Pillon B, Blin J, Vidailhet M, Deweer B, Sirigu A, Dubois B, Agid Y
INSERM U 289, Hôpital de la Salpêtrière, Paris, France.
Neurology. 1995 Aug;45(8):1477-83. doi: 10.1212/wnl.45.8.1477.
The pattern of cortical and subcortical neuropathologic lesions in corticobasal degeneration (CBD) should predict a specific cognitive profile in this disease. To characterize this profile and to determine its specificity by comparison with progressive supranuclear palsy (PSP) and senile dementia of the Alzheimer's type (SDAT), we used an extensive neuropsychological battery assessing global efficiency, executive functions, various tests of encoding and retrieval, dynamic motor organization, and upper limb praxis. We compared the performance of patients with CBD (n = 15) with that of controls (n = 19) matched for age and education, and with that of patients with PSP and SDAT (15 in each group), matched for severity of dementia and depression. Patients with CBD showed: (1) a moderate global deterioration; (2) a dysexecutive syndrome similar to that of patients with PSP and more severe than in SDAT; (3) explicit learning deficits, without retention difficulties and easily compensated by using the same semantic cues at encoding and retrieval as in PSP; this was in contrast with SDAT where cued recall and recognition were also impaired; (4) disorders of dynamic motor execution (temporal organization, bimanual coordination, control, and inhibition) similar to those of patients with PSP and not in SDAT; (5) asymmetric praxis disorders (posture imitation, symbolic gesture execution, and object utilization) that were not observed in PSP or SDAT. Patients with CBD show a specific neuropsychological pattern associating a dysexecutive syndrome, likely due to degeneration of the basal ganglia and prefrontal cortex, and asymmetric praxis disorders, which might be related to premotor and parietal lobe lesions. This neuropsychological profile may help to distinguish this condition clinically from other neurodegenerative diseases.
皮质基底节变性（CBD）中皮质和皮质下神经病理损伤模式应能预测该疾病的特定认知特征。为了描述这一特征并通过与进行性核上性麻痹（PSP）和阿尔茨海默病型老年痴呆（SDAT）相比较来确定其特异性，我们使用了一套广泛的神经心理学测试组合，评估整体效能、执行功能、各种编码和检索测试、动态运动组织以及上肢运用能力。我们将CBD患者（n = 15）的表现与年龄和教育程度相匹配的对照组（n = 19）进行比较，并与痴呆和抑郁严重程度相匹配的PSP和SDAT患者（每组15例）进行比较。CBD患者表现出：（1）中度的整体功能衰退；（2）与PSP患者相似且比SDAT患者更严重的执行功能障碍综合征；（3）明确的学习缺陷，不存在记忆保留困难，并且通过在编码和检索时使用与PSP相同的语义线索能够轻松弥补；这与SDAT不同，在SDAT中线索回忆和识别也受损；（4）与PSP患者相似但SDAT患者不存在的动态运动执行障碍（时间组织、双手协调、控制和抑制）；（5）PSP或SDAT中未观察到的不对称运用障碍（姿势模仿、象征性手势执行和物体使用）。CBD患者表现出一种特定的神经心理学模式，伴有可能由于基底节和前额叶皮质变性导致的执行功能障碍综合征以及可能与运动前区和顶叶病变相关的不对称运用障碍。这种神经心理学特征可能有助于在临床上将该疾病与其他神经退行性疾病区分开来。
