Superficial Siderosis of the Central Nervous System associated with Hemophilia A: A case report

Abstract Superficial Siderosis of the Central Nervous System (SSCNS) is a condition secondary to deposition hemosiderin within subpial layers central nervous system leading its progressive degeneration, clinically responsible for hearing impairment, cerebellar ataxia and pyramidal syndrome. Here we report case 61-year-old man with medical history congenital hemophilia A presenting typical clinical features SSCNS associated extensive hypo-intensity on fast 2D gradient-echo-weighted sequences, along spinal cord, posterior fossa's structures cerebral cortex. Interestingly, although his disorder was revealed by lumbar stenosis, presurgical imaging subclinical impairment preceding surgery suggest another underlying cause. As comprehensive work-up performed in our department ruled-out usual causes SSCNS, this reinforces question ability generate own.