家族性三叉神经痛

Familial Trigeminal Neuralgia

三叉神经痛是一种临床常见疾病,典型的三叉神经痛主要表现为阵发性、闪电样的疼痛发作,疼痛剧烈,常无法忍受,呈电灼、针刺、撕裂样,每次发作持续时间数秒至数分钟不等。疼痛多发生于单侧,常有扳机点表现,其多表现为散发,而家族性三叉神经痛报道罕见,至今世界范围内报道仅50余个家系,其临床表现及发病特点与散发性三叉神经痛存在明显差别,尽管散发三叉神经痛患者的病因为责任血管压迫三叉神经REZ区已被普遍接受,但关于家族性三叉神经痛的病因是否为血管压迫存在争议,其遗传模式也没有达成一致的意见,文章复习了相关文献,并通过对这些文献进行分析综合,结合我们治疗三叉神经痛的经验,对其病因、发病机制、诊断和治疗原则、遗传模式等作了系统综述。

Trigeminal neuralgia is a common disorder characterized by unilateral, paroxysmal, and shock-like facial pain attacks lasting a few seconds with remissions and recurrences, often triggered by sensory stimulation. However, familial trigeminal neuralgia is rare, until now, only about 50 families were reported in the literature. There are distinct differences in clinical manifestations and characteristics between familial and sporadic trigeminal neuralgia patients. The mechanism and inheritance pattern of the disease has not been understood by now. In this paper, with the literature reviewed, we analyzed familial trigerminal neuralgia comprehensively in aspects of etiology, mechanism, and inheritance patter as well as treatment.