THE CLINICAL AND PATHOLOGICAL SPECTRUM OF STEELE-RICHARDSON-OLSZEWSKI SYNDROME (PROGRESSIVE SUPRANUCLEAR PALSY) - A REAPPRAISAL

THE CLINICAL AND PATHOLOGICAL SPECTRUM OF STEELE-RICHARDSON-OLSZEWSKI SYNDROME (PROGRESSIVE SUPRANUCLEAR PALSY) - A REAPPRAISAL
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DOI:
10.1093/brain/118.3.759
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发表时间:
1995-06-01
期刊:
影响因子:
14.5
通讯作者:
LEES, AJ
LEES, AJ
中科院分区:
医学1区
文献类型:
--
作者:
DANIEL, SE;DEBRUIN, VMS;LEES, AJ

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研究人员对 17 名患有进行性运动迟缓综合征的患者进行了研究,死后发现大脑皮层、皮层下核团和脑干神经原纤维变性。 7 例符合目前公认的 Steele-Richardson-Olszewski 综合征临床诊断标准,而其余缺乏核上性凝视麻痹的患者有其他临床诊断(特发性帕金森病,6 例;脑血管疾病,2 例;帕金森综合征,1 例;阿尔茨海默病,1 例)。比较两组的临床和病理结果,试图更好地定义 Steele-Richardson-Olszewski 病的谱系。
Seventeen patients with a progressive bradykinetic syndrome and post-mortem findings of neurofibrillary degeneration in cerebral cortex, subcortical nuclei and brainstem were studied. Seven fulfilled currently accepted clinical diagnostic criteria for Steele-Richardson-Olszewski syndrome, whereas the remainder who lacked supranuclear gaze palsy had alternative clinical diagnoses (idiopathic Parkinson's disease, six cases; cerebrovascular disease, two cases; Parkinson's syndrome, one case; Alzheimer's disease, one case). The clinical and pathological findings of the two groups were compared in an attempt to better define the spectrum of Steele-Richardson-Olszewski disease.