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  • Title: Syringomyelia in myotonic dystrophy due to spinal hemangioblastoma.
    Author: Mascalchi M, Padovani R, Taiuti R, Quilici N.
    Journal: Surg Neurol; 1998 Nov; 50(5):446-8. PubMed ID: 9842869.
    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.
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