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RESEARCH PRODUCT
Superficial Siderosis of the Central Nervous System associated with Hemophilia A: A case report
Yannick BéjotMaurice GiroudSophie MohrGuy-victor OssebyGauthier DuloquinQuentin Thomassubject
Pathologymedicine.medical_specialtyCentral nervous systemlcsh:SurgerySuperficial Siderosis of the Central Nervous System[INFO] Computer Science [cs]lcsh:RC346-429030218 nuclear medicine & medical imaging03 medical and health sciences0302 clinical medicinemedicineMedical history[INFO]Computer Science [cs]lcsh:Neurology. Diseases of the nervous systemSubclinical infectionetiological diagnosisCerebellar ataxiabusiness.industryLumbar spinal stenosislcsh:RD1-811medicine.diseaseSpinal cordSuperficial siderosis3. Good healthmedicine.anatomical_structureCongenital Hemophilia AHemosiderinchronical bleedingSurgeryNeurology (clinical)medicine.symptombusiness030217 neurology & neurosurgerydescription
Abstract 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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2021-03-01 |