Superficial siderosis of the central nervous system: report of three cases and review of the literature

doi:10.1016/0022-510X(92)90107-V

Journal of the Neurological Sciences

Volume 111, Issue 1, August 1992, Pages 20-25

https://doi.org/10.1016/0022-510X(92)90107-V Get rights and content

Abstract

We present 3 cases and a review of the literature to demonstrate the current state of clinical diagnosis and therapy of superficial siderosis of the central nervous system. Typical symptoms were progressive cerebellar ataxia, spasticity and hearing loss. Repeated subarachnoid hemorrhage was indicated by persistent xanthochromia of the cerebrospinal fluid and confirmed by the presence of erythrophages, siderophages and iron-containing pigments. Deposition of free iron and hemosiderin in pial and subpial structures leads to intoxication of the central nervous system and represents the pathophysiological mechanism of superficial siderosis. Hypointensity of the marginal zones of the central nervous system on T2 weighted MR images indicates an iron-induced susceptibility effect and seems pathognomonic for superficial siderosis. In 39 of the 43 previously described cases superficial siderosis was verified by biopsy or autopsy. Today magnetic resonance imaging enables diagnosis at an early stage of the disease. Therapeutic management requires the elimination of any potential source of bleeding. In patients with unknown etiology no proofed therapy is yet available.

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Cited by (33)

Superficial siderosis due to dural defect with thoracic spinal cord herniation
2012, Journal of the Neurological Sciences
Citation Excerpt :
Superficial siderosis (SS) of the central nervous system (CNS) is a rare disorder caused by chronic or recurrent hemorrhages into the subarachnoid space with hemosiderin and ferritin deposition in the subpial layer of the brain and spinal cord, which leads to neuronal damage [1–6]. Common causes of bleeding are CNS tumors, intradural surgery, head or back trauma, root avulsions, arteriovenous malformations or aneurysms, and amyloid angiopathy [1–6]. However, the source of bleeding remains unknown in 50% of cases [3].
Superficial siderosis (SS) of the central nervous system is a rare disorder caused by chronic or recurrent hemorrhages into the subarachnoid space with hemosiderin and ferritin deposition, which leads to neuronal damage. The source of bleeding remains unknown in 50% of cases. Recently, attention has been focused on fluid-filled collection in the spinal canal, suggesting the presence of a dural defect which may be the bleeding point.
We present a patient with SS and spinal extradural fluid collection due to midthoracic dural defect with spinal cord herniation. The reduction of the spinal cord herniation and the repair of the dural defect resulted in the disappearance of the fluid collection and cerebrospinal fluid abnormalities. The case here reported is, to our knowledge, the first case of spinal cord herniation presenting with SS and confirms the key role played by dural lacerations in the pathogenesis of both SS and spinal cord herniation. The search for dural lacerations should be one of the primary aims in patients with SS.
Secondary superficial siderosis of the central nervous system: Report of three cases
2009, Neurochirurgie
Superficial siderosis (SS) is an under-recognized entity. It is due to repeated microhemorrhages in the subarachnoid spaces resulting in a deposit of hemosiderin at the surface of the central nervous system and/or the cranial nerves. The origin of microhemorrhages remains unknown in almost one third of cases and therefore no treatment can be recommended. Through a literature review and new observations, our goal is to detail the outcome of patients with a recognized etiology of SS and treated surgically.
Series of three cases and review of the literature.
We present three patients with symptomatic SS for whom the origin of microhemorrhages was found. The first two patients complained of longstanding ataxia and neurosensory deafness while the third patient presented with urinary retention, vertigo, diplopia and facial paresis. Neuroradiological explorations revealed a left temporoparietal cavernoma, a fronto-orbital arterio-venous malformation and a cauda equina myxopapillary ependymoma respectively. Surgical resection of the source of hemorrhage was performed in all cases. All outcomes were good with improvement of their SS-related symptoms. These cases are discussed and the current literature is reviewed.
It is important to recognize SS since the treatment of the bleeding source may prevent further deterioration and may even in some cases improve the clinical condition.
La sidérose superficielle (SS) est une entité méconnue. Elle résulte de microhémorragies répétées dans l’espace sous-arachnoïdien entraînant un dépôt d’hémosidérine à la surface du système nerveux central (SNC) et/ou des nerfs crâniens. La source des microhémorragies demeure inconnue dans près d’un tiers des cas et ne peut donc être traitée. À travers une revue de la littérature et de nouvelles observations notre objectif est de préciser le devenir des patients dont l’étiologie est traitée chirurgicalement.
Série de trois cas et revue de la littérature.
Nous présentons trois patients symptomatiques de SS pour lesquels une source hémorragique a été découverte. Deux d’entre eux présentaient une ataxie, une dysarthrie et une surdité tandis que le troisième souffrait d’une rétention urinaire, de vertige, de diplopie et de parésie faciale. Les bilans d’imagerie ont conclu respectivement en un angiome caverneux temporoparietal, une malformation artérioveineuse intracrânienne et un épendymome myxopapillaire de la queue de cheval. Pour chacun de ces cas, la source des microhémorragies a été traitée chirurgicalement. Tous ont évolué favorablement avec une régression de leur symptomatologie en période postopératoire. Nous confrontons ces observations à celles de la littérature.
Il est important de reconnaître la SS car le traitement de la source potentielle d’hémorragie peut enrayer la progression de ses symptômes, voire même en permettre la régression.
Superficial siderosis of the central nervous system associated with Parkinsonism
2004, Parkinsonism and Related Disorders
Superficial siderosis of the central nervous system: Its MRI manifestations
1997, Clinical Imaging
Three cases of superficial siderosis of the central nervous system are reported here. Using a 1.0-T magnetic resonance (MR) unit, typical hypointense rims were observed under the brain surface on T2-weighted images. In one patient, marked atrophy of the superior cerebellar vermis and cerebellar parenchymal hyperintensity were also detected. The spinal cord was involved in two of the three patients. On T1-weighted images, hyperintense rims were demonstrated over the brain surface in two of the three patients. This finding has not been previously reported.
Superficial siderosis
2006, Neurology
Superficial siderosis (SS) of the CNS is caused by repeated slow hemorrhage into the subarachnoid space with resultant hemosiderin deposition in the subpial layers of the brain and spinal cord. Despite extensive investigations, the cause of bleeding is frequently undetermined.
To review the clinical and imaging features of 30 consecutive patients with SS and provide insights into the underlying causes of subarachnoid bleeding in this disabling disorder.
The authors reviewed the medical records of 30 consecutive patients with clinical and MRI evidence of SS.
The commonest neurologic manifestations included gait ataxia and hearing impairment. A clinical history of subarachnoid hemorrhage was relatively rare. Possible predisposing conditions were identified on history in 22 patients, the commonest being a prior trauma (15 patients). In addition to the characteristic MRI findings of SS, 18 patients had abnormalities on MRI possibly related to chronic bleeding. The most common of these was the presence of a fluid-filled collection in the spinal canal seen in 14 patients.
A history of prior subarachnoid hemorrhage is often absent in patients with superficial siderosis (SS). A past history of trauma is common. Prior intradural surgery may be an additional risk factor. Xanthochromia or the presence of red blood cells in the CSF is a common finding. Only rarely does angiography demonstrate the bleeding source. The presence of a fluid-filled collection in the spinal canal is a common finding on MRI and is likely related to the SS. With longitudinally extensive cavities, a dynamic CT myelogram may help localize the defect and direct the site of laminectomy. Surgical repair of a dural defect, if present, should be considered. Surgical correction of bleeding should be documented by CSF examination months after surgery. Friable vessels in the dural defect are a possible source of the chronic bleeding.
Superficial siderosis of the central nervous system with epilepsy originating from traumatic cervical injury: illustrative case
2021, Journal of Neurosurgery: Case Lessons

