Die Phänomenologie des nach Klüver und Bucy benannten Syndroms beim Menschen*

Abstract

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)

Partial Kluver-Bucy Syndrome: Two Cases

“…EEG and brain imaging are frequently abnormal, but not specific for KBS. 7 The current treatments for KBS reflect KBS pathophysiology. Burns and colleagues 13 found that in a sample of 178 patients with Alzheimer disease, 174 exhibited at least partial signs of KBS.…”

Organische Psychosyndrome

“…Das urspr€ unglich tierexperimentell nach bilateraler Ablation des Temporallappens und Rhinenzephalons bei Rhesusaffen identifizierte Kl€ uver-Bucy-Syndrom wird beim Menschen in aller Regel nur in inkompletter Ausprägung beobachtet, wobei dort gleichzeitig komplexere Verhaltensstörungen im Sinne von Enthemmungsphänomenen beobachtet werden (Aichner 1984). Im Einzelnen zählen zu den Symptomen eine visuelle Agnosie mit Verlust des Erkennens von Personen und Objekten, vermehrte Ablenkbarkeit durch optische Reize, Hyperoralität mit oralen Automatismen und Hypersexualität, aber auch motorische Verlangsamung, Antriebs-und Affektverflachung sowie Lern-und Gedächtnisstörungen.…”

„Hypermetamorphosis“

“…In weiteren Einzelfällen wurde die Korrespondenz zu den tierexperimentellen Beobachtungen kontrovers diskutiert [1,10], und das Klüver-Bucy-Syndrom beim Menschen gilt meist als Kuriosität. Medline-Recherchen weisen allerdings weit über 100 Literaturstellen nach: häufig liegen Herpesenzephalitis oder ausgedehnte Kontusionen bei Schädel-Hirn-Trauma zugrunde.…”

“…Aber nirgends hält er Stich, kaum erschienen, ist er schon wieder verschwunden. Er rennt in das Schauspiel, in Concerte, in Spielhäuser, oder je nach der gesellschaftlichen Categorie, von einem Bierhause oder Branntweinladen zum andern, schwärmt für Cigarren, die er kaum angeraucht wieder wegwirft ... Bei alledem spricht der Kranke keinen Unsinn, er kennt seine Umgebung, er begeht keine gesetzwidrige Handlungen und erscheint daher seiner Umgebung höchstens Abb 1. 8 Repräsentative Fotographie von…”