Superficial siderosis of the central nervous system: magnetic resonance imaging and pathological correlation

Abstract

The authors report a 32-year-old woman who had undergone repair of an occipital encephalocele in infancy and who experienced a 20-year history of progressive hearing loss and intermittent vertigo. After parturition, she developed a rapidly progressive quadriparesis and brain-stem dysfunction associated with persistent intraventricular and subarachnoid hemorrhage. Serial magnetic resonance (MR) images showed progressive deposition of hemosiderin along the surface of the brain, brain stem, and spinal cord, and enhanced thickened membranes at the site of the original encephalocele repair. Posterior fossa exploration disclosed hemorrhagic membranes, which were resected; despite removal of this tissue, the patient deteriorated and died. Postmortem examination confirmed iron-containing pigment along the meninges, cerebral hemispheres, brain stem, spinal cord, and cranial nerves accompanied by atrophy of the superficial cerebellar cortex. It is concluded that superficial siderosis may accompany encephalocele repair. This is believed to be the first report in the literature of superficial siderosis of the central nervous system to correlate in vivo MR images with autopsy results.

Intracranial hemorrhage: principles of CT and MRI interpretation

“…This causes a hypointense lining of the brain surface on T2-weighted images ( Fig. 11) [46,47]. Fig.…”

Neuroimaging in Superficial Siderosis: An In-Depth Look

“…A prior history of trauma (at times trivial) or intradural surgery (commonly involving the posterior fossa) may be present. 2,[8][9][10][11][12][13][14][15][16][17][18] Often decades elapse between the presumed inciting event and the development of symptoms attributable to SS.…”

“…2,3,8,[19][20][21][22][23][24][25][26][27][28][29][30][31] Before the advent of MR imaging, approximately 40 cases had been reported in the world literature. 32 The diagnosis of SS had largely been postmortem or made during surgical exploration.…”

“…25 In vivo MR imaging and postmortem correlation have shown that the hemosiderin deposition around the brain, brain stem, and spinal cord is the cause of the characteristic hypointensity seen on T2-weighted MR imaging. 8 In recent years, an extra-arachnoid longitudinally extensive intraspinal fluid-filled collection has been frequently noted in patients with SS. 2,3,[28][29][30][31]35,36 A CT myelogram, dynamic CT myelogram, or digital substraction myelogram can identify a dural defect that connects the intrathecal space with the fluid-filled collection.…”

Superficial Siderosis of the Central Nervous System in a Patient with Neurofibromatosis Type I

“…Apart from chronic or recurrent sources of bleeding deriving from spinal pseudomeningocele of traumatic or iatrogenic origin [2], only 2 other cases of intracranial pseudomeningocele or pseudo-encephalocele were reported in association with SS of the CNS [6, 7]. In both cases meningocele or encephalocele was present at birth, localized in the occipital area, resulting in cystic cavities after surgery.…”