Superficial siderosis due to a lumbar ependymoma mimicking adult-onset spinocerebellar ataxia

Spengos, Konstantinos; Vassilopoulou, Sofia; Tsivgoulis, Georgios; Karachalios, Georgios; Vassilopoulos, D.

doi:10.1016/j.clineuro.2007.04.015

Cited by 18 publications

(16 citation statements)

References 9 publications

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“…Superficial siderosis complicating spinal ependymoma has been reported in the literature 3. Ependymomas present as soft, encapsulated ‘sausage-like’ masses with a propensity to haemorrhage (figure 2).…”

Section: Discussionmentioning

confidence: 99%

Spinal ependymoma complicated by superficial siderosis

et al. 2013

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DESCRIPTIONA 64-year-old woman presented with progressive hearing loss. A pure-tone audiography confirmed bilateral high-frequency sensorineural hearing loss. Her medical and surgical history was unremarkable except for long-standing back pain. Neurological examination revealed mild dysmetria and gait ataxia. There was no leg weakness or sphincteric dysfunction.MRI revealed superficial siderosis lining the cerebellum and brainstem, which prompted further imaging of the entire neuraxis in order to exclude an underlying cause. A myxopapillary ependymoma seen at the level of the conus medullaris was felt to be responsible for recurrent haemorrhage within the subarachnoid space.Excision of the spinal ependymoma was performed and the patient regained near-normal hearing following a cochlear implant. DISCUSSIONSuperficial siderosis presents with slowly progressive sensorineural hearing loss, gait ataxia and cerebellar dysarthria. Involvement of other cranial nerves has also been described. Clinical history and examination often suggest a neurodegenerative disorder.1 Superficial siderosis results from haemosiderin deposition in the subpial layers of the neuraxis, and complicates chronic haemorrhage within the subarachnoid space. The posterior fossa is preferentially affected, which may in part be explained by the presence of Bergmann glia in the cerebellum that display increased ferritin synthesis.2 The long glial segment of the vestibulocochlear nerve makes it more vulnerable to axonal damage by iron deposition.1 Bilateral sensorineural hearing loss is present in 95% of affected individuals.3 Figure 1 Linear hypointensity is seen along the cerebellar folia and superior vermis on the axial gradient echo images (A) compatible with extensive haemosiderin deposition. There is also hypointense lining of the midbrain tectum and pons. The sagittal T1-weighted image (B) reveals low signal intensity of the upper cerebellum due to extensive haemosiderin preferentially deposited in the upper cerebellum and superior vermis. The linear hypointensities are also appreciated on the axial T2 sequence (C) where they clearly outline the cerebellar folia and ventral pons and reflect the characteristic subpial haemosiderin deposition. Mild underlying cerebellar atrophy is also appreciated. The radiological findings are pathognomonic of superficial siderosis.

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Section: Discussionmentioning

confidence: 99%

Spinal ependymoma complicated by superficial siderosis

et al. 2013

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“…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%

“…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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“…[3,4] The other lesions causing SS are oligodendroglioma, astrocytoma, paraganglioma, [2,4] spinal and cranial vascular malformations, cranial or cervical trauma, brachial plexus injury, [6] previous surgery especially in posterior fossa and bleeding disorders. [7] In cases of recurrent sub-arachnoid hemorrhage,…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3] Sensorineural deafness, cerebellar ataxia and myelopathy form the classical clinical triad. [4] MRI of the brain with gradient echo sequences is the gold standard for diagnosing this condition, [5] the treatment of which is to surgically ablate the source of bleeding.…”

Section: Introductionmentioning

confidence: 99%

Spinal ependymoma presenting with visual and hearing impairment – An unusual sequelae

Gangadharan¹,

Pandey²,

Santosh³

et al. 2012

IJNS

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Superficial siderosis of the central nervous system is a rare complication of recurrent bleeding in the neuraxis which may present with various symptoms. We report a case of spinal tumor presenting with superficial siderosis with involvement of vision and hearing along with ataxia. This is a rare presentation of superficial siderosis. The pathogenesis of selective involvement of these cranial nerves is discussed.

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Superficial siderosis due to a lumbar ependymoma mimicking adult-onset spinocerebellar ataxia

Cited by 18 publications

References 9 publications

Spinal ependymoma complicated by superficial siderosis

Spinal ependymoma complicated by superficial siderosis

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

Spinal ependymoma presenting with visual and hearing impairment – An unusual sequelae

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