未经授权:一个82岁的右撇子,一位退休教师,报告了事件,三年前,移动他的左臂和脚的困难,他的左手颤抖,左上肢的手势似乎与病人的意志无关。
UNASSIGNED:我们描述了一个不寻常的皮质基底综合征(CBS)病例,显示路易体痴呆(DLB)的疾病相关生物标志物。
未经证实:临床,神经心理学,成像,并进行了生物标志物评估,包括脑脊液(CSF)中的tau和淀粉样β水平以及CSF和嗅觉粘膜(OM)中α-突触核蛋白的RT-QuIC测定,以及QEEG评估。
未经证实:患者出现静息性震颤,轻度锥体外系高张力,左侧轻度运动迟缓,左上肢严重失用症.脑MRI显示弥漫性右半球萎缩,在后顶叶和颞叶皮质突出,额叶皮质和前突区域中等。18F-FDGPET显像显示右侧顶叶葡萄糖代谢降低,temporal,和右前肌的额叶皮质。在DaTQUANT,壳核似乎不是病理性的。神经心理学测试显示记忆和视觉感知缺陷。CSFtau和淀粉样蛋白测量没有显示出明确的病理值。CSF和OM样品中α-突触核蛋白的RT-QuIC为阳性。QEEG分析显示后验导数中的前α主导频率,典型的DLB早期阶段。
未经证实:虽然本患者的临床诊断为可能的CBS,DLB的意外阳性生物标志物提示多种病理并存.
UNASSIGNED: An 82-year-old right-handed man, a retired teacher, reported the occurrence, three years earlier, of difficulties in moving his left arm and foot, tremor in his left hand, and gestures of the left upper limb that appeared to be independent of the patient\'s will.
UNASSIGNED: We describe an unusual
case of corticobasal syndrome (CBS) showing disease-associated biomarkers of dementia with Lewy bodies (DLB).
UNASSIGNED: Clinical, neuropsychological, imaging, and biomarker evaluations were conducted, including tau and amyloid-β levels in the cerebrospinal fluid (CSF) and a RT-QuIC assay for α-synuclein both in the CSF and olfactory mucosa (OM), as well as a QEEG assessment.
UNASSIGNED: The patient presented resting tremor, mild extrapyramidal hypertonus, mild bradykinesia on the left side, and severe apraxia on the left upper limb. Brain MRI showed a diffuse right hemisphere atrophy which was prominent in the posterior parietal and temporal cortices, and moderate in the frontal cortex and the precuneus area. 18F-FDG PET imaging showed reduced glucose metabolism in the right lateral parietal, temporal, and frontal cortices with involvement of the right precuneus. The putamen did not appear to be pathological at DaTQUANT. Neuropsychological tests showed memory and visual-perceptual deficits. CSF tau and amyloid measurements did not show clear pathological values. RT-QuIC for α-synuclein in CSF and OM samples were positive. The QEEG analysis showed a pre-alpha dominant frequency in posterior derivations, typical of early stages of DLB.
UNASSIGNED: Although in the present patient the clinical diagnosis was of probable CBS, unexpectedly positive biomarkers for DLB suggested the co-presence of multiple pathologies.