McMonagle Paul, Blair Mervin, Kertesz Andrew
Department of Cognitive Neurology, University of Western Ontario, St Joseph's Hospital, London, Ontario, Canada.
Neurology. 2006 Oct 24;67(8):1444-51. doi: 10.1212/01.wnl.0000240215.43492.01.
To describe language impairment in the corticobasal degeneration syndrome (CBDS) presenting as either a cognitive or motor disorder, to compare the evolution of aphasia in CBDS with primary progressive aphasia (PPA), and to examine whether the side of maximal cerebral atrophy or akinesia reflects the severity of aphasia.
We divided 40 patients with CBDS according to motor or cognitive onsets and conducted detailed language assessments with the Western Aphasia Battery (WAB). We analyzed scores according to the side of atrophy and motor rigidity. Longitudinal performance over three annual assessments was compared against matched patients with PPA and Alzheimer disease.
Language at baseline was more impaired in cognitive than motor-onset CBDS but there was no correlation between the side of atrophy or motor impairment and the WAB. Serial assessment (n = 19) showed a similar evolution of aphasia in cognitive-onset CBDS and PPA and delayed aphasia in motor-onset CBDS.
Aphasia is common in the corticobasal degeneration syndrome but there is little correlation with the laterality of clinical deficits. Cognitive-onset corticobasal degeneration syndrome and primary progressive aphasia are similar such that their aphasia appears identical.
描述表现为认知或运动障碍的皮质基底节变性综合征（CBDS）中的语言障碍，比较CBDS中失语症与原发性进行性失语症（PPA）的演变情况，并检查大脑最大萎缩或运动不能的一侧是否反映失语症的严重程度。
我们根据运动或认知起病情况将40例CBDS患者进行分组，并用西方失语成套测验（WAB）进行详细的语言评估。我们根据萎缩和运动僵硬的一侧分析得分。将三年年度评估的纵向表现与匹配的PPA患者和阿尔茨海默病患者进行比较。
基线时，认知起病的CBDS患者的语言障碍比运动起病的患者更严重，但萎缩或运动障碍的一侧与WAB之间没有相关性。系列评估（n = 19）显示，认知起病的CBDS和PPA中的失语症演变相似，而运动起病的CBDS中失语症进展延迟。
失语症在皮质基底节变性综合征中很常见，但与临床缺陷的偏侧性几乎没有相关性。认知起病的皮质基底节变性综合征和原发性进行性失语症相似，因此它们的失语症表现相同。
