Oktem-Tanör O, Baykan-Kurt B, Gürvit I H, Akman-Demir G, Serdaroğlu P
University of Istanbul, Istanbul Faculty of Medicine, Department of Neurology, Turkey.
J Neurol. 1999 Feb;246(2):113-9. doi: 10.1007/s004150050317.
We analyzed the data obtained from neuropsychological evaluations of 12 neuro-Behcet Disease (NBD) patients who had been followed up for 35.6+/-23.7 months with successive neuropsychological testing by a comprehensive battery. Memory impairment, which seems to stem basically from a retrieval deficit, was the major finding in this series. The most severely affected memory process was delayed recall, being impaired in all of the patients in the verbal and/or visual modalities. This was followed closely by an impairment in the process of acquisition and storage. In addition to the memory impairment, a "clinical impression of personality change" toward either disinhibition or apathy was seen in 8 of the 12 patients. Attention deficit was of the third highest frequency and was present in 7 patients, followed by deficits of executive functions of frontal system which were present in 5. Other cognitive domains were rarely involved. Neuropsychological status deteriorated insidiously, regardless of the neurological attacks during the follow-up period in most of the patients. Furthermore, our observations also showed the presence of cognitive decline prior to detectable lesions on CT or MRI, emphasizing the need for neuropsychological testing in NBD patients. The late stages of the disease seem to be reflected in MRI as an enlargement of the third ventricle and atrophy of the upper brainstem, which could be compatible with memory loss. Our series, a rather selected group, suggests that NBD can be associated with a special pattern of cognitive deficit, especially memory loss and personality change. The designation of any specific neurobehavioral syndrome for NBD, however, awaits further study.
我们分析了12例神经白塞病（NBD）患者的神经心理学评估数据，这些患者接受了连续35.6±23.7个月的综合成套神经心理学测试随访。记忆障碍似乎主要源于提取缺陷，是本系列研究的主要发现。受影响最严重的记忆过程是延迟回忆，在所有患者的言语和/或视觉模式中均受损。其次是获取和存储过程受损。除记忆障碍外，12例患者中有8例出现了向去抑制或冷漠方向的“人格改变临床印象”。注意力缺陷的出现频率排第三，有7例患者存在，其次是额叶系统执行功能缺陷，有5例患者存在。其他认知领域很少涉及。在大多数患者中，无论随访期间是否发生神经发作，神经心理状态都在不知不觉中恶化。此外，我们的观察还显示，在CT或MRI上可检测到病变之前就存在认知衰退，这强调了对NBD患者进行神经心理学测试的必要性。疾病晚期在MRI上似乎表现为第三脑室扩大和上脑干萎缩，这可能与记忆丧失有关。我们的系列研究是一个经过挑选的群体，表明NBD可能与一种特殊的认知缺陷模式有关，尤其是记忆丧失和人格改变。然而，为NBD指定任何特定的神经行为综合征尚有待进一步研究。
