Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches

Leussink, Verena I.; Flachenecker, P.; Brechtelsbauer, D.; Bendszus, Martin; Sliwka, Ulrich; Gold, R; Becker, Georg

doi:10.1034/j.1600-0404.2003.02001.x

Cited by 54 publications

(54 citation statements)

References 21 publications

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“…Superficial siderosis (SS) of the central nervous system (CNS) is a rare disease that produces haemosiderin deposition in the subpial layers of the brain and spinal cord due to chronic and repeated haemorrhaging into the subarachnoid space, leading to progressive and irreversible cerebellar ataxia, auditory disturbance and dementia [1][2][3][4][5][6][7][8]. The causes of SS include a prior intradural surgery, carcinoma, arteriovenous malformation, amyloid angiopathy and fluid collection in the spinal canal [1][2][3]8].…”

Section: Introductionmentioning

confidence: 99%

CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: Two case reports

Ikeda

Noto

Noguchi‐Shinohara

et al. 2010

Clinical Neurology and Neurosurgery

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We report two cases of superficial siderosis (SS) of the central nervous system (CNS), which is caused by chronic haemorrhaging into the subarachnoid space with haemosiderin deposition in the superficial portion of the CNS. Patient 1 had fluid collection in the spinal canal, which was reported as the source of the chronic bleeding. Patient 2 was bleeding from thickened dura at the level of the sacral vertebrae. Both of the patients had xanthochromic cerebrospinal fluid. We surgically repaired the sources of bleeding. Subsequently the cerebrospinal fluid (CSF) cleared and their symptoms were not aggravated for about one year. We measured several CSF markers of SS before and after surgery. Total tau protein (CSF-t-tau), phosphorylated tau protein (CSF-p-tau), iron (CSF-iron) and ferritin (CSF-ferritin) in the CSF were highly elevated at diagnosis. After surgery, the levels of CSF-t-tau and CSF-p-tau were markedly reduced while CSF-iron and CSF-ferritin had not decreased. It is suggested that CSF-t-tau and CSF-p-tau reflected the neural damage in SS and were useful to evaluate the effectiveness of SS therapies.-2 -CSF tau protein in superficial siderosis

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Section: Introductionmentioning

confidence: 99%

CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: Two case reports

Ikeda

Noto

Noguchi‐Shinohara

et al. 2010

Clinical Neurology and Neurosurgery

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“…The cerebellum, basal frontal lobe, cortex adjacent to the interhemispheric fissure, insular and peri-Sylvian regions and the mesial temporal lobe around the hippocampal and choroidal fissures may be involved. 1,2,4 This hypointensity is more pronounced on gradient-echo T2* sequences. Angiography and technetiumlabeled red blood cell scans seldom show the bleeding source.…”

Section: Discussionmentioning

confidence: 95%

“…Cervical nerve root pathology, for example, avulsion injury, is one cause of recurrent hemorrhage. 1,2 We report superficial siderosis because of malpositioning of a spinal instrument as a complication of spinal surgery.…”

Section: Introductionmentioning

confidence: 99%

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Malpositioned spinal instrument as a possible cause of superficial siderosis

et al. 2009

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Study design: Case report. Objective: To report a patient with superficial siderosis as a complication after posterior fixation surgery for odontoid fracture. Setting: Department of Neurosurgery, Hokkaido University, Japan. Methods: A 36-year-old man had undergone C1-C2 posterior fixation using lamina hooks for an odontoid fracture in 1997. In 2003, he presented with hearing loss and ataxia; and in 2006, a diagnosis of superficial siderosis was made and spinal instrument malpositioning was detected. Results: The malpositioned instrument, suspected as the cause of superficial siderosis, was removed. Conclusions: Superficial siderosis of the central nervous system is rare; it results in progressive hearing loss, cerebellar ataxia and pyramidal sign. Chronic hemorrhage in the subarachnoid space precipitates hemosiderin around the cerebellum and brainstem resulting in neurological symptoms. Recurrent hemorrhage and cervical root pathology, for example, root avulsion, are factors; the symptoms worsen gradually and result in hemostasis. Superficial siderosis because of complications from spinal instrumentation surgery is extremely rare. If the instrument is malpositioned in the subarachnoid space, we suggest its removal.

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“…2,3,7 Including the present report, 16 cases of intracranial vascular malformations have been associated with SS in the literature (Table). Superficial siderosis was attributed to a venous malformation in one case, 8 an aneurysm in 3 cases, 9,10 a cavernous angioma in 7 cases, [11][12][13][14] an AVM in five cases. [3][4][5][6] The AVMs' were located at the ventricular or paraventricular region in two cases 3,5,6 and at the cerebral convexity in two other cases.…”

Section: Discussionmentioning

confidence: 99%

Symptomatic Superficial Siderosis due to an Intracranial Arteriovenous Malformation

McLaughlin

Bojanowski

2007

Can. j. neurol. sci.

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Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches

Abstract: Our cases underline the pathogenetic heterogeneity of superficial siderosis and favor the early diagnosis for prompt initiation of therapy. Besides treatment of the underlying condition, antioxidants and radical scavengers may be effective in halting the progression of the disease.

Cited by 54 publications

References 21 publications

CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: Two case reports

CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: Two case reports

Malpositioned spinal instrument as a possible cause of superficial siderosis

Symptomatic Superficial Siderosis due to an Intracranial Arteriovenous Malformation

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