher than that expected on the basis of a chance association. Therefore we underline the importance of serial MRI studies in patients with NF1 to assess the presence of cranio-cervical anomalies. Future studies should try to better understand what are the pathogenetic mechanisms underlying this close association.
authors.affiliation: Department of Dermatology and Venereology, Sapienza - University of Rome, Rome.
authors.forename: E
authors.initials: E
authors.lastname: Miraglia
authors.affiliation: Department of Neurology and Psychiatry, Sapienza - University of Rome, Rome - IRCCS Neuromed Institute, Pozzilli (IS), Italy.
authors.forename: G
authors.initials: G
authors.lastname: Fabbrini
authors.affiliation: Department of Radiology DEA, Sapienza - University of Rome, Rome.
authors.forename: C
authors.initials: C
authors.lastname: Di Biasi
authors.affiliation: Department of Dermatology and Venereology, Sapienza - University of Rome, Rome.
authors.forename: C
authors.initials: C
authors.lastname: Iacovino
authors.affiliation: Department of Neurology and Psychiatry, Sapienza - University of Rome, Rome.
authors.forename: G
authors.initials: G
authors.lastname: Ferrazzano
authors.affiliation: Department of Radiology DEA, Sapienza - University of Rome, Rome.
authors.forename: G
authors.initials: G
authors.lastname: Gualdi
authors.affiliation: Department of Dermatology and Venereology, Sapienza - University of Rome, Rome.
authors.forename: S
authors.initials: S
authors.lastname: Calvieri
authors.affiliation: Department of Dermatology and Venereology, Sapienza - University of Rome, Rome.
authors.forename: S
authors.initials: S
authors.lastname: Giustini
mesh_terms: D000328:Adult; D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000175:diagnosis*; D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis*; D013116:Spinal Cord / Q000000981:diagnostic imaging* / Q000473:pathology
keywords: Arnold-Chiari malformations; MRI scansinclude; Neurofibromatosis type 1