• Surg Neurol · Nov 1998

    Case Reports

    Syringomyelia in myotonic dystrophy due to spinal hemangioblastoma.

    • M Mascalchi, R Padovani, R Taiuti, and N Quilici.
    • Cattedra di Radiologia, Universita' di Pisa, Italy.
    • Surg Neurol. 1998 Nov 1; 50 (5): 446-8.

    BackgroundSyringomyelia is an uncommon, poorly understood finding in patients with myotonic dystrophy.MethodsWe describe a patient with myotonic dystrophy and neck pain in whom an extensive neuroradiologic diagnostic work-up was carried out.ResultsMagnetic 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.ConclusionsIntramedullary tumors can underlie syringomyelia in patients with myotonic dystrophy and have to be actively investigated with modern neuroradiologic investigations.

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