Hamlat Abderrahmane, Adn Mahmoudreza, Ben Yahia Mourad, Morandi Xavier, Brassier Gilles, Guegan Yvon
Department of Neurosurgery, CHRU Pontchaillou, Rennes, France.
J Neurosurg Spine. 2005 Dec;3(6):477-81. doi: 10.3171/spi.2005.3.6.0477.
The concept of hemorrhage in a preexisting syringomyelic cavity was first described by Gowers in 1904. Since its first description only 13 cases have been reported. The aims of this report are to describe a new case, bring this entity to wider attention, and summarize the existing literature on the subject. This 36-year-old woman presented with progressive gait disturbance and unsteadiness. Physical examination revealed incomplete quadriparesis, predominantly on the left side, and hypesthesia below C-7. Magnetic resonance imaging revealed hematomyelia characterized by a heterogeneous hyperintense signal within the central cervical cord. A liquefied well-limited hematoma was evacuated. The postoperative course was uneventful; a near-complete recovery was observed at the 7-year follow-up examination. Most cases of intrasyringal hemorrhage (ISH) have occurred in syringomyelic cavities associated with scoliosis or Chiari malformation Type I. Although there is no specific clinical picture associated with this entity, it can be characterized by three neurological forms: 1) sudden onset or rapid development of signs and symptoms, 2) acute worsening of symptoms that may improve but leaving greater neurological dysfunction than before the previous episode, and 3) ISH may initiate progressive deterioration in a patient with known syringomyelia. Intrasyringal bleeding is most probably caused by a sudden dilation of the syringomyelic cavity, which may provoke rupture of the intrasyringal vessels by an acute distension of the accompanying strands. Magnetic resonance imaging is the most accurate diagnostic modality, and recognition of ISH can lead to early, safe, and efficient surgical treatment.
1904年,高尔斯首次描述了已有脊髓空洞症腔内出血的概念。自首次描述以来,仅报告了13例病例。本报告的目的是描述一例新病例,使这一实体得到更广泛的关注,并总结关于该主题的现有文献。这位36岁的女性表现为进行性步态障碍和不稳。体格检查发现不完全性四肢轻瘫,主要在左侧,以及C-7以下感觉减退。磁共振成像显示脊髓血肿,其特征为颈髓中央内不均匀的高信号。一个液化的界限清楚的血肿被清除。术后过程顺利;在7年的随访检查中观察到近乎完全恢复。大多数脊髓内出血(ISH)病例发生在与脊柱侧弯或I型Chiari畸形相关的脊髓空洞症腔内。虽然该实体没有特定的临床表现,但它可以表现为三种神经学形式:1)症状和体征突然发作或迅速发展,2)症状急性恶化,可能改善,但留下比前一次发作前更严重的神经功能障碍,3)ISH可能引发已知脊髓空洞症患者的进行性恶化。脊髓内出血很可能是由脊髓空洞症腔的突然扩张引起的,这可能通过伴随条索的急性扩张引发脊髓内血管破裂。磁共振成像是最准确的诊断方式,认识到ISH可导致早期、安全和有效的手术治疗。