Neurosciences Research Australia, and University of New South Wales, Sydney, Australia.
J Neurol Neurosurg Psychiatry. 2012 Apr;83(4):405-10. doi: 10.1136/jnnp-2011-300875. Epub 2011 Oct 21.
There are no well-established criteria for patients with corticobasal syndrome. The authors have attempted to clarify this area by comparing and applying three sets of well-known criteria (from Toronto, the Mayo Clinic and Cambridge).
The authors first compared the three criteria for overlap and differences, and then applied them to a group of 40 consecutive patients (22 men, mean age 67 years) with focal cortical syndrome characterised by apraxia and Parkinsonism, at both the early stages and later in their illness.
Despite an overall similarity, there were major differences in the criteria which affect their applicability. Cognitive impairment was ubiquitous even at presentation, with speech and language impairment the commonest feature. Some classic features, alien limb and myoclonus, were present in a minority only even late in their course. The three criteria were equally applicable to patients with advanced disease (Toronto 92.5%, Cambridge 90% and Mayo 87.5%). Thirty patients (75%) satisfied all three criteria. Using this group as a 'gold standard', 73.3% fulfilled the Cambridge criteria at presentation compared with 46.7% and 36.7% for the Toronto and Mayo Clinic criteria, respectively. Concordance between criteria was poor.
Cognitive impairment, especially language impairment, was prominent from onset of disease. The Cambridge criteria apply to a higher proportion of cases at an early stage of corticobasal syndrome. The authors suggest a minor modification to capture the high prevalence of aphasia.
皮质基底节综合征患者目前尚无明确的诊断标准。作者试图通过比较和应用三套知名的标准（多伦多、梅奥诊所和剑桥）来阐明这一领域。
作者首先比较了这三套标准的重叠和差异，然后将其应用于一组 40 例连续的皮质局灶性综合征患者（22 例男性，平均年龄 67 岁），这些患者均存在以失用症和帕金森病为特征的早期和晚期病变。
尽管总体相似，但这些标准存在较大差异，影响其适用性。认知障碍甚至在发病时就普遍存在，言语和语言障碍是最常见的特征。一些经典特征，如生疏肢体和肌阵挛，即使在病程后期也仅少数患者存在。这三种标准对晚期疾病的患者同样适用（多伦多 92.5%，剑桥 90%和 Mayo 87.5%）。30 例患者（75%）符合所有三项标准。使用这组作为“金标准”，在发病时，73.3%符合剑桥标准，而多伦多和 Mayo 诊所标准的符合率分别为 46.7%和 36.7%。标准之间的一致性较差。
认知障碍，特别是语言障碍，从疾病开始就很明显。剑桥标准适用于皮质基底节综合征早期更高比例的病例。作者建议对其进行微小的修改，以纳入高发病率的失语症。
