Aichner F
Fortschr Neurol Psychiatr. 1984 Nov;52(11):375-97. doi: 10.1055/s-2007-1002207.
The KBS was first described on the basis of experimental bilateral temporal lobectomy in monkeys. Extrapolating any syndrome from monkey to man is frought with controversy, but the 53 patients with KBS reported here are significant for the completeness of its expression and the opportunity to follow its evolution. The rarity of the human KBS is attested to because less than 200 cases have been reported in the literature. The KBS in man, presented here as a clinical entity, includes the following symptoms: (1) Increased oral activity: a strong tendency to examine all objects orally, putting objects in mouth, licking, biting, chewing, touching with lips. (2) Hypersexuality (hetero-, homo-, autosexual). (3) Hypermetamorphosis: to touch everything in sight, to attend to every visual stimulus with grasping, hugging movements. (4) Memory disorders. (5) Placidity, flattened affect and pet-like compliance with lack of aggressive affective behaviour. (6) Loss of recognition of people (7) Bulimia. The KBS in man shows little etiologic specificity and has been seen in our cases with posttraumatic encephalopathy, encephalitis, anoxia and subarachnoid hemorrhage as well as with Pick's disease, Alzheimer's disease, bilateral temporal infarction, partial or complete removal of both temporal lobes, hypoglycemia, adrenoleucodystrophy, porphyria and neuroleptic medication reported in previous studies. Since the lesions in man are not as extensive and not as consistently localized as in animal experiments, it is not surprising that all abnormal behaviour deficits seen in monkey following bitemporal lobectomy have seldom been exactly reproduced in man. The full syndrome is not usually seen. Indicating a partial KBS in man, combinations of three or more different elements must be present concerning cognitive, behavioural and emotional deficits. In most cases of human KBS the evolution demonstrates a transient profile but in few patients a partial KBS is remaining. The dynamics in the development of a KBS show a delineation between a pre-stage, full-stage, and remission stage. These stages only occur in posttraumatic cases and have not been seen in KBS of other etiology. The results of CT-investigations in 50 patients with KBS mainly show nonspecific abnormalities (brain atrophy, focal lesion, subdural hematoma, hydrocephalus) and the exact anatomic localization of the lesions necessary to produce KBS could not be demonstrated in particular cases by CT. Also no specific EEG-pattern is associated with KBS and the EEG often shows a wide range of findings.(ABSTRACT TRUNCATED AT 400 WORDS)
Klüver-Bucy综合征(KBS)最初是在对猴子进行双侧颞叶切除的实验基础上被描述的。将任何一种综合征从猴子类推到人类都充满争议,但本文报道的53例KBS患者因其症状表现的完整性以及追踪其病情发展的机会而具有重要意义。人类KBS的罕见性得到了证实,因为文献报道的病例不足200例。本文作为一种临床实体呈现的人类KBS包括以下症状:(1)口腔活动增加:有强烈的用口检查所有物体的倾向,将物体放入口中、舔、咬、咀嚼、用嘴唇触碰。(2)性欲亢进(异性、同性、自体性欲)。(3)动作增多:触碰视野内的一切,对每个视觉刺激都做出抓握、拥抱动作。(4)记忆障碍。(5)平静、情感平淡以及像宠物一样顺从,缺乏攻击性情感行为。(6)对人的识别能力丧失。(7)贪食症。人类KBS几乎没有病因特异性,在我们的病例中,它可见于创伤后脑病、脑炎、缺氧和蛛网膜下腔出血,以及先前研究报道的匹克氏病、阿尔茨海默病、双侧颞叶梗死、双侧颞叶部分或完全切除、低血糖、肾上腺脑白质营养不良、卟啉病和使用抗精神病药物之后。由于人类的病变不像动物实验中那样广泛且定位一致,所以毫不奇怪,猴子双侧颞叶切除后出现的所有异常行为缺陷在人类中很少能完全重现。通常不会出现完整的综合征。若人类出现三种或更多不同的认知、行为和情感缺陷组合,则提示为部分性KBS。在大多数人类KBS病例中,病情发展呈短暂性,但少数患者会残留部分KBS。KBS发展过程中的动态变化表现为前期、完全期和缓解期的区分。这些阶段仅出现在创伤后病例中,在其他病因导致的KBS中未见。对50例KBS患者进行CT检查的结果主要显示非特异性异常(脑萎缩、局灶性病变、硬膜下血肿、脑积水),在某些病例中,CT无法明确显示产生KBS所需病变的确切解剖定位。而且也没有与KBS相关的特异性脑电图模式,脑电图往往显示出广泛的结果。(摘要截选至400字)