The Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Brescia, Italy.
J Alzheimers Dis. 2011;23(3):505-12. doi: 10.3233/JAD-2010-101407.
Frontotemporal lobar degeneration (FTLD) refers to heterogeneous clinical and biological conditions. In FTLD, cerebrospinal fluid (CSF) tau levels have been reported highly variable. The aim of the present study was to evaluate whether CSF tau might be the hallmark of a distinct FTLD phenotype. Fifty-five FTLD patients, who underwent CSF analysis, were considered in the present study. In each patient, a wide standardized neuropsychological evaluation, and CSF tau, phospho-tau, and amyloid-β (Aβ) dosages were performed. Each patient was followed-up to five years, and outcomes carefully recorded. In a subgroup of patients (n = 24), magnetic resonance imaging scanning was performed, by using voxel-based morphometry, for grey matter investigation. The higher the CSF tau levels, the worse the neuropsychological and neuroimaging pattern, mainly characterized by greater language disturbances and left temporal grey matter loss. The same pattern, even if less significant, was associated with CSF phospho-tau, while CSF Aβ levels did not play any influence on FTLD phenotype. FTLD patients with high CSF tau showed poor prognosis compared to those with low CSF tau (p = 0.031). In FTLD, CSF tau levels might be considered a marker of neurodegeneration, associated with a specific clinical and neuroimaging picture, and significantly related to poor outcome. Further studies aimed at defining the biological underpinnings of these findings are warranted.
额颞叶变性（FTLD）是指异质性的临床和生物学条件。在 FTLD 中，脑脊液（CSF）中的 tau 水平被报道存在高度变异性。本研究旨在评估 CSF tau 是否可能是独特 FTLD 表型的标志。本研究共纳入了 55 名接受 CSF 分析的 FTLD 患者。每位患者均进行了广泛的标准化神经心理学评估，以及 CSF tau、磷酸化 tau 和淀粉样蛋白-β（Aβ）的检测。每位患者均进行了长达五年的随访，并仔细记录了结果。在一组患者（n = 24）中，通过基于体素的形态计量学进行了磁共振成像扫描，以进行灰质研究。CSF tau 水平越高，神经心理学和神经影像学模式越差，主要表现为更严重的语言障碍和左侧颞叶灰质丢失。即使不太明显，与 CSF 磷酸化 tau 相关的也是相同模式，而 CSF Aβ 水平对 FTLD 表型没有任何影响。与 CSF tau 水平较低的患者相比，CSF tau 水平较高的 FTLD 患者预后较差（p = 0.031）。在 FTLD 中，CSF tau 水平可能被视为神经退行性变的标志物，与特定的临床和神经影像学表现相关，并与不良结局显著相关。需要进一步研究来确定这些发现的生物学基础。
