Department of Neurology, Cooper University Hospital, 3 Cooper Plaza, Suite 320, Camden, NJ 08103, USA.
Spine J. 2012 Jul;12(7):e5-8. doi: 10.1016/j.spinee.2012.07.006. Epub 2012 Aug 28.
One hallmark of neurofibromatosis Type 1 (NF1) is the presence of multiple neurofibromas, which are hormonally responsive and may undergo rapid growth during periods of hormonal surge. Although spinal neurofibromas occur in up to 40% of patients with NF1, they rarely cause neurologic sequelae, especially in the young.
To document a unique case of acute postpartum paraparesis in a young woman because of an extradural neurofibroma compressing the conus medullaris and discuss the possible hormonal influences related to rapid growth of this otherwise "benign" nerve sheath tumor. This case demonstrates the importance of closely monitoring patients with known NF1 for neurologic symptoms, especially during pregnancy and the postpartum period. We also seek to describe the management of this patient, which resulted in an excellent outcome.
Case report and literature review.
Detailed history was obtained from the patient and her family members. All medical records, imaging studies, histopathological findings, and pertinent literature were reviewed.
After evaluating a 26-year-old postpartum woman with NF1 for paraparesis, magnetic resonance imaging of the lumbar spine revealed a large heterogeneously enhancing mass at the conus medullaris. She subsequently underwent resection of the mass via laminectomy. Postoperatively, the patient exhibited marked improvement in weakness and was able to ambulate with assistance 2 days later. Histopathological examination of the mass revealed a neurofibroma with strong expression of estrogen and progesterone receptors.
This patient had a spinal neurofibroma, which likely grew in size within the peripartum period, causing a neurologic emergency. The rapid growth of the neurofibroma may have been the result of hormonal influence of estrogen or progesterone or both. To the best of our knowledge, rapid paraparesis caused by a spinal neurofibroma has never been reported in a peripartum setting. Laminectomy with complete tumor resection is the preferred treatment for spinal neurofibromas causing acute neurologic symptoms.
神经纤维瘤病 1 型（NF1）的一个标志是存在多个神经纤维瘤，这些肿瘤对激素有反应，并且在激素激增期间可能会迅速生长。尽管 NF1 患者中多达 40%存在脊柱神经纤维瘤，但它们很少引起神经后遗症，尤其是在年轻人中。
记录一例年轻女性因硬膜外神经纤维瘤压迫脊髓圆锥而导致的急性产后截瘫的罕见病例，并讨论与这种“良性”神经鞘瘤快速生长相关的可能激素影响。该病例表明密切监测已知 NF1 患者出现神经症状的重要性，尤其是在怀孕期间和产后期间。我们还旨在描述该患者的治疗方法，该方法取得了极好的结果。
病例报告和文献复习。
详细询问患者及其家属的病史。回顾所有病历、影像学研究、组织病理学发现和相关文献。
在评估一名患有 NF1 的 26 岁产后女性的截瘫后，腰椎磁共振成像显示脊髓圆锥处有一个大的异质性增强肿块。随后，她通过椎板切除术切除了肿块。术后，患者的无力明显改善，两天后可在协助下行走。肿块的组织病理学检查显示为神经纤维瘤，雌激素和孕激素受体表达强烈。
该患者存在脊柱神经纤维瘤，其大小可能在围产期内增大，导致神经急症。神经纤维瘤的快速生长可能是雌激素或孕激素或两者的激素影响的结果。据我们所知，在围产期从未有过脊柱神经纤维瘤引起的急性截瘫。对于导致急性神经症状的脊柱神经纤维瘤，首选的治疗方法是椎板切除术和完全肿瘤切除术。
