Arrhythmias  (心律失常)

心律失常是TANGO2相关代谢性脑病和心律失常的一种持续性且可能危及生命的表现，TANGO2是一种由TANGO2基因突变引起的常染色体隐性遗传疾病。该疾病的特征是反复发作的代谢危机，以横纹肌溶解症、心律失常和神经退行性病变为特征，通常在儿童期发病，表现为急性脑肌病症状，包括肌张力低下、神经系统退化和精神运动发育迟缓。进行性神经退行性病变会导致癫痫、认知障碍、锥体束和小脑体征以及表达性语言丧失，而心脏受累则表现为严重的心律失常，是发病率和死亡率的重要因素。

Arrhythmias are a consistent and potentially life-threatening manifestation in TANGO2-related metabolic encephalopathy and arrhythmias, an autosomal recessive disorder caused by mutations in the TANGO2 gene. This condition is characterized by recurrent metabolic crises featuring rhabdomyolysis, cardiac arrhythmias, and neurodegeneration, with onset typically in childhood presenting as acute encephalomyopathic symptoms including hypotonia, neurologic regression, and delayed psychomotor development. Progressive neurodegeneration leads to epilepsy, cognitive impairment, pyramidal and cerebellar signs, and loss of expressive language, while cardiac involvement manifests as severe arrhythmias, contributing significantly to morbidity and mortality.

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