NeuroradiologySiderosis, superficial
Superficial siderosis of the central nervous system is a rare condition characterized by deposition of haemosiderin on the leptomeninges, the surface of the brain,
cerebellum, brain stem, cranial nerves and spinal cord. Progressive bilateral hearing loss and ataxia are the main clinical manifestations. The deposition of haemosiderin is due to repeated chronic subarachnoid or intraventricular bleeding. The majority of the neuropathological cases described in the literature are secondary to repeated haemorrhages from tumours (especially ependymomas),
vascular malformations and subdural haematomas; however, in the majority of cases the source of bleeding remains unknown.
The gold standard for diagnosis is MR (Fig.1) which shows a rim of marked hypointensity on T2-weighted images, consistent with haemosiderin deposits, on the surface of the cerebellum, brain stem, inferior part of the cerebral hemispheres and spinal cord. CT may show cerebellar atrophy and rarely mild hyperdensity around the brain stem. Angiography is usually negative.
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T2-weighted MR axial image: marked hypointense rim on the surface of the midbrain, mesial temporal lobes, cerebellar vermis, due to haemosiderin deposition.
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Siderosis, superficial, Fig.1 | |