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

摘要

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