Superficial siderosis of the central nervous system: An unusual cause for headache and hearing loss (original) (raw)
Superficial siderosis of the central nervous system (SSCN) is a very rare disorder caused by deposition of haemosiderin in the superficial and subpial layers of the central nervous system due to repeated chronic subarachnoid or intraventricular haemorrhage. The clinical syndrome of SSCN consists of sensorineural hearing loss, progressive ataxia and spasticity. A 52 year-old woman who has a history of chronic progressive hearing loss, severe headache and gait instability for one year is presented. The neurological examination revealed bilateral sensorineural hearing loss, cerebellar ataxia and mild spasticity of the lower extremities. Brain MRI showed classical superficial siderosis in the form of hyposignal intensity along the leptomeninges. The prominent sites of hemosiderin deposition in this case were cerebellar vermis, around the brain stem and whole spinal cord surface. Cerebrospinal fluid findings confirmed chronic subarachnoid hemorrhage but bleeding site could not be demonstrated by cerebral angiography. This case report draws attention to this rare complication of chronic subarachnoid hemorrhage, which can be recognized early by its clinical triad and MRI findings.
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