Petit H, Rousseaux M, Gozet G, Mazingue M
Rev Neurol (Paris). 1984;140(2):144-7.
A 25-year-old man developed marked weakness of the upper limbs and shoulder girdle over a period of 6 months. On examination there was amyotrophy, fasciculations and areflexia of the upper limbs with increased lower limb reflexes. Sensation and CSF were normal. CT scan showed a thickened cord with a central medullary cavity of triangular shape with a ventral base. Myelobulbography showed a type I Arnold-Chiari malformation. Spinal angiography was normal. Isotopic studies showed early ventricular reflux. Although infusion tests gave negative results a ventriculoperitoneal shunt was performed and the condition stabilized. Follow-up examination 4 years later confirmed the absence of sensory disorders and nervous arthropathies. The mechanism of production of cervical cord lesions during hydromyelia may be related to hydrocephalus, with fluid absorption across the ependymal walls, or to venous stasis determined by the Arnold-Chiari malformation. The ventral extension of the central cord cavity accounted for the isolated atypical lesion of the anterior horns.
一名25岁男性在6个月内出现上肢和肩胛带明显无力。检查发现上肢有肌萎缩、肌束震颤和反射消失，下肢反射增强。感觉和脑脊液正常。CT扫描显示脊髓增粗，中央髓腔呈三角形，底部位于腹侧。脊髓造影显示I型阿诺德-基亚里畸形。脊髓血管造影正常。同位素研究显示早期脑室反流。尽管灌注试验结果为阴性，但仍进行了脑室腹腔分流术，病情得以稳定。4年后的随访检查证实没有感觉障碍和神经性关节病。脊髓积水时颈髓病变的产生机制可能与脑积水有关，脑脊液通过室管膜壁吸收，或者与阿诺德-基亚里畸形导致的静脉淤滞有关。中央髓腔的腹侧延伸解释了前角孤立的非典型病变。
