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

Wilson, D; Chatterjee, F; Farmer, SF; Rudge, P; McCarron, MO; Cowley, P; Werring, DJ; (2017) Infratentorial superficial siderosis: Classification, diagnostic criteria and rational investigation pathway. Annals of Neurology , 81 (3) pp. 333-343. 10.1002/ana.24850. Green open access

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Abstract

Central nervous system infratentorial superficial siderosis (iSS) is increasingly detected by blood-sensitive 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 from 2004-2016 using pre-defined standardized radiological criteria. All imaging findings were rated blinded to clinical details. We identified 65 patients with iSS, which we classified into two groups: type 1 (classical) and type 2 (secondary) iSS. Type 1 (classical) iSS included 48 patients without any potentially causal radiologically-confirmed 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.

Type: Article
Title: Infratentorial superficial siderosis: Classification, diagnostic criteria and rational investigation pathway
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/ana.24850
Publisher version: http://dx.doi.org/10.1002/ana.24850
Language: English
Additional information: This is the peer reviewed version of the following article: Wilson, D; Chatterjee, F; Farmer, SF; Rudge, P; McCarron, MO; Cowley, P; Werring, DJ; (2016) Infratentorial superficial siderosis: Classification, diagnostic criteria and rational investigation pathway, Annals of Neurology, which has been published in final form at: http://dx.doi.org/10.1002/ana.24850. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases > MRC Prion Unit at UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Brain Repair and Rehabilitation
URI: https://discovery.ucl.ac.uk/id/eprint/1534163
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