Chatkupt S, Ruzicka P O, Lastra C R
Department of Neurosciences, UMDNJ-New Jersey Medical School, Newark 07103.
Dev Med Child Neurol. 1993 Aug;35(8):737-41. doi: 10.1111/j.1469-8749.1993.tb11720.x.
A 20-year-old woman with myelomeningocele presented with acute right-ear pain and right hemiplegia which improved, but then progressively deteriorated. Surgery, after MRI, revealed a large arteriovenous malformation (AVM) involving the right side of the upper cervical cord and brainstem. The woman also had two epidermal nevi at the same site as the spinal cord AVMs. There has been no previous report of an association of myelomeningocele, spinal cord AVMs and epidermal nevi syndrome. The same location of the nevus and spinal cord AVMs, with a proposed common pathogenesis, raise the possibility that the association may be more than chance occurrence. Spinal cord AVMs should be considered in patients with myelomeningocele and similar clinical features.
一名患有脊髓脊膜膨出的20岁女性出现急性右耳疼痛和右半身瘫痪，症状曾有所改善，但随后逐渐恶化。MRI检查后进行的手术显示，一个大型动静脉畸形（AVM）累及上颈髓右侧和脑干。该女性在与脊髓AVM相同的部位还有两个表皮痣。此前尚无关于脊髓脊膜膨出、脊髓AVM和表皮痣综合征关联的报道。痣和脊髓AVM位于同一位置，且存在共同发病机制，这增加了这种关联可能并非偶然发生的可能性。对于患有脊髓脊膜膨出且有类似临床特征的患者，应考虑脊髓AVM的可能性。
