Superficial hemosiderosis of the central nervous system:: Clinico-pathological survey and case report

Superficial hemosiderosis of the central nervous system is a rare chronic progressive disease caused by the deposition of hemosiderin in the meninges, brain and spinal cord surface and cranial nerves. It results from recurrent haemorrhage into the subarachnoid space due to dural pathology, CSF cavity lesion, cervical root pathology, tumor, vascular abnormity, etc. The influence of radiation and the role of transthyretin amyloidosis-related angiopathy have been also discussed. Typical clinical manifestation is characterized by progressive bilateral sensorineural hearing loss, cerebellar ataxia and myelopathy. The disorder can potentially lead to serious disability. The essential method of diagnosis is MRI, which shows a rim of hemosiderin around the structures mentioned above. The cerebrospinal fluid examination reveals haemorrhage or xanthochromia in about half of cases. Biopsy and DNA examination are necessary for the diagnosis of transthyretin-related amyloidosis as a cause of the CNS angiopathy. Although superficial hemosiderosis is a disorder potentially resulting in irreversible damage to the CNS and, unfortunately, a condition difficult to treat, it should be taken into consideration when cummulation of typical symptoms is present or by an accidential finding on MRI. There is a good chance to prevent the disease progression by surgical treatment if the source of the bleeding is revealed.