Superficial siderosis

Kumar, Neeraj; Cohen-Gadol, Aaron; Wright, R. A.; Miller, Gary M.; Piepgras, David G.; Ahlskog, J. E.

doi:10.1212/01.wnl.0000208510.76323.5b

Cited by 219 publications

(275 citation statements)

References 23 publications

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“…Patient 1 had fluid collection in the spinal canal, which was related to chronic bleeding [2]. In Patient 2, we detected the bleeding point in the thickened dura at the level of the sacral vertebrae and resected it with the surrounding dura.…”

Section: Discussionmentioning

confidence: 98%

“…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%

“…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%

See 2 more Smart Citations

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: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

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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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“…Some patients with diffuse deposits have minimal symptoms whereas others with less severe findings may be debilitated significantly. In patients with a longitudinal fluid-filled collection in the spinal canal visible on MRI scan; dynamic CT myelogram can sometimes help localize the site of the defect when angiography fails to localize the bleeding source [14].…”

Section: Discussionmentioning

confidence: 99%

Superficial Siderosis of the CNS and Otoneurological Manifestations

Ramadass

Narayanan

Dhanraj

et al. 2011

Indian J Otolaryngol Head Neck Surg

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Superficial siderosis is an uncommon condition occurring in central nervous system (CNS) due to deposition of hemosiderin in the subpial meningeal layer causing progressive neurological dysfunction. The classical clinical manifestation is a triad of chronic ataxia, bipyramidal signs and progressive bilateral symmetrical sensorineural hearing loss (SNHL). It has rarely been reported in Indian literature. We report an unusual case of superficial siderosis in a 60-year-old farmer who presented with the above triad along with involvement of olfactory nerve. We present this case to highlight the fact that progressive SNHL can be an important sign for the early awareness of this rare disorder. The literature on superficial siderosis is reviewed and the pathogenesis is discussed.

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“…More recently, it has become recognized that a subset of patients with this pattern of spinal dural tear and epidural fluid collection manifest imaging and clinical features of superficial siderosis. 3 Friable vessels at the site of the dural tear are the likely source of chronic bleeding into the subarachnoid space, leading to superficial siderosis. In these instances, surgical correction of the dural tear is necessary and holds hope to prevent progressive neurologic deficits.…”

Section: Discussionmentioning

confidence: 99%

Localization of a Rapid CSF Leak with Digital Subtraction Myelography

Hoxworth¹,

Patel²,

Bosch³

et al. 2008

AJNR Am J Neuroradiol

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SUMMARY:A 53-year-old woman with superficial siderosis underwent spinal MR imaging, which demonstrated a large cervicothoracic epidural fluid collection compatible with a CSF leak. Conventional and dynamic CT myelography failed to localize the dural tear because of rapid equilibration of myelographic contrast between the thecal sac and the extradural collection. The superior temporal resolution of digital subtraction myelography precisely localized the CSF leak preoperatively and led to the successful surgical correction of the dural tear.O ne limitation of CT myelography to identify a CSF leak is temporal resolution. During the time elapsed from intrathecal contrast injection under fluoroscopy to acquisition of the CT images, equilibration of contrast between intradural and extradural CSF collections occurs. When the extradural CSF collection is large, localizing the responsible dural tear can be challenging as a result. Dynamic CT myelography has been used to overcome this problem 1 but still has temporal limitations inherent to the volume of tissue that must be imaged. We describe the case of a patient in whom chronic bleeding from a thoracic dural tear was the presumptive cause of superficial siderosis. The site of the tear was not adequately defined by myelography, postmyelography CT, or dynamic CT myelography, but digital subtraction myelography provided accurate preoperative localization. Case ReportA 53-year-old woman with progressive impaired balance, hearing loss, lower extremity numbness, and chronic daily headaches for the past 5 years underwent neurologic evaluation at our institution. On physical examination, the salient abnormalities included brisk reflexes in both upper and lower limbs, mute plantar responses, asymmetric impairment to light-touch and pin-prick sensation in both legs below the groin, prominent dysmetria on heel-to-shin testing, a widebased ataxic gait, and positive Romberg test. An audiogram confirmed right-greater-than-left sensorineural hearing loss. Tibial somatosensory-evoked responses revealed a prolonged lumbar-scalp interpeak latency suggesting a central conduction delay. The results of an atraumatic lumbar puncture included 1ϩ xanthochromia, 48 red blood cells per microliter, elevated protein of 122 mg/dL, and hemosiderin-containing macrophages.MR imaging of the brain and entire spine revealed prominent T2 shortening along the surface of the cerebellum, brain stem, and spinal cord consistent with susceptibility from hemosiderin deposition. A nonenhancing ventral epidural spinal fluid collection extended from the inferior aspect of C4 through the T6 -T7 disk space ( Figs 1A, -B). Moreover, a T2 hypointense fluid-fluid level suggestive of blood products was layering dependently in the distal thecal sac (not shown).Results of 3D time-of-flight head MR angiography were unremarkable. Because gadolinium-enhanced MR angiography of the spine suggested mildly prominent and serpiginous vessels along the dorsal aspect of the distal cord, the patient underwent catheter spinal angiogra...

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Superficial siderosis

Cited by 219 publications

References 23 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

Superficial Siderosis of the CNS and Otoneurological Manifestations

Localization of a Rapid CSF Leak with Digital Subtraction Myelography

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