Neuroscience Division, Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CRCHUM), Montreal, Quebec, Canada.
Department of Neuroscience, Université de Montréal, Montreal, Quebec, Canada.
Am J Med Genet A. 2023 Jul;191(7):1942-1947. doi: 10.1002/ajmg.a.63209. Epub 2023 Apr 12.
The sodium leak channel (NALCN) gene encodes a sodium leak channel that plays an important role in the regulation of the resting membrane potential and the control of neuronal excitability. Mutations in the NALCN gene have been reported in patients with infantile hypotonia with psychomotor retardation and characteristic facies (IHPRF) and congenital contractures of the limbs and face with hypotonia and developmental delay (CLIFAHDD syndrome). We describe the case of a father with drug-resistant left temporo-orbitofrontal epilepsy and his son with mildly-symptomatic temporal epilepsy (only recurrent déjà vu auras) whose genetic panels identified a likely pathogenic deletion of exon 27 on the NALCN gene. Our study helps broaden the clinical spectrum of diseases associated with mutations in the NALCN gene.
钠漏通道(NALCN)基因编码一种钠漏通道,在调节静息膜电位和控制神经元兴奋性方面发挥着重要作用。NALCN 基因突变已在伴有运动发育迟缓及特征性面容的婴儿张力减退伴智力运动发育迟缓(IHPRF)和伴有张力减退和发育迟缓的四肢及面骨先天性挛缩(CLIFAHDD 综合征)的患者中报道。我们描述了一位耐药性左侧颞叶眶额部癫痫患者及其儿子的病例,其儿子表现为轻度症状性颞叶癫痫(仅反复出现幻觉先兆),其基因谱鉴定出 NALCN 基因外显子 27 的可能致病性缺失。我们的研究有助于拓宽与 NALCN 基因突变相关疾病的临床谱。