Department of Experimental Psychology, University of Ghent, Ghent, Belgium.
Cerebellum. 2012 Jun;11(2):587-92. doi: 10.1007/s12311-011-0322-x.
The posterior fossa syndrome (PFS) consists of transient cerebellar mutism, cognitive symptoms and neurobehavioural abnormalities that typically develop in children following posterior fossa tumour resection. Although PFS has been documented in more than 350 paediatric cases, reports of adult patients with a vascular aetiology are extremely rare. In addition, the pathophysiological substrate of the syndrome remains unclear. We report an adult patient with PFS after surgical evacuation of a cerebellar bleeding. After 45 days of (akinetic) mutism, the patient's cognitive and behavioural profile closely resembled the "cerebellar cognitive-affective syndrome". A quantified SPECT study showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, executive functioning, spatial cognition and affective regulation. We hypothesize that cerebello-cerebral diaschisis might be an important pathophysiological mechanism underlying akinetic mutism, cognitive deficits and behavioural-affective changes in adult patients with PFS.
后颅窝综合征（PFS）包括短暂性小脑缄默症、认知症状和神经行为异常，这些症状通常发生在儿童后颅窝肿瘤切除后。尽管已有超过 350 例小儿病例记录，但血管性病因的成人患者报告极为罕见。此外，该综合征的病理生理基础仍不清楚。我们报告了一例成人患者，在小脑出血手术后出现 PFS。缄默（无动性）45 天后，患者的认知和行为特征与“小脑认知情感综合征”非常相似。一项定量 SPECT 研究显示，在解剖学上疑似与语言动力学、执行功能、空间认知和情感调节相关的额顶区域存在灌注不足。我们假设，大脑-小脑失联络可能是导致成年 PFS 患者出现无动性缄默、认知缺陷和行为情感变化的重要病理生理机制。
