Center for Memory and Attention Disorders.
Sackler School of Medicine.
Alzheimer Dis Assoc Disord. 2019 Jul-Sep;33(3):279-281. doi: 10.1097/WAD.0000000000000283.
Cognitive deficits beyond memory impairment, such as those affecting language production or executive functioning, can be useful in clinically distinguishing between dementia syndromes. We tested the hypothesis that Ashkenazi Jewish (AJ) patients who have dementia with Lewy bodies (DLB) and carry glucocerebrosidase (GBA) mutations will have verbal fluency deficits different from those found in Alzheimer disease (AD), whereas AJ patients with DLB who have no GBA mutations will have similar deficits in verbal fluency to those found in AD. We compared performance in phonemic and semantic verbal fluency tasks in 44 AJ patients with DLB and 20 patients with AD, matched for age, education, and age of immigration. All groups were found to have a deficit in semantic verbal fluency. On conducting the phonemic task, patients with DLB who carried GBA mutations scored more poorly than patients with AD, whereas DLB-noncarriers performed similarly to patients with AD. We suggest that verbal fluency tasks could serve as a possible clinical marker to subtype patients with DLB, with phonemic fluency being a marker for GBA-associated DLB.
除了记忆损伤以外的认知缺陷，如语言产生或执行功能方面的缺陷，有助于临床上鉴别痴呆综合征。我们检验了这样一个假设，即携带葡萄糖脑苷脂酶（GBA）突变的阿什肯纳兹犹太（AJ）族痴呆路易体病（DLB）患者会出现不同于阿尔茨海默病（AD）的语言流畅性缺陷，而 AJ 族 DLB 患者无 GBA 突变者则会出现与 AD 相似的语言流畅性缺陷。我们比较了 44 名 AJ 族 DLB 患者和 20 名 AD 患者在语音和语义流畅性任务中的表现，这些患者在年龄、教育程度和移民年龄方面相匹配。所有组均发现语义流畅性缺陷。在进行语音任务时，携带 GBA 突变的 DLB 患者的得分明显低于 AD 患者，而非携带者的表现与 AD 患者相似。我们认为语言流畅性任务可作为亚组患者的可能临床标志物，语音流畅性是 GBA 相关 DLB 的标志物。
