Pavone Piero, Praticò Andrea D, Pavone Vito, Lubrano Riccardo, Falsaperla Raffaele, Rizzo Renata, Ruggieri Martino
University-Hospital "Policlinico-Vittorio Emanuele", University of Catania, Catania, Italy.
Unit of Pediatrics and Pediatric Emergency, Azienda Ospedaliera Universitaria Vittorio Emanuele-Policlinico, University of Catania, Italy, Via Plebiscito 767, 95123, Catania, Italy.
Ital J Pediatr. 2017 Jan 13;43(1):6. doi: 10.1186/s13052-016-0325-9.
Ataxia is a sign of different disorders involving any level of the nervous system and consisting of impaired coordination of movement and balance. It is mainly caused by dysfunction of the complex circuitry connecting the basal ganglia, cerebellum and cerebral cortex. A careful history, physical examination and some characteristic maneuvers are useful for the diagnosis of ataxia. Some of the causes of ataxia point toward a benign course, but some cases of ataxia can be severe and particularly frightening.
Here, we describe the primary clinical ways of detecting ataxia, a sign not easily recognizable in children. We also report on the main disorders that cause ataxia in children.
The causal events are distinguished and reported according to the course of the disorder: acute, intermittent, chronic-non-progressive and chronic-progressive.
Molecular research in the field of ataxia in children is rapidly expanding; on the contrary no similar results have been attained in the field of the treatment since most of the congenital forms remain fully untreatable. Rapid recognition and clinical evaluation of ataxia in children remains of great relevance for therapeutic results and prognostic counseling.
共济失调是一种涉及神经系统任何水平的不同疾病的体征，表现为运动和平衡协调受损。它主要由连接基底神经节、小脑和大脑皮层的复杂神经回路功能障碍引起。详细的病史、体格检查和一些特征性动作对共济失调的诊断很有帮助。共济失调的一些病因预示着病情良性，但有些病例可能很严重，尤其令人恐惧。
在此，我们描述了检测共济失调的主要临床方法，共济失调在儿童中不易识别。我们还报告了导致儿童共济失调的主要疾病。
根据疾病病程区分并报告了病因事件：急性、间歇性、慢性非进行性和慢性进行性。
儿童共济失调领域的分子研究正在迅速扩展；相反，在治疗领域尚未取得类似成果，因为大多数先天性形式仍然完全无法治疗。对儿童共济失调的快速识别和临床评估对于治疗效果和预后咨询仍然具有重要意义。
