Baillieux Hanne, De Smet Hyo Jung, Lesage Geoffrey, Paquier Philippe, De Deyn Peter P, Mariën Peter
Department of Linguistics, Vrije Universiteit Brussel, Brussels.
Cerebellum. 2006;5(4):289-95. doi: 10.1080/14734220601009606.
The posterior fossa syndrome (PFS) consists of a variety of symptoms, including cerebellar mutism, behavioral disturbances and personality changes. We report longitudinal clinical, neuroradiological and neurobehavioral findings in a 19-year-old left-handed patient, diagnosed with attentional deficit hyperactivity disorder (ADHD) at the age of 12, who underwent posterior fossa tumor resection. Although the patient did not develop cerebellar mutism after surgery, marked apathy and emotional indifference, urinary retention, eye-lid apraxia and visual hallucinosis became apparent after a brief interval of normal functioning. Based on these findings it is argued that the PFS might be considered a semiological heterogeneous condition with variable clinical expressions. Long-term follow-up investigations revealed subtle, but significant cognitive and affective deficits, resembling the cerebellar cognitive affective syndrome in adults. As demonstrated by functional neuroimaging studies with SPECT, symptoms were associated with perfusional deficits in the anatomoclinically suspected supratentorial regions, reflecting the distant impact of the cerebellum on cognitive and affective functions.
后颅窝综合征(PFS)由多种症状组成,包括小脑缄默症、行为障碍和人格改变。我们报告了一名19岁左利手患者的纵向临床、神经放射学和神经行为学研究结果,该患者12岁时被诊断为注意力缺陷多动障碍(ADHD),接受了后颅窝肿瘤切除术。尽管患者术后未出现小脑缄默症,但在短暂的正常功能期后,明显的冷漠和情感淡漠、尿潴留、眼睑失用和视幻觉变得明显。基于这些发现,有人认为PFS可能被视为一种具有不同临床表型的症状学异质性疾病。长期随访研究发现了细微但显著的认知和情感缺陷,类似于成人的小脑认知情感综合征。单光子发射计算机断层扫描(SPECT)功能神经影像学研究表明,症状与解剖学上疑似幕上区域的灌注不足有关,反映了小脑对认知和情感功能的远距离影响。