Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK.
J Neurol. 2010 Apr;257(4):569-74. doi: 10.1007/s00415-009-5371-4. Epub 2009 Nov 12.
The clinical and neuroanatomical correlates of specific apraxias in neurodegenerative disease are not well understood. Here we addressed this issue in progressive nonfluent aphasia (PNFA), a canonical subtype of frontotemporal lobar degeneration that has been consistently associated with apraxia of speech (AOS) and in some cases orofacial apraxia, limb apraxia and/or parkinsonism. Sixteen patients with PNFA according to current consensus criteria were studied. Three patients had a corticobasal syndrome (CBS) and two a progressive supranuclear palsy (PSP) syndrome. Speech, orofacial and limb praxis functions were assessed using the Apraxia Battery for Adults-2 and a voxel-based morphometry (VBM) analysis was conducted on brain MRI scans from the patient cohort in order to identify neuroanatomical correlates. All patients had AOS based on reduced diadochokinetic rate, 69% of cases had an abnormal orofacial apraxia score and 44% of cases (including the three CBS cases and one case with PSP) had an abnormal limb apraxia score. Severity of orofacial apraxia (but not AOS or limb apraxia) correlated with estimated clinical disease duration. The VBM analysis identified distinct neuroanatomical bases for each form of apraxia: the severity of AOS correlated with left posterior inferior frontal lobe atrophy; orofacial apraxia with left middle frontal, premotor and supplementary motor cortical atrophy; and limb apraxia with left inferior parietal lobe atrophy. Our findings show that apraxia of various kinds can be a clinical issue in PNFA and demonstrate that specific apraxias are clinically and anatomically dissociable within this population of patients.
特定失用症在神经退行性疾病中的临床和神经解剖学相关性尚不清楚。在这里，我们研究了进行性非流利性失语症（PNFA），这是额颞叶变性的典型亚型，与言语失用症（AOS）一致，在某些情况下还与口颜面失用症、肢体失用症和/或帕金森病有关。根据目前的共识标准，研究了 16 名患有 PNFA 的患者。其中 3 名患者患有皮质基底节综合征（CBS），2 名患者患有进行性核上性麻痹（PSP）综合征。使用成人失语症评估量表（Apraxia Battery for Adults-2）评估言语、口颜面和肢体运动功能，并对患者队列的脑 MRI 扫描进行基于体素的形态计量学（VBM）分析，以确定神经解剖学相关性。所有患者均基于减少的双音节词速率出现 AOS，69%的病例存在异常口颜面失用症评分，44%的病例（包括 3 例 CBS 病例和 1 例 PSP 病例）存在异常肢体失用症评分。口颜面失用症的严重程度（但不是 AOS 或肢体失用症）与估计的临床疾病持续时间相关。VBM 分析确定了每种失用症的不同神经解剖学基础：AOS 的严重程度与左后下额叶萎缩相关；口颜面失用症与左额中、运动前和辅助运动皮质萎缩相关；肢体失用症与左顶下叶萎缩相关。我们的研究结果表明，各种失用症在 PNFA 中可能是一个临床问题，并表明在这组患者中，特定的失用症在临床上和解剖学上是可分离的。
