[Superficial hemosiderosis of the central nervous system improved by corticosteroids].

Abstract

INTRODUCTION:

Superficial siderosis is of the Central Nervous System (CNS) is an uncommon and often disabling disorder. There is no evidence that any treatment, including removal of an identified source of bleeding, affects disease progression.

OBSERVATION:

We report the case of a 49-year-old woman exhibiting progressive and various neurological disorders associating chorea, pyramidal syndrome, cerebellar ataxia, cognitive disorders and cranial nerve deficits. She had a prior history of right occipital arterioveinous malformation (AVM) revealed four years before by ventricular hemorrhage. The AVM was treated by radiosurgery. Because of a pronounced progression of the symptoms, treatment with steroid therapy was initiated before the diagnosis of siderosis of the central nervous system was asserted by magnetic resonance imaging (rim of hypo-intensity due to hemosiderin around the brainstem, the cerebellum and the spinal cord on T-2 weighted and gradient echo T-2* imaging) and cerebrospinal fluid (CSF) examination (high CSF levels of iron and ferritin). Over the next months the neurological condition improved under steroid therapy.

CONCLUSION:

Our observation is interesting because of the chorea movement disorders which are rarely reported in the disease and because of the improvement of the neurological condition after steroid therapy which is described in only another case in the literature. Steroid therapy could constitute a new track for the treatment of siderosis of CNS.

• Authors: E Le Rhun, G Soto Ares, N Pécheux, A Destée, L Defebvre
• Journal: Revue neurologique (Rev Neurol (Paris)) Vol. 164 Issue 3 Pg. 264-70 (Mar 2008) ISSN: 0035-3787 [Print] France
• Vernacular Title: Hémosidérose marginale du névraxe d'évolution favorable sous corticoïdes.
• PMID: 18405778 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Anti-Inflammatory Agents
• Hemosiderin

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Anti-Inflammatory Agents (therapeutic use)
• Brain (pathology)
• Central Nervous System Diseases (drug therapy)
• Cerebral Hemorrhage (etiology)
• Chorea (drug therapy, etiology)
• Female
• Hemosiderin (metabolism)
• Hemosiderosis (drug therapy)
• Humans
• Intracranial Arteriovenous Malformations (complications, surgery)
• Magnetic Resonance Imaging
• Middle Aged
• Radiosurgery
• Spinal Cord (metabolism, pathology)