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. (C) 1998 by Elsevier Science Inc.