Mascalchi M, Padovani R, Taiuti R, Quilici N
Cattedra di Radiologia, Universita' di Pisa, Italy.
Surg Neurol. 1998 Nov;50(5):446-8. doi: 10.1016/s0090-3019(97)00050-5.
Syringomyelia is an uncommon, poorly understood finding in patients with myotonic dystrophy.
We describe a patient with myotonic dystrophy and neck pain in whom an extensive neuroradiologic diagnostic work-up was carried out.
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.
Intramedullary tumors can underlie syringomyelia in patients with myotonic dystrophy and have to be actively investigated with modern neuroradiologic investigations.
脊髓空洞症在强直性肌营养不良患者中并不常见，人们对此了解甚少。
我们描述了一名患有强直性肌营养不良和颈部疼痛的患者，对其进行了广泛的神经放射学诊断检查。
磁共振成像显示一个大的髓内空洞，从延髓-脊髓交界处延伸至脊髓圆锥。静脉注射钆喷酸葡胺后，在T6水平可见一个强化结节。脊髓血管造影显示一个单一的高血运结节以及与血管母细胞瘤相符的髓周引流静脉血流缓慢。切除结节后，观察到髓内囊肿部分塌陷。
脊髓内肿瘤可能是强直性肌营养不良患者脊髓空洞症的潜在病因，必须通过现代神经放射学检查进行积极排查。
