Lisik Małgorzata, Sieroń Aleksander
Katedra i Zakład Biologii Ogólnej, Molekularnej i Genetyki, Śląski Uniwersytet Medyczny, ul. Medyków 18, 42-583 Katowice.
Neurol Neurochir Pol. 2010 Sep-Oct;44(5):504-10. doi: 10.1016/s0028-3843(14)60141-9.
The fragile X syndrome (FRAX) is the most common familial form of mental retardation. The incidence is estimated at 1 in 4000 males. The leading symptom of the syndrome is mental retardation, with accompanying behavioural problems. About 25-35% of affected persons meet the criteria of autism. The behavioural problems involve attention problems, hyperactivity, tactile defensiveness, speech problems (echolalia), aggression, emotional problems, depression, anxiety, and stereotypic movements. There is no causal treatment and management is mainly symptomatic. Many specialists should be involved in this process. Behavioural and educational therapy is indicated. The basic step is identification of the child's problems. Its goal is to promote development of the child's abilities. Pharmacological intervention should be accompanied by supporting methods. The diagnosis of FRAX involves the entire family. The family members should be offered genetic counselling and the possibility of diagnostic DNA analysis.
脆性X综合征(FRAX)是最常见的家族性智力障碍形式。据估计,男性发病率为1/4000。该综合征的主要症状是智力障碍,并伴有行为问题。约25%-35%的患者符合自闭症标准。行为问题包括注意力问题、多动、触觉防御、言语问题(模仿言语)、攻击行为、情绪问题、抑郁、焦虑和刻板动作。目前尚无因果治疗方法,主要是对症治疗。这一过程需要许多专科医生参与。建议采用行为和教育疗法。基本步骤是识别儿童的问题。其目标是促进儿童能力的发展。药物干预应辅以支持性方法。脆性X综合征的诊断涉及整个家庭。应向家庭成员提供遗传咨询以及进行诊断性DNA分析的可能性。