Objective

To describe the frequency, motor phenotype, clinical patterns and functional consequences of dystonia in patients with cerebral palsy (CP) in the setting of periventricular leukomalacia.

Methods

Retrospective analysis of a cohort of 31 patients with CP and periventricular leukomalacia. Gross Motor Function Classification System (GMFCS) and Manual Ability Classification System (MACS) were used to classify functional ability. Spasticity was rated using the Modified Ashworth Scale. Presence of dystonia was assessed by reviewing video recordings, and its severity by using the Burke–Fahn–Marsden Dystonia Rating Scale.

Results

All patients showed evidence of dystonia involving upper and/or lower limbs, neck, trunk, mouth and eyes in order of frequency. In 29% of patients dystonia involved only the limbs and in 71% it was multifocal. Dystonia severity ranged from slight to severe. Severity and distribution of dystonia did not correlate with gender, age, weeks of gestation or duration of neonatal unit stay. GMFCS and MACS correlated with dystonia but not with spasticity.

Conclusions

Severity of dystonia, but not spasticity is associated with the severity of motor functional disability in CP patients with periventricular leukomalacia and demonstrates the key role of dystonia in the motor function of these patients.

中文翻译：

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小儿脑瘫和脑室周围白细胞减少症的肌张力障碍评估

客观的

要描述脑瘫（CP）患者在室周白细胞减少的情况下肌张力障碍的频率，运动表型，临床模式和功能后果。

方法

回顾性分析31例CP和脑室周围白细胞减少症患者。大运动功能分类系统（GMFCS）和手动能力分类系统（MACS）用于对功能能力进行分类。使用改良的阿什沃思量表对痉挛进行评定。通过回顾视频记录评估肌张力障碍的存在，并使用伯克－法恩－马斯登肌张力障碍评定量表评估肌张力障碍的严重程度。

结果

所有患者均显示出肌张力障碍的迹象，涉及频率和频率，涉及上肢和/或下肢，颈部，躯干，嘴和眼。29％的肌张力障碍患者仅累及四肢，而71％的肌张力障碍多灶性。肌张力障碍的严重程度从轻微到严重不等。肌张力障碍的严重程度和分布与性别，年龄，妊娠周数或新生儿住院时间无关。GMFCS和MACS与肌张力障碍相关，但与痉挛无关。

结论

肌张力障碍的严重程度与痉挛无关，但与痉挛性脑瘫的CP患者的运动功能障碍的严重程度有关，这表明肌张力障碍在这些患者的运动功能中起关键作用。