Surgical neurology · 1998
Syringomyelia in myotonic dystrophy due to spinal hemangioblastoma.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Mascalchi M, Padovani R, Taiuti R, Quilici N
Аннотация
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.
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.