Superficial siderosis of the central nervous system: an usual cause of sensorineural hearing loss

Hemosiderosis of Central Nervous System: a case report of a rare and underdiagnosed disease

“…MRI is essential for diagnosis confirmation and depends on the radiologist's experience, as subtle differences in hemosiderin deposition may lead to the conclusion of a normal examination. On the other hand, abnormal MRI results may be present in the absence of symptoms (Ribeiro et al, 2013;Pichler et al, 2017;Cerqueira, Nardi & Bezerra, 2010).…”

“…This disease affects approximately one in ten million people between the ages of 14 and 77 and becomes evident on average at age 50. It affects mainly the areas of the cerebellum, the frontobasal lobe, the olfactory bulbs, the temporal cortex, the brain stem, cranial nerves, spinal cord and nerve roots (Ribeiro et al, 2013;Lee et al, 2018).…”

“…SS may be due to idiopathic cause if there is no detectable bleeding source or secondary when the bleeding lesion can be identified. The cause of bleeding is detected in about half of the cases and the most frequent are in highly vascularized spinal tumors, vascular malformations of the CNS, surgical procedures in the posterior fossa, trauma or previous intradural surgery (Sighary, Cohen-Addad & Linder, 2018;Ribeiro et al, 2013;Hiraka et al, 2018;Fragoso et al, 2017;Fearnley, Stevens & Rudge, 1995;Anderson, Sheffield & Hope, 1999). These bleeds may be infratentorial with deposition of hemosiderin in posterior fossa, brain stem and spine or cortical, in which the deposit is restricted to brain convexities and associated with cerebral amyloid angiopathy (CAA).…”

“…The classical clinical triad is composed of bilateral sensorineural deafness, progressive cerebellar ataxia and pyramidal tract involvement. Other symptoms may appear such as headaches, bladder disorders, dementia, anosmia, nystagmus, anterior horn syndrome (paralysis, loss of sensation of pain and temperature) and parkinsonian symptoms (Ribeiro et al, 2013;Fragoso et al, 2017;Tuñón Gómez, Brea Álvarez & Piazza Dobarganes, 2015).…”

Superficial siderosis of the central nervous system: case report

“…7 A SS pode ser dividida em casos idiopáticos, onde não há fonte de sangramento detectável e secundária, onde existe lesão sangrante detectável. 8 Também não costuma ocorrer distúrbio do metabolismo da hemoglobina em nível sistêmico ou associação com hemocromatose. 4 Deve-se ressaltar que em alguns casos as lesões sangrantes localizam-se na medula espinhal como meningoceles e MAV nestas regiões e até mesmo lesões nas raízes do plexo braquial.…”