A cast-iron guarantee of ataxia and deafness.
Superficial siderosis of the central nervous system (CNS) is a rare condition that is thought to be caused by repeated subarachnoid haemorrhages. The major clinical features are progressive bilateral hearing loss, cerebellar ataxia and pyramidal dysfunction. Ante-mortem diagnosis is now possible due to MRI and post mortem reports of this condition are becoming rare. Despite the occurrence of typical clinical features the diagnosis is often missed during life if CT scanning alone is performed. We describe a case of superficial siderosis of the CNS with clinical and post mortem findings as a reminder of this unusual condition.
Shingde
Meena
M
Department of Pathology, The University of Sydney, Sydney, Australia.
Ell
Jonathan
J
Pamphlett
Roger
R
D000369:Aged, 80 and over; D001259:Ataxia / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D003638:Deafness / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D006801:Humans; D007501:Iron; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D011180:Postmortem Changes; D012806:Siderosis / Q000150:complications*; D014057:Tomography, X-Ray Computed / Q000379:methods
D007501:Iron