Department of Neurology, ZNA Middelheim Hospital, Antwerp, Belgium.
Cortex. 2013 Jan;49(1):284-300. doi: 10.1016/j.cortex.2011.06.018. Epub 2011 Jul 22.
Although the posterior fossa syndrome (PFS) can be considered as an aetiologically heterogeneous condition affecting children and adults, it most often occurs in paediatric patients after cerebellar tumour surgery. In patients with a tumoural aetiology, the syndrome is typically characterised by a short symptom-free postoperative period followed by mutism of variable duration and behavioural and affective changes. More than 200 paediatric cases have been described but reports of adult patients are extremely rare. This paper discusses PFS in adults on the basis of a comprehensive literature survey and describes the pre- and postoperative findings in a new adult patient. In the preoperative phase, cognitive, behavioural and affective abnormalities were identified, matching a diagnosis of cerebellar cognitive affective syndrome (CCAS) (Schmahmann and Sherman, 1998; Schmahmann, 2004). The immediate postoperative course was characterised by prefrontal-like behavioural and affective abnormalities, peduncular hallucinations and confusion evolving to psychosis. Akinetic mutism subsequently developed, lasted for 12 days and then alternated with episodes of diminished responsiveness in which pathological laughing and crying (PLC) occurred. Akinetic mutism resolved after treatment with a non-ergoline dopamine-agonist but CCAS persisted during longitudinal follow-up. From a semiological point of view "relapsing-remitting akinetic mutism" and PLC in our patient might add relevant information to current insights in the clinical expression of the PFS. As evidenced by a close parallelism between single photon emission computed tomography (SPECT) and clinical findings, CCAS as well as PFS seem to reflect functional disruption of the cerebello-cerebral network involved in cognitive, behavioural and affective functions. These findings may indicate that both syndromes share overt semiological resemblances and a common pathophysiological substrate. Consequently, CCAS and PFS may both be regarded as cerebellar-induced clinical conditions showing different aspects of a spectrum that range in degree of severity and symptom duration.
尽管后颅窝综合征（PFS）可被视为一种病因学上异质的疾病，影响儿童和成人，但它最常发生在小脑肿瘤手术后的儿科患者中。在肿瘤病因的患者中，该综合征的特征通常是术后无症状期短，随后出现不同持续时间的缄默、行为和情感变化。已经描述了超过 200 例儿科病例，但成人病例的报告极为罕见。本文基于全面的文献调查讨论了成人 PFS，并描述了一名新的成年患者的术前和术后发现。在术前阶段，发现了认知、行为和情感异常，符合小脑认知情感综合征（CCAS）的诊断（Schmahmann 和 Sherman，1998；Schmahmann，2004）。术后即刻病程的特点是出现类似前额叶的行为和情感异常、脚间幻觉和混乱，发展为精神病。随后出现无动性缄默症，持续了 12 天，然后间歇性地出现反应能力下降，出现病理性哭笑（PLC）。无动性缄默症在用非麦角碱多巴胺激动剂治疗后得到缓解，但 CCAS 在纵向随访期间持续存在。从半侧学的角度来看，我们患者的“复发性无动性缄默症”和 PLC 可能为 PFS 的临床表现提供了当前见解的相关信息。单光子发射计算机断层扫描（SPECT）和临床发现之间的紧密平行关系表明，CCAS 以及 PFS 似乎反映了涉及认知、行为和情感功能的小脑-大脑网络的功能障碍。这些发现可能表明，这两种综合征在明显的半侧学相似性和共同的病理生理基础上具有共同的表现。因此，CCAS 和 PFS 都可能被视为小脑引起的临床情况，表现出严重程度和症状持续时间不同的表现谱。
