Diagnostic criteria for corticobasal syndrome: a comparative study

Diagnostic criteria for corticobasal syndrome: a comparative study
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DOI:
10.1136/jnnp-2011-300875
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发表时间:
2012-04-01
影响因子:
11
通讯作者:
Hodges, John R.
Hodges, John R.
中科院分区:
医学1区
文献类型:
--
作者:
Mathew, Robert;Bak, Thomas H.;Hodges, John R.

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目前对于皮质基底综合征患者还没有明确的诊断标准。作者试图通过比较和应用三套众所周知的标准(来自多伦多、梅奥诊所和剑桥)来澄清这一领域。患者和方法作者首先比较了三个标准的重叠和差异,然后将它们应用于40个连续的以失用症和帕金森症为特征的局灶性皮质综合征患者(22名男性,平均年龄67岁),包括早期和晚期。结果尽管总体上相似,但在标准上存在重大差异,影响了其适用性。认知障碍甚至在演讲时也普遍存在,言语和语言障碍是最常见的特征。一些典型的特征,异肢和肌阵挛,在少数患者甚至在病程晚期才出现。这三个标准同样适用于晚期患者(多伦多92.5%,剑桥90%,梅奥87.5%)。30例患者(75%)满足所有三个标准。以该组为“金标准”,73.3%的患者在就诊时符合剑桥标准,而多伦多和梅奥诊所的标准分别为46.7%和36.7%。标准间的一致性较差。结论认知功能障碍,尤其是语言功能障碍自发病起就表现突出。剑桥标准适用于较高比例的早期皮质基底综合征病例。作者提出了一个小的修改,以捕捉失语症的高患病率。
Introduction 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).Patient and methods 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.Results 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.Conclusion 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.