Superficial Siderosis of the Central Nervous System: Neuroradiological Evaluation of Two Cases

Daniele, D.; Bracchi, M.; Riva, Andrés de la; Duca, Sergio; Stura, G.; Bradac, Gianni Boris

doi:10.1159/000116840

Cited by 6 publications

(3 citation statements)

References 3 publications

(9 reference statements)

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“…Other cranial nerve may be involved like oculomotor ( 3 ), optic, and trigeminal nerves ( 4 ). Moreover, pyramidal and somesthesic signs, as well as vesicosphincterian disorders suggestive of a neurogenic bladder, are not exceptional ( 1 ), suggesting sometimes a spinal cord syndrome ( 5 ). Our patients presented with unusual clinical symptoms because the fi rst case did have hearing loss but no cerebellar sign and the second had none of these last features.…”

Section: Discussionmentioning

confidence: 75%

Superficial Siderosis Revealed by Isolated Cognitive Impairment

Scanff¹,

Vighetto

Gédéon³

et al. 2009

The Journals of Gerontology Series A: Biological Sciences and M

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Superficial siderosis (SS) is a rare disorder due to chronic bleeding into the subarachnoid or intraventricular space. The most common clinical presentation is progressive ataxia and hearing loss. The authors report two patients who presented with dementia as the primary manifestation of SS. The cognitive impairment marked by cortical frontotemporoparietal dysfunction was consistent with the pattern of signal abnormalities seen on brain magnetic resonance imaging (MRI). Diagnosis of SS must be considered when T2*-weighted MRI shows typical signal hypointensity outlining the brain and spinal cord surfaces. Performing such MRI sequences appears to be of particular interest in the context of dementia etiological diagnosis.

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Section: Discussionmentioning

confidence: 75%

Superficial Siderosis Revealed by Isolated Cognitive Impairment

Scanff¹,

Vighetto

Gédéon³

et al. 2009

The Journals of Gerontology Series A: Biological Sciences and M

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“…In other described cases of superficial siderosis the source of bleeding was either a tumour, a vascular malformation (Tomlinson and Walton, 1964;Mamourian, 1993) or damage to the meninges (Janss et al, 1993;Bonito et al, 1994). In other cases a cause could not be identified (Zimmermann et al, 1988;Daniele et al, 1992;Willeit et al, 1992;Bracchi et al, 1993;de Souza et al, 1993;Pyhtinen et al, 1995). Haemosiderin deposition is conspicuous mainly in the basal subarachnoid cisterns probably due to both the cerebro spinal fluid dynamics and to local differences in haem metabolism (Koeppen et al, 1993).…”

Section: Resultsmentioning

confidence: 98%

“…Prior to the introduction of MRI scanning the diagnosis was made at autopsy (Tomlinson and Walton, 1964). MRI can now easily demonstrate even small deposits of ironcontaining pigments due to their marked paramagnetic effect (Zimmermann et al, 1988;Daniele et al, 1992;Bracchi et al, 1993;Grunshaw et at., 1993;Gomori et al, 1985).…”

Section: Discussionmentioning

confidence: 99%

Superficial siderosis of the central nervous system: an underestimated cause of hearing loss

Castelli

Husband

1997

J. Laryngol. Otol.

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Superficial siderosis of the central nervous system (CNS) is a rare disease resulting in the accumulation of haemosiderin in the meninges, the brain surface, the spinal cord and the cranial nerves. The pigment is deposited as a result of chronic bleeding in the subarachnoid space. This produces a clinical picture of deafness, ataxia, cranial nerve deficits and in the latest stages dementia. In some cases the source of bleeding can be identified, whilst in others it can not. Despite its rarity the disease should be considered in the differential diagnosis of sensorineural deafness, particularly as it is a progressive and in some cases curable disease which is easily diagnosed by magnetic resonance imaging (MRI). In this case report the haemosiderin was derived from an ependymoma of the fourth ventricle with extension into the cerebello-pontine angle. The first symptom was a worsening sensorineural hearing loss.

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Superficial siderosis of the central nervous system: MRI findings and clinical significance

et al. 1996

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We reviewed a 7-year series of brain MRI examinations to determine the frequency and clinical significance of superficial siderosis of the central nervous system (SS). SS was defined by widespread bilateral signal loss at the surfaces of the cerebral or cerebellar hemispheres, the brain stem and the spinal cord on T2-weighted images. Clinical data comprised a neurological examination of identified patients and a review of their case notes. Among 8843 consecutive studies we identified 13 (0.15%) patients with MRI evidence of SS. Only 2 had symptoms or signs characteristic of SS, such as cerebellar ataxia, hearing loss, myelopathy and dementia. Haemosiderin deposition was most widespread in both symptomatic individuals. A definite cause for SS was detected in 9 patients (69%). None of them had a full clinical picture of SS. These data indicate SS per se to be much more frequent than may be assumed from the literature. It appears to become symptomatic only with extensive amounts of widespread iron deposition which develop preferentially with cryptic or unidentified causes of bleeding.

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Superficial Siderosis of the Central Nervous System: Neuroradiological Evaluation of Two Cases

Cited by 6 publications

References 3 publications

Superficial Siderosis Revealed by Isolated Cognitive Impairment

Superficial Siderosis Revealed by Isolated Cognitive Impairment

Superficial siderosis of the central nervous system: an underestimated cause of hearing loss

Superficial siderosis of the central nervous system: MRI findings and clinical significance

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