Cuny M L, Pallone M, Piana H, Boddaert N, Sainte-Rose C, Vaivre-Douret L, Piolino P, Puget S
Laboratory of Memory and Cognition, Institute of Psychology, University Paris Descartes, Sorbonne Paris Cité, Paris, France.
CESP, University Paris-Sud, UVSQ, INSERM 1018, University Paris-Saclay, Villejuif, France.
Childs Nerv Syst. 2017 Jan;33(1):135-141. doi: 10.1007/s00381-016-3285-x. Epub 2016 Nov 10.
Posterior fossa arachnoid cysts (PFAC) are mostly considered as benign lesions of the cerebellum. Although many studies have shown the major role of the cerebellum in modulating movement, language, cognition, and social interaction, there are few studies on the cognitive impact and surgical decompression of PFAC.
We present the cases of two brothers successively diagnosed with PFAC and neuropsychological delay. After multidisciplinary discussion with the boys' parents, it was decided to drain these lesions. Clinical signs, cerebral images, and neuropsychological status were assessed on admission and then 1 and 3 years after surgery.
At presentation, both children had mild cerebellar signs, associated with cognitive and visual-motor impairments and academic regression. CT scans revealed retrovermian cysts, which were shunted. Post-operatively, both brothers demonstrated improved visual-motor skills and behavior. At follow-up, we observed disappearance of dysarthria and academic delay and significant improvement in cognition especially at the intelligence scale and in language. Fine motor skills had improved but remained slower than the average and writing skills appeared limited.
Except for PFAC which impair cerebrospinal fluid circulation or which are responsible for a significant mass effect, most PFAC are usually considered as "asymptomatic" and do not require surgical treatment. The two cases reported herein suggest that these lesions might be responsible for some associated but potentially reversible neuropsychological impairment. In the future, clinical assessment should include neuropsychological evaluation to help inform decision for surgical decompression in these children with PFAC.
后颅窝蛛网膜囊肿(PFAC)大多被视为小脑的良性病变。尽管许多研究表明小脑在调节运动、语言、认知和社会互动方面起主要作用,但关于PFAC的认知影响和手术减压的研究却很少。
我们报告了两兄弟先后被诊断为PFAC并伴有神经心理发育迟缓的病例。在与患儿父母进行多学科讨论后,决定对这些病变进行引流。在入院时以及术后1年和3年对临床体征、脑部影像和神经心理状态进行评估。
就诊时,两个孩子都有轻度小脑体征,伴有认知和视运动障碍以及学业退步。CT扫描显示蚓部后方囊肿,进行了分流手术。术后,两兄弟的视运动技能和行为均有改善。随访时,我们观察到构音障碍和学业延迟消失,认知能力有显著改善,尤其是在智力量表和语言方面。精细运动技能有所提高,但仍比平均水平慢,书写技能似乎有限。
除了那些影响脑脊液循环或产生明显占位效应的PFAC外,大多数PFAC通常被认为是“无症状的”,不需要手术治疗。本文报道的两个病例表明,这些病变可能是一些相关但潜在可逆的神经心理损害的原因。未来,临床评估应包括神经心理评估,以帮助为这些患有PFAC的儿童的手术减压决策提供依据。