Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches.
OBJECTIVE
Superficial siderosis of the central nervous system (CNS) is a rare chronic progressive disorder caused by chronic subarachnoid hemorrhage. We present four patients with superficial siderosis of the CNS to describe the characteristic symptoms, and to discuss the pathogenetic heterogeneity and possible new therapeutic approaches.


RESULTS
The causes of chronic subarachnoid bleeding in superficial siderosis were different. In two patients surgical treatment of ependymoma or cerebral cavernomas were the underlying diseases. No cause was detected in one patient. For the first time, we present one patient with vasculitis of the central nervous system associated with systemic hemochromatosis in superficial siderosis. Therapeutic approaches included exstirpation of cavernomas as the source of chronic bleeding in one patient, immunosuppressive therapy and venupunctures in the patient with vasculitis and hemochromatosis, and symptomatic treatment with chelating agents and antioxidants. The patients remained clinically stable for the follow-up period of up to 2 years.


CONCLUSIONS
Our cases underline the pathogenetic heterogeneity of superficial siderosis and favor the early diagnosis for prompt initiation of therapy. Besides treatment of the underlying condition, antioxidants and radical scavengers may be effective in halting the progression of the disease.
Leussink
V I
VI
Department of Neurology, University of Würzburg, Germany. verena.leussink@mail.uni-weurzburg.de
Flachenecker
P
P
Brechtelsbauer
D
D
Bendszus
M
M
Sliwka
U
U
Gold
R
R
Becker
G
G
D000328:Adult; D000368:Aged; D000975:Antioxidants / Q000627:therapeutic use; D001927:Brain Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D001932:Brain Neoplasms / Q000601:surgery; D002533:Cerebral Angiography; D004806:Ependymoma / Q000601:surgery; D005260:Female; D016166:Free Radical Scavengers / Q000627:therapeutic use; D006392:Hemangioma, Cavernous / Q000601:surgery; D006486:Hemosiderosis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination / Q000187:drug effects; D011183:Postoperative Complications / Q000175:diagnosis / Q000209:etiology; D011379:Prognosis; D013345:Subarachnoid Hemorrhage / Q000175:diagnosis* / Q000209:etiology
D000975:Antioxidants; D016166:Free Radical Scavengers