Superficial Siderosis Revealed by Isolated Cognitive Impairment

Abstract

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.

Superficial Siderosis and Anticoagulation Therapy: Different Presentations, Different Outcomes

“…Preferential deposition over frontal, temporal, and insular cortices, lumbar and sacral spinal segments, and adjacent arachnoid seems to draw a parallel with the clinical symptoms. Isolated cognitive deficits have been reported in two patients suffering from SS [ 10 ]; however overt dementia is still an exceptional form of presentation. Paraparesis may be due to iron-related injury in lumbar anterior horns and peripheral nerves, with dysfunction of upper and lower corticospinal neurons leading to atrophy and fasciculations.…”