Gouider-Khouja N, Vidailhet M, Bonnet A M, Pichon J, Agid Y
INSERM U 289, Hópital de la Salpétrière, Paris, France.
Mov Disord. 1995 May;10(3):288-94. doi: 10.1002/mds.870100310.
Striatonigral degeneration (SND) is difficult to diagnose in vivo. The purpose of this study was to detect the best indicators for an early and reliable diagnosis of the disease. Eighteen patients clinically diagnosed as having SND were selected with rigorous inclusion criteria and compared to 18 patients with Parkinson's disease (PD) matched for age and disease duration. Apart from dysautonomia, the principal discriminant clinical features that distinguished SND from PD were the early appearance of the following symptoms and signs: (a) severe and atypical progressive parkinsonism characterized by bilateral bradykinesia and rigidity, slowness of gait, postural instability, and falls, and poor or absent response to adequate levodopa treatment; (b) increased tendon reflexes associated or not with frank pyramidal signs, severe dysarthria, and less consistently, dysphagia, stridor, antecollis, and stimulus-sensitive myoclonus, which, when present, are highly suggestive of the disease.
纹状体黑质变性（SND）在活体中难以诊断。本研究的目的是检测出能够早期且可靠诊断该疾病的最佳指标。选取了18例临床诊断为SND的患者，其入选标准严格，并与18例年龄和病程相匹配的帕金森病（PD）患者进行比较。除自主神经功能障碍外，将SND与PD区分开来的主要鉴别临床特征是以下症状和体征的早期出现：（a）严重且非典型的进行性帕金森综合征，其特征为双侧运动迟缓、强直、步态缓慢、姿势不稳和跌倒，以及对足量左旋多巴治疗反应不佳或无反应；（b）腱反射增强，可伴有或不伴有明显的锥体束征、严重构音障碍，较少见的还有吞咽困难、喘鸣、颈前屈和刺激敏感性肌阵挛，当出现这些症状时，强烈提示该疾病。
