TRS-A National Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesoddtangen, Norway.
Disabil Rehabil. 2012;34(9):740-6. doi: 10.3109/09638288.2011.619617. Epub 2011 Oct 17.
Myelomeningocele (MMC) is caused by incomplete neural tube development, affecting physical, cognitive and adaptive functioning. The aim of this study was to characterize women and men with problematic psychosocial adaptation with respect to cognitive functions and psychological symptoms. It was expected that cognitive functions, especially the executive functions, were mostly impaired. In addition, the clinical history, the neurological and the psychological functioning was investigated.
Twelve participants (six men, six women; age range 24-41 years) with MMC were recruited from TRS National Resource Centre for Rare Disorders. Inclusion criteria were (1) uncompleted upper secondary school, (2) unemployment and (3) lack of social relationships. The participants underwent a systematic clinical history, a clinical neurological examination, a neuropsychological assessment and questionnaires regarding cognitive and psychological functioning.
All participants had major neurological deficits and most of them had hydrocephalus and Chiari-II malformation. Cognitive deficits were present in a range of domains, especially the executive functions. The neuropsychological findings showed no obvious difference between men and women. Symptoms of psychopathology were more pronounced in women. The men were less capable to structure daily living.
The neuropsychological findings disclosed that the executive functions were most impaired. Future research should be directed towards cognitive rehabilitation.
脊髓脊膜膨出(MMC)是由神经管发育不完全引起的,会影响身体、认知和适应功能。本研究的目的是描述在认知功能和心理症状方面存在心理社会适应问题的女性和男性。预计认知功能,尤其是执行功能,会受到较大影响。此外,还对临床病史、神经功能和心理功能进行了调查。
从 TRS 国家罕见疾病资源中心招募了 12 名 MMC 参与者(6 名男性,6 名女性;年龄 24-41 岁)。纳入标准为(1)未完成高中教育,(2)失业和(3)缺乏社会关系。参与者接受了系统的临床病史、临床神经检查、神经心理学评估以及认知和心理功能问卷。
所有参与者都有严重的神经功能缺陷,大多数人都有脑积水和 Chiari-II 畸形。认知缺陷存在于多个领域,尤其是执行功能。神经心理学发现男性和女性之间没有明显差异。女性的精神病理学症状更为明显。男性在日常生活的组织能力方面较差。
神经心理学发现表明执行功能受到的影响最大。未来的研究应针对认知康复。