Superficial Siderosis of the Central Nervous System in a Patient with Neurofibromatosis Type I

Manfredi, M.; Togni, Lucrezia; Beltramello, Alberto

doi:10.1159/000008134

Cited by 7 publications

(4 citation statements)

References 5 publications

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“…15,16,22,[24][25][26][27][28] However, our findings differ from some previous reports on the causes of type 1 (classical) iSS; although arteriovenous malformations have been reported to cause 9% of cases of superficial siderosis of the CNS, 9 we found none, and only 1 very atypical aneurysm, visible on plain MRI, with a contrast-enhancing wall. 29,30 Neurofibromatosis, ankylosing spondylosis, Marfan syndrome, and arachnoid cysts might all cause iSS by injury or distortion of the dura, leading to a weak point and a source of slow blood leakage through communication with the subarachnoid space. Whereas cerebral amyloid angiopathy (CAA) has been suggested to account for 3% of cases of superficial siderosis of the CNS, 9 we did not find any cases of CAA as a cause of type 1 (classical) iSS; moreover, patients with CAA-related secondary iSS did not have any of the "classical" iSS clinical features of hearing loss, ataxia, or myelopathy.…”

Section: Results In the Context Of Previous Findingsmentioning

confidence: 99%

“…Neurofibromatosis was the presumed cause of type 1 (classical) iSS in 2 patients, in keeping with previous single case reports. 29,30 Neurofibromatosis, ankylosing spondylosis, Marfan syndrome, and arachnoid cysts might all cause iSS by injury or distortion of the dura, leading to a weak point and a source of slow blood leakage through communication with the subarachnoid space.…”

Section: Results In the Context Of Previous Findingsmentioning

confidence: 99%

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Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway

Wilson¹,

Chatterjee

Farmer

et al. 2017

Annals of Neurology

196

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Central nervous system infratentorial superficial siderosis (iSS) is increasingly detected by blood-sensitive magnetic resonance imaging (MRI) sequences. Despite this, there are no standardized diagnostic criteria, and the clinical-radiological spectrum, causes, and optimum investigation strategy are not established. We reviewed clinical and radiological details of patients with iSS assessed at a specialist neurological center during 2004-2016 using predefined standardized radiological criteria. All imaging findings were rated blinded to clinical details. We identified 65 patients with iSS, whom we classified into 2 groups: type 1 (classical) and type 2 (secondary) iSS. Type 1 (classical) iSS included 48 patients without any potentially causal radiologically confirmed single spontaneous or traumatic intracranial hemorrhage, of whom 39 (83%) had hearing loss, ataxia, or myelopathy; type 2 (secondary) iSS included 17 patients with a potentially causal radiologically confirmed spontaneous or traumatic intracranial hemorrhage, of whom none had hearing loss, ataxia, or myelopathy. Of the patients with type 1 (classical) iSS, 40 (83%) had a potentially causal cranial or spinal dural abnormality, 5 (11%) had an alternative cause, and 3 (6%) had no cause identified. Intra-arterial digital subtraction angiography did not identify any underlying causal lesions for type 1 iSS. Type 1 (classical) iSS, defined using simple radiological criteria, is associated with a characteristic neurological syndrome. Rational investigation, including spinal MRI, nearly always reveals a potential cause, most often a dural abnormality. Catheter angiography appears to be unhelpful, suggesting that classical iSS is not associated with macrovascular arterial pathology. Recognition of type 1 (classical) iSS should allow timely diagnosis and early consideration of treatment. Ann Neurol 2017;81:333-343.

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Section: Results In the Context Of Previous Findingsmentioning

confidence: 99%

Section: Results In the Context Of Previous Findingsmentioning

confidence: 99%

Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway

Wilson¹,

Chatterjee

Farmer

et al. 2017

Annals of Neurology

196

View full text Add to dashboard Cite

show abstract

“…Progressive sensorineural hearing loss is an integral part of the Muckle–Wells syndrome, another form of dominantly inherited amyloidosis that could share a common pathogenic mechanism [Gerbig et al, 1998]. Isolated examples of superficial siderosis have also been reported in patients with coexisting neurofibromatosis Type I, and Marfan syndrome (Manfredi et al, 2000; Lin and Westesson, 2001].…”

Section: Discussionmentioning

confidence: 99%

Ultrasonic sealing of polyester and spectra fabrics using thermo plastic properties

Ghosh

Reddy

2009

J of Applied Polymer Sci

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A study of polyethylene terephthalate (PET) and spectra fabric's ultrasonic sealing (welding) potential was conducted using both continuous and discontinuous ultrasonic welding machines. The effects of two important welding parameters such as weld pressure and weld time were investigated on the fabric's bond strength. Depending on the thermal property of the polymer, a certain level of temperature rise during sealing is required to soften the fibers, to facilitate the development of rows of welding points between the fabric layers. This happens under the compaction due to weld pressure. The gaps between the points are so narrow that the rows appear to be a complete sealing of the layers. Excessive melting under weld pressure, to create melt bonding caused polymer degradation and poor bond strength. Scanning Electron Microcopy images and the temperature measurements at the fabric's interface were used to examine the bond locations of the fabric. Differential Scanning Calorimetry analyses of PET and spectra fabrics have been used to examine the thermal behavior of the ultrasonic sealed material. Adequate seam strength was achieved under certain conditions of sealing for both the fabrics using both continuous and discontinuous methods of operation.

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“…Progressive sensorineural hearing loss is an integral part of the Muckle-Wells syndrome, another form of dominantly inherited amyloidosis that could share a common pathogenic mechanism [Gerbig et al, 1998]. Isolated examples of superficial siderosis have also been reported in patients with coexisting neurofibromatosis Type I, and Marfan syndrome (Manfredi et al, 2000;Lin and Westesson, 2001].…”

Section: Discussionmentioning

confidence: 99%

Superficial siderosis: A potentially important cause of genetic as well as non‐genetic deafness

Dodson

Sismanis

Nance

2004

American J of Med Genetics Pt A

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Superficial siderosis is an important disease that is increasingly being recognized as a cause of sensorineural hearing loss. Hemosiderin, resulting from repeated episodes of subarachnoid bleeding, is deposited preferentially on the surface of the eighth nerve, cerebellum, and brain stem as a consequence of glial catabolism of ferritin within those structures. This deposition eventually results in destruction and demyelination within the central nervous system, leading to the cardinal clinical findings of superficial siderosis: hearing loss, ataxia, and myelopathy. This mechanism may contribute to the pathogenesis of several forms of genetic deafness, and should be considered as a diagnostic possibility in cases of late onset deafness even in the absence of an overt history of subarachnoid bleeding.

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Superficial Siderosis of the Central Nervous System in a Patient with Neurofibromatosis Type I

Cited by 7 publications

References 5 publications

Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway

Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway

Ultrasonic sealing of polyester and spectra fabrics using thermo plastic properties

Superficial siderosis: A potentially important cause of genetic as well as non‐genetic deafness

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