Vallée B, Mercier P, Menei P, Bouhour F, Fischer C, Fournier D, Bougeard R, Diabira S, Mahla K
Neurochirurgie C, Hôpital Neurologique Lyon, Cedex, France.
Acta Neurochir (Wien). 1999;141(9):907-13. doi: 10.1007/s007010050396.
A specific cause of progressive Brown-Sequard syndrome has been identified: a ventral herniation of the thoracic spinal cord through the dural sleeve on one side.
Four female patients who were affected by a progressive Brown Sequard syndrome related to a transdural spinal cord herniation have been investigated and were submitted to surgery and postoperative evaluation.
The MRI scan showed atrophy and forward displacement of the spinal cord on one side and adhesion of the spinal cord to the dura mater. CT myelography demonstrated the disappearance of the premedullar rim at the level of the herniation and the shadow of the extradural herniation. Surgical treatment consisted in the excision of the arachnoid cyst when there was one, section of the dentate ligament, release of the adhesions, detachment of the spinal cord from the hernial orifice, and lastly suture of the dural tear or placement by a patch. Follow-up examination showed motor improvement with persistent sensory deficit in two cases and stabilisation in two cases.
The cause of the dural tear, either traumatic or congenital could not be confirmed in the four cases. Symptoms probably occur when herniation fills the orifice and strangulation happens which explains the late appearance and progressive evolution of this myelopathy. Mobilisation of the herniated spinal cord back into the intradural space can be achieved by surgery and may stop the evolution of the symptoms and signs.
已确定进行性布朗 - 塞卡尔综合征的一个特定病因：胸段脊髓一侧经硬脊膜袖套腹侧疝出。
对4例与经硬脊膜脊髓疝相关的进行性布朗 - 塞卡尔综合征女性患者进行了研究，并接受了手术及术后评估。
MRI扫描显示一侧脊髓萎缩并向前移位，以及脊髓与硬脑膜粘连。CT脊髓造影显示疝出水平的髓前边缘消失及硬膜外疝的影像。手术治疗包括如有蛛网膜囊肿则将其切除、切断齿状韧带、松解粘连、将脊髓从疝孔分离，最后缝合硬脑膜撕裂处或用补片修补。随访检查显示，2例患者运动功能改善但仍存在感觉障碍，2例患者病情稳定。
这4例患者中无法确定硬脑膜撕裂是创伤性还是先天性原因。当疝出物充满疝孔并发生绞窄时可能出现症状，这解释了这种脊髓病的晚期出现和进行性发展。通过手术可将疝出的脊髓回纳到硬膜内空间，这可能会阻止症状和体征的发展。
