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In superficial siderosis (SS), haemosiderin is deposited in the subpial layers of the central nervous system. SS can be diagnosed by the presence of a hypointense rim over the brain surface on gradient recalled echo (GRE) T2 weighted MRI, typically surrounding the brainstem and filling the cerebellar sulci. The classical triad consists of slowly progressive hearing loss, cerebellar ataxia and corticospinal tract signs. SS has classically been attributed to chronic or repeated subarachnoid haemorrhage, which may be otherwise clinically silent. In up to 65% of patients a bleeding source can be detected; the other cases have been labelled idiopathic.1 Recently, Feldman et al presented two patients with SS and histopathology of neuritic plaques and cerebral amyloid angiopathy (CAA).2 A few more patients have been published with both sporadic CAA and SS, without evidence of intraparenchymal amyloid.1 3 In a population based study Vernooij et al found SS in 0.7% of non-demented individuals, all of whom also had intracerebral microbleeds.4
11C-Pittsburgh compound B (PIB) positron emission tomography (PET) marks both vascular and parenchymal β amyloid plaques in vivo. We report 11C-PIB PET findings in two cases with SS. Our findings suggest that SS in the context of β amyloidosis may have a distinct clinical and MRI signature.
Case No 1
A 76-year-old woman with a history of hypercholesterolaemia who was taking acetylsalicylate presented with two episodes of hypoesthesia migrating from the right gluteal region to the right arm, associated with non-fluent speech, which lasted for a total of …
Funding Research Foundation Flanders and Bijzonder Onderzoeksfonds KU Leuven.
Competing interests RV has been the principal investigator and KVL co-investigator of a GE HC sponsored phase I trial. RV, KVL and VT are senior clinical investigators and NN is a PhD Fellow of the Flemish Research Foundation Flanders (FWO).
Patient consent Obtained.
Ethics approval This study was conducted with the approval of the ethics committee of the University Hospitals Leuven.
Provenance and peer review Not commissioned; externally peer reviewed.
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