Syringomyelia in myotonic dystrophy due to spinal hemangioblastoma.
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.
Mascalchi
M
M
Cattedra di Radiologia, Universita' di Pisa, Italy.
Padovani
R
R
Taiuti
R
R
Quilici
N
N
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*