Boucher John J, Counihan Timothy J
Department of Neurology, University College Hospital Galway, Ireland; School of Medicine, National University of Ireland Galway, Ireland.
Department of Neurology, University College Hospital Galway, Ireland; School of Medicine, National University of Ireland Galway, Ireland.
Mult Scler Relat Disord. 2020 Sep;44:102375. doi: 10.1016/j.msard.2020.102375. Epub 2020 Jul 5.
The cause of progressive disability in Primary Progressive Multiple Sclerosis (PPMS) is unknown. Pathogenic genes have been described in some MS cases that may contribute to progressive disability, independent of immune - mediated mechanisms (Jia et al., 2018). The autosomal dominant SPG4 (Spastin) mutation is the most common genotype in Hereditary Spastic Paraplegia (Solowska and Baas, 2015) and has been found in some patients with Relapsing Remitting Multiple Sclerosis (Mead et al., 2001, Yazici et al., 2013). Here, we describe the novel association of PPMS and the SPG4 (Spastin) mutation, in a patient with a family history of Hereditary Spastic Paraplegia, and discuss the therapeutic implications. While this single case report cannot discrimiate between simple co-occurence and the possibility of a pathogenic association, our report invites larger scale investigation.
原发性进行性多发性硬化症(PPMS)中进行性残疾的病因尚不清楚。在一些多发性硬化症病例中已描述了致病基因,这些基因可能导致进行性残疾,与免疫介导机制无关(Jia等人,2018年)。常染色体显性SPG4(痉挛蛋白)突变是遗传性痉挛性截瘫中最常见的基因型(Solowska和Baas,2015年),并且在一些复发缓解型多发性硬化症患者中也有发现(Mead等人,2001年,Yazici等人,2013年)。在此,我们描述了一名有遗传性痉挛性截瘫家族史的患者中PPMS与SPG4(痉挛蛋白)突变的新关联,并讨论了其治疗意义。虽然这个单病例报告无法区分是单纯的同时出现还是致病关联的可能性,但我们的报告促使进行更大规模的研究。