Bosma G P, van Buchem M A, Voormolen J H, van Biezen F C, Brouwer O F
Department of Neurology, Leiden University Medical Centre, The Netherlands.
J Intellect Disabil Res. 1999 Aug;43 ( Pt 4):283-8. doi: 10.1046/j.1365-2788.1999.00212.x.
Progressive walking difficulties and bladder dysfunction may be attributed to Alzheimer disease or atlanto-axial subluxation in people with Down's syndrome (DS). The present authors describe five patients with DS suffering from the above symptoms as a result of cervical spondylarthrotic myelopathy. Clinical and radiological data were collected from all patients with DS who underwent surgery for cervical spondylarthrotic myelopathy at the Leiden University Medical Centre during the period between 1991 and 1995. Five patients with DS (four males and one female) were identified. Their mean age at diagnosis was 42 years. The main clinical features were weakness of the arms and legs, ataxic gait, hyperreflexia and bilateral Babinski signs. Radiological examination showed spondylarthrosis, compression of the spinal cord and myelomalacia. The mean delay in diagnosis was 3 years. All five individuals showed clinical stabilization after laminectomy. Cervical spondylarthrotic myelopathy seems a rather frequent disorder in DS, occurring at a relatively young age. Early diagnosis may prevent irreversible neurological deficits.
进行性步行困难和膀胱功能障碍可能归因于唐氏综合征（DS）患者的阿尔茨海默病或寰枢椎半脱位。本文作者描述了5例因颈椎病性脊髓病而出现上述症状的DS患者。收集了1991年至1995年期间在莱顿大学医学中心因颈椎病性脊髓病接受手术的所有DS患者的临床和放射学资料。确定了5例DS患者（4例男性和1例女性）。他们诊断时的平均年龄为42岁。主要临床特征为四肢无力、共济失调步态、反射亢进和双侧巴宾斯基征。放射学检查显示脊椎关节病、脊髓受压和脊髓软化。诊断的平均延迟时间为3年。所有5例患者在椎板切除术后临床症状均得到稳定。颈椎病性脊髓病在DS中似乎是一种相当常见的疾病，发病年龄相对较小。早期诊断可预防不可逆的神经功能缺损。
