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Syringomyelia in myotonic dystrophy due to spinal hemangioblastoma
M Mascalchi1, R Padovani, R Taiuti
1Cattedra di Radiologia, Universita' di Pisa, Italy.
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.