Superficial siderosis of the central nervous system associated with Parkinsonism

Rare association of secondary superficial siderosis caused by a fourth ventricle hemorrhagic ependymoma mimicking a cavernoma: Case report and literature review

“…The radiological findings suggested a preoperative differential diagnosis which included cavernous malformation and myxopapillary ependymoma. After revising the literature on the association of these pathologies with SS, we have found 13 cases of cavernoma with secondary SS, none of which had a fourth ventricle location,[ 17 18 24 25 29 30 32 35 42 47 49 ] and all 8 cases secondary to myxopapillary ependymoma had an intraspinal location;[ 12 13 14 20 30 38 45 52 ] thus, it would seem that our differential diagnosis was statistically improbable. All four previously published cases of a fourth ventricular hemorrhagic mass and SS have been diagnosed as ependymoma.…”

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

“…According to Koppen et al 8 the following mechanisms have been implicated in the pathogenesis of SS: [1] chronic or intermittent extravasations of blood into the subarachnoid space and dissemination of heme by circulating cerebrospinal fluid (CSF), [2] hemolysis, [3] entry of heme into exposed tissue, [4] conversion of heme to free iron, ferritin, and hemossiderin, leading to [5] damage of neural tissue. Microscopically, there is reactive gliosis, demyelination, neuronal loss, and intracellular ovoid bodies.…”

“…Less frequently, it is also associated with bladder disturbance, anosmia, extraocular palsies, anisocoria, dementia, and Parkinsonism 1,2 . SS is caused by chronic or recurrent haemorrhage into the subaracnoid space with haemossiderin deposition in the subpial layers of brain and spinal cord, which leads to progressive and irreversible neurological dysfunction.…”