Superficial siderosis (SS) of central nervous system is a rare condition characterized by hemosiderin deposition diffusely involving supratentorial and infratentorial compartments. SS usually manifests with ataxia and sensorineural hearing loss. Basal ganglia are almost always spared by the degenerative process, and movement disorders are only rarely reported. We describe the case of an aged woman with apparently idiopathic SS presenting with cerebellar ataxia, hearing loss, and orofacial dyskinesias. Together with some previously reported patients affected by SS and presenting with dystonic manifestations, our case reinforces the current hypothesis supporting a wide network disruption, rather than a direct basal ganglia damage, as the likely underlying cause of some dystonic syndromes.

中枢神经系统的浅表铁屑病（SS）是一种罕见的疾病，其特征在于铁血黄素的沉积弥漫性累及上，上，下肌室。SS通常表现为共济失调和感觉神经性听力减退。基底神经节几乎总是在退化过程中幸免，运动障碍很少报道。我们描述了一个患有明显特发性SS并伴有小脑性共济失调，听力下降和口面部运动障碍的老年妇女的病例。与一些先前报道过的受SS影响并表现出肌张力异常表现的患者一起，我们的病例进一步证实了目前的假设，即支持广泛的网络中断，而不是直接的基底神经节损伤，这是某些肌张力异常综合征的潜在病因。