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儿童后天性出血性小脑病变的长期后遗症。

Long-term sequelae after acquired pediatric hemorrhagic cerebellar lesions.

作者信息

Wingeier Kevin, Bigi Sandra, El-Koussy Marwan, Heinks-Maldonado Theda, Boltshauser Eugen, Steinlin Maja

机构信息

Department of Pediatric Neurology (J212), University Children's Hospital, Inselspital, 3010, Bern, Switzerland.

出版信息

Childs Nerv Syst. 2011 Jun;27(6):923-31. doi: 10.1007/s00381-010-1357-x. Epub 2010 Dec 29.

Abstract

PURPOSE

The aim of the present study was to assess cognitive, affective, and motor long-term sequelae after acquired focal pediatric cerebellar lesions.

METHODS

Eight patients with a history of isolated acquired hemorrhagic cerebellar lesions before the age of 13 participated in this study. All participants underwent a neurologic examination, including the Zurich Neuromotor Assessment (ZNA) and the International Cooperative Ataxia Rating Scale (ICARS). Cognitive functions have been evaluated with a general cognitive assessment and an extensive neuropsychological battery. Furthermore, patients and parents filled in questionnaires about quality of life and possible behavioral or emotional problems.

RESULTS

The results revealed that all patients exhibited motor problems (ZNA). Most participants had further restricted oculomotor movements (ICARS). Age at injury and the full scale IQ were significantly positively correlated (Pearson correlation 0.779; p = 0.023). Conversely, no overall neuropsychological profile could be identified except for marginally reduced reaction times and susceptibility to interference. In addition, borderline results in semantic and phonemic word fluency tasks were apparent. A dysexecutive syndrome was diagnosed in one patient. However, verbal performance and reading abilities were non-pathologic in all participants. The patients reported having a good quality of life without major physical restrictions.

CONCLUSIONS

Emotional disturbances and the presence of a mild cerebellar cognitive affective syndrome (as frequently described in adult patients) could only be confirmed in adolescents with vermis lesions. Nevertheless, in laboratory conditions, neuropsychological impairments were present in all patients. Heterogeneity of age at injury and exact lesion site may have led to interpersonal differences in neuropsychological outcome.

摘要

目的

本研究旨在评估儿童后天性局灶性小脑病变后的认知、情感和运动长期后遗症。

方法

8例13岁前有孤立性后天性出血性小脑病变病史的患者参与了本研究。所有参与者均接受了神经系统检查,包括苏黎世神经运动评估(ZNA)和国际合作共济失调评定量表(ICARS)。通过一般认知评估和广泛的神经心理测验对认知功能进行了评估。此外,患者和家长填写了关于生活质量以及可能的行为或情绪问题的问卷。

结果

结果显示所有患者均表现出运动问题(ZNA)。大多数参与者还有进一步受限的眼球运动(ICARS)。受伤时的年龄与全量表智商显著正相关(Pearson相关系数0.779;p = 0.023)。相反,除了反应时间略有缩短和易受干扰外,未发现整体神经心理特征。此外,语义和音素词流畅性任务的临界结果很明显。1例患者被诊断为执行功能障碍综合征。然而,所有参与者的言语表现和阅读能力均无病理学异常。患者报告生活质量良好,没有重大身体限制。

结论

仅在患有蚓部病变的青少年中证实存在情绪障碍和轻度小脑认知情感综合征(如成年患者中常见)。然而,在实验室条件下,所有患者均存在神经心理损害。受伤时年龄和确切病变部位的异质性可能导致神经心理结果的个体差异。

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