Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Brescia, Italy.
J Neurol Neurosurg Psychiatry. 2011 Aug;82(8):834-8. doi: 10.1136/jnnp.2010.221853. Epub 2011 Feb 22.
Corticobasal syndrome (CBS) has a heterogeneous neuropathological spectrum, ranging from the classical corticobasal degeneration to Alzheimer's disease (AD). The neuropathology of CBS is still unpredictable. CSF tau/abeta ratio is a reliable marker of AD.
To evaluate the presence of a distinct clinical and neuroimaging CBS phenotype according to CSF pattern.
30 patients fulfilling current clinical criteria for CBS entered the study. Each patient underwent a clinical and standardised neuropsychological assessment, and CSF analysis (total tau and abeta42 dosages). CSF AD-like pattern and CSF non-AD like pattern (nAD-like) were identified. In 23 CBS cases, (99m)Tc-ECD single photon emission computed tomography (SPECT) scan was performed and analysed by statistical parametric mapping.
CSF AD-like pattern was reported in six cases (20%). The two subgroups did not differ in demographic characteristics or global cognitive impairment. The AD-like group showed greater impairment of memory performances, language and psychomotor speed while the nAD-like group had more severe extrapyramidal syndrome with comparable apraxia scores. Voxel by voxel analysis on SPECT images demonstrated that CBS AD-like patients had greater hypoperfusion in the brain areas typically affected by AD-namely, precuneus, posterior cingulate and hippocampus, bilaterally-compared with nAD-like patients (p<0.001). No clusters above the pre-established threshold were detected when nAD-like were compared with AD-like patients.
CSF AD-like profile in CBS is associated with earlier memory impairment and brain abnormalities typically found in classical AD. These findings argue for the usefulness of CSF testing to identify AD in CBS, and might suggest a different pharmacological approach on the basis of biological data.
皮质基底节综合征（CBS）具有异质性的神经病理学谱，从经典的皮质基底节变性到阿尔茨海默病（AD）不等。CBS 的神经病理学仍然难以预测。CSF 中的 tau/abeta 比值是 AD 的可靠标志物。
根据 CSF 模式评估存在明显的临床和神经影像学 CBS 表型。
30 名符合当前 CBS 临床标准的患者进入研究。每位患者均接受临床和标准化神经心理学评估以及 CSF 分析（总 tau 和 abeta42 剂量）。确定 CSF AD 样模式和 CSF 非 AD 样模式（nAD 样）。在 23 例 CBS 病例中，进行了 (99m)Tc-ECD 单光子发射计算机断层扫描（SPECT）扫描，并通过统计参数映射进行分析。
报告了 6 例（20%）CSF AD 样模式。这两个亚组在人口统计学特征或整体认知障碍方面没有差异。AD 样组的记忆、语言和运动速度表现更差，而 nAD 样组的锥体外系综合征更严重，且具有可比的失用评分。SPECT 图像的体素分析表明，与 nAD 样患者相比，CBS AD 样患者双侧大脑区域（包括后扣带回和海马）的灌注减少更为明显，这些区域通常受到 AD 的影响（p<0.001）。当 nAD 样与 AD 样患者进行比较时，没有发现超过预先设定阈值的簇。
CBS 中的 CSF AD 样特征与早期记忆障碍和经典 AD 中常见的大脑异常有关。这些发现表明 CSF 检测对于识别 CBS 中的 AD 很有用，并且可能基于生物学数据提出不同的药物治疗方法。
