Cervical Myelopathy Due to Atlantoaxial Instability Associated with Down Syndrome: A Case Report

唐氏症合併頸部寰樞關節不穩定引起之頸部脊髓病變：一病例報告

蔡明蘭(Min-Lan Tsai) ； 張嘉侃(Jia-Kan Chang)

《重症醫學雜誌》 10卷1期 (2009/03) Pp. 90-96

https://doi.org/10.30035/TCCM.200903.0013

摘要

唐氏症合併頸部寰樞關節不穩定引起之頸部脊髓病變在年齡小的小孩並不常見，我們報告一個四歲的唐氏症小男孩出現右側肢體無力，走路不穩，易跌倒已有二星期，據照顧者陳述，除了有上呼吸道感染外，並無明顯外傷，亦無做翻根斗的動作。頸椎x光檢查發現寰樞關節距離有顯著增加，而造成半脫位情形，核磁共振檢查呈現脊髓被脫位的關節壓迫，而呈現局部脊髓軟化現象，病人經開刀手術固定及裝置halo-vest固定後，運動功能已經有顯著改善。對於唐氏症的孩童，因其第一、二頸椎易於鬆脫及其韌帶鬆弛，建議應早期做頸椎x光篩檢，而有神經學的臨床症狀時亦應早期診斷及處置，以減少唐氏症兒的後遺症。

關鍵字

唐氏症 ； 頸部寰樞關節不穩定 ； 脊髓病變

並列摘要

Spinal cord compression secondary to atlantoaxial instability in Down syndrome is relatively uncommon in young children. We report a 4-year-old boy with Down syndrome and myelopathy caused by atlantoaxial instability (AAI). At presentation he had right limb hemiparesis, ataxia, and bilateral hyper-reflexia for 2 weeks. There was no obvious history of trauma except for frequent upper respiratory tract infection. Cervical spine x-ray showed increased distance between the atlas and the dental process, which fulfilled the criteria for atlantoaxial instability, and cervical MRI showed spinal cord compression and myelomalacia. His neurologic signs gradually improved after surgical treatment. This report emphasized the importance of early recognition and diagnostic approach for prevention of the potential cervical spinal cord injury secondary to AAI in very young children with Down syndrome.

並列關鍵字

Cervical myelopathy ； Atlantoaxial instability ； Down syndrome