Department of Paediatric Neurology, Erasmus University/Sophia Children's Hospital, Dr. Molewaterplein 60, Rotterdam, The Netherlands.
Cortex. 2010 Jul-Aug;46(7):933-46. doi: 10.1016/j.cortex.2009.10.007. Epub 2009 Oct 29.
The Posterior Fossa Syndrome (PFS) may occur in children after resection of cerebellar tumours. The most common feature is mutism, but also oropharyngeal dyspraxia, emotional lability and neuropsychiatric symptoms occur. We analysed the spectrum of behavioural abnormalities, speech and language characteristics during PFS.
In order to identify PFS, all children with a cerebellar tumour admitted to our centre in the study period were prospectively assessed before and after tumour surgery. In the case of PFS, children were systematically followed by means of a standard protocol that included a daily neurological examination and assessment of speech behaviour. Speech was recorded on videotape before and immediately after surgery, and in cases of PFS at as short possible intervals for 4 weeks and subsequently every second week until the recurrence of speech and normalisation of behaviour. Data regarding clinical and behavioural features, duration of symptoms and mode of recovery were collected. Pre- and postoperative MRI data were studied. In 13 children with and two children without PFS a (99m)Tc-hexamethylpropyleneamine oxime-Single Photon Emission Tomography (SPECT) scan could be performed.
PFS occurred in 41 of 148 children. During recovery all children were dysarthric, but only in a few speech features specific for cerebellar dysarthria occurred. A significant correlation was found between duration of mutism and severity of neurological symptoms. Significant correlations were also found between duration of mutism and abnormalities on SPECT scans of the left temporal lobe, the left and right basal nuclei, and the right frontal lobe.
In this study, impairments of higher cognitive functions were observed in the context of PFS. They varied in severity and composition between children with symptoms fitting into the spectrum of the Cerebellar Cognitive Affective Syndrome. SPECT scan findings suggest that these impairments are secondary to supratentorial metabolic hypofunction following cerebellar surgery.
后颅窝综合征（PFS）可能发生在小脑肿瘤切除术后的儿童中。最常见的特征是缄默症，但也会出现构音障碍、情绪不稳和神经精神症状。我们分析了 PFS 期间行为异常、言语和语言特征的范围。
为了确定 PFS，研究期间所有被收入我院的小脑肿瘤患儿均前瞻性评估肿瘤手术前后。如果发生 PFS，通过包括每日神经系统检查和言语行为评估的标准方案对患儿进行系统随访。在手术前和手术后立即对言语进行录像，并在 PFS 情况下在最短 4 周内且随后每两周进行一次，直到言语恢复正常且行为正常。收集了有关临床和行为特征、症状持续时间和恢复方式的数据。研究了术前和术后的 MRI 数据。在 13 例伴有和 2 例不伴有 PFS 的患儿中进行了（99m）Tc-六甲基丙烯胺肟单光子发射断层扫描（SPECT）扫描。
PFS 发生在 148 例患儿中的 41 例。在恢复过程中，所有患儿都有构音障碍，但只有少数出现与小脑构音障碍相关的特定言语特征。缄默症的持续时间与神经系统症状的严重程度之间存在显著相关性。缄默症的持续时间与左侧颞叶、左侧和右侧基底核以及右侧额叶 SPECT 扫描异常之间也存在显著相关性。
在本研究中，在 PFS 背景下观察到了更高认知功能的损伤。它们在符合小脑认知情感综合征谱的症状患儿之间在严重程度和组成上存在差异。SPECT 扫描结果表明，这些损伤是小脑手术后额上代谢功能低下的继发表现。