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Research articleSuperficial siderosis of the central nervous system: report of three cases and review of the literature

Abstract

Subpial siderosis of the central nervous system

Acta Neuropathol.

Deposition of iron in paraventricular areas of the human brain in hemochromatosis

J. Neuropathol. Exp. Neurol.

Étude anatomo-clinique d'un cas de sidérose marginale du système nerveux central

Rev. Neurol.

Toxic iron and the nervous system

Acta Neuropathol.

Un cas d'hémosidérose marginale du névraxe consécutif à un angiome médullaire

Rev. Neurol.

Sur un cas de surcharge ferrique du système nerveux central

Rev. Neurol.

High-field MR imaging of superficial siderosis of the central nervous system

J. Comput. Assist. Tomogr.

Superficial siderosis of the central nervous system. A report on nine cases with autopsy

Acta Neuropathol.

The effects of subarachnoid injections of iron-containing substances on the central nervous system

J. Neuropathol. Exp. Neurol.

Aseptic hemogenic meningitis - an experimental study of aseptic meningeal reaction due to blood and its breakdown products

Arch. Neurol. Psychiatry

Hemosiderosis meningoencefálica, crónica, progresiva, necrotizante

Acta Neuropsiquiát. Argent.

Die Randzonensiderose des Zentralnervensystems

Zbl. Allg. Path. Anat.

An autopsy case of idiopathic superficial hemosiderosis of the central nervous system: a microscopic and immunohistochemical study

Clin. Neuropathol.

Research article
Superficial siderosis of the central nervous system: report of three cases and review of the literature