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首页> 外文期刊>Degenerative Neurological and Neuromuscular Disease >Update on pediatric dystonias: etiology, epidemiology, and management
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Update on pediatric dystonias: etiology, epidemiology, and management

机译:小儿肌张力障碍的最新资料:病因学,流行病学和管理

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Abstract: Dystonia is a movement disorder characterized by sustained muscle contractions producing twisting, repetitive, and patterned movements or abnormal postures. Dystonia is among the most commonly observed movement disorders in clinical practice both in adults and children. It is classified on the basis of etiology, age at onset of symptoms, and distribution of affected body regions.Etiology: The etiology of pediatric dystonia is quite heterogeneous. There are many different genetic syndromes and several causes of symptomatic syndromes. Dystonia can be secondary to virtually any pathological process that affects the motor system, and particularly the basal ganglia.Classification: The etiological classification distinguishes primary dystonia with no identifiable exogenous cause or evidence of neurodegeneration and secondary syndromes.Treatment: Treatment for most forms of dystonia is symptomatic and includes drugs (systemic or focal treatments, such as botulinum toxin) and surgical procedures. There are several medications including anticholinergic, dopamine-blocking and depleting agents, baclofen, and benzodiazepines. In patients with dopamine synthesis defects L-dopa treatment may be very useful. Botulinum toxin treatment may be helpful in controlling the most disabling symptoms of segmental or focal dystonia. Long-term electrical stimulation of the globus pallidum internum appears to be especially successful in children suffering from generalized dystonia.
机译:摘要:肌张力障碍是一种运动障碍,其特征在于持续的肌肉收缩会产生扭曲,重复和有规律的运动或异常姿势。肌张力障碍是成人和儿童中临床上最常见的运动障碍之一。它是根据病因,症状发作的年龄以及受影响的身体部位的分布进行分类的。病因:小儿肌张力障碍的病因非常不同。有许多不同的遗传综合症和症状综合症的几种原因。肌张力障碍几乎可以继发于任何影响运动系统的病理过程,特别是基底神经节。有症状,包括药物(全身或局部治疗,如肉毒杆菌毒素)和外科手术。有几种药物,包括抗胆碱能药,多巴胺阻滞剂和消耗药,巴氯芬和苯二氮卓类药物。在多巴胺合成缺陷患者中,左旋多巴治疗可能非常有用。肉毒杆菌毒素治疗可能有助于控制节段性或局灶性肌张力障碍最致残的症状。在患有广泛性肌张力障碍的儿童中,长期电刺激苍白球间质似乎特别成功。

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