title: Syringomyelia in myotonic dystrophy due to spinal hemangioblastoma.
abstract: BACKGROUND
  Syringomyelia is an uncommon, poorly understood finding in patients with myotonic dystrophy.
  
  
  METHODS
  We describe a patient with myotonic dystrophy and neck pain in whom an extensive neuroradiologic diagnostic work-up was carried out.
  
  
  RESULTS
  Magnetic resonance imaging revealed a large intramedullary cavity extending from the bulbo-medullary junction to the conus medullaris. After intravenous Gadolinium-DTPA administration, an enhanced nodule was seen at T6. Spinal arteriography showed a single hypervascular nodule and slow flow perimedullary draining veins consistent with hemangioblastoma. After removal of the nodule, a partial collapse of the intramedullary cyst was observed.
  
  
  CONCLUSIONS
  Intramedullary tumors can underlie syringomyelia in patients with myotonic dystrophy and have to be actively investigated with modern neuroradiologic investigations.
authors.affiliation: Cattedra di Radiologia, Universita' di Pisa, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Mascalchi
authors.forename: R
authors.initials: R
authors.lastname: Padovani
authors.forename: R
authors.initials: R
authors.lastname: Taiuti
authors.forename: N
authors.initials: N
authors.lastname: Quilici
mesh_terms: D000293:Adolescent; D002574:Cervical Vertebrae; D018325:Hemangioblastoma / Q000150:complications* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009223:Myotonic Dystrophy / Q000150:complications / Q000209:etiology*; D010146:Pain / Q000209:etiology; D013120:Spinal Cord Neoplasms / Q000150:complications* / Q000601:surgery; D013595:Syringomyelia / Q000150:complications / Q000473:pathology*