de la Monte S M, Horowitz S A, Larocque A A, Richardson E P
Arch Neurol. 1986 Sep;43(9):926-9. doi: 10.1001/archneur.1986.00520090056017.
Communicating syringes confined to the brain stem are extraordinarily rare. Two patients, presenting with signs and symptoms of cerebellar dysfunction, later developed evidence of brain-stem disease with dysarthria, nystagmus, deafness, and internuclear ophthalmoplegia. The condition of both patients had been diagnosed clinically as multiple sclerosis, but at autopsy they had a striking keyhole-shaped syrinx in the midbrain and upper pons, which communicated with the aqueduct and fourth ventricle without associated syringomyelia. In addition, both patients had marked atrophy and gliosis of the cerebellum, one with extension of the syrinx into cerebellar folia. The unique character of these lesions coupled with the similarity of the clinical features of the cases prompted us to name this disorder--"keyhole aqueduct syndrome."
局限于脑干的交通性空洞极为罕见。两名患者最初表现为小脑功能障碍的体征和症状，随后出现脑干疾病的证据，包括构音障碍、眼球震颤、耳聋和核间性眼肌麻痹。两名患者的病情临床上均被诊断为多发性硬化症，但尸检时发现他们的中脑和脑桥上段有一个显著的钥匙孔状空洞，与导水管和第四脑室相通，且无相关脊髓空洞症。此外，两名患者均有明显的小脑萎缩和胶质增生，其中一名患者的空洞延伸至小脑小叶。这些病变的独特特征以及病例临床特征的相似性促使我们将这种疾病命名为——“钥匙孔导水管综合征”。
