Learning objectives
To improve knowledge of causes,
pathology,
diagnosis and clinical presentation of superficial siderosis.
To outline the radiological findings of superficial siderosis using case examples.
Background
Superficial siderosis (SS-CNS) is a rare condition of the central nervous system characterized by deposition of haemosiderin into the sub-pial layers of the brain and spinal cord secondary to chronic or intermittent haemorrhage into the subarachnoid space [1,
2].
Advances in MRI have led to increased rates of detection [3].
Causes of SS-CNS include: trauma,
tumours,
vascular malformations,
intradural surgery and nerve root injury [1,
4-7].
Spontaneous intracranial hypotension has also been found to cause SS-CNS [8].
However,
approximately 30% of patients diagnosed with SS-CNS...
Imaging findings OR Procedure details
Magnetic resonance imaging (MRI) is the preferred imaging modality.
The classic imaging finding is a rim of hypointensity along the contours of the brain and spinal cord on T2 weighted MRI,
often in association with cerebellar and spinal cord atrophy [10].
Hypointensities are more prominent on T2* and on susceptibility weighted images (SWI).
Within the spine,
cavities may be seen,
indicative of a possible dural tear [10].
Conclusion
SS-CNS is an uncommon condition,
diagnosed increasingly frequently due to advances in imaging techniques.
It should be suspected in patients with progressive neurological symptoms,
particularly gait ataxia and sensorineural hearing loss.
Personal information
M Fielder 1,
P Jenvey 2,
3,
A Coulthard 2,
3
1 Sunshine Coast University Hospital,
Sunshine Coast,
QLD,
Australia
2 Department of Medical Imaging,
Royal Brisbane and Women’s Hospital,
Brisbane,
QLD,
Australia
3 School of Medicine,
University of Queensland,
Brisbane,
QLD,
Australia
References
1.
Kumar N,
Cohen-Gadol AA,
Wright RA,
Miller GM,
Piepgras DG,
Ahlskog JE.
Superficial siderosis.
Neurology.
2006;66(8):1144-52.
2.
Pyhtinen J,
Paakko E,
Ilkko E.
Superficial siderosis in the central nervous system.
Neuroradiology.
1995;37(2):127-8.
3.
Koeppen AH,
Michael SC,
Li D,
Chen Z,
Cusack MJ,
Gibson WM,
et al.
The pathology of superficial siderosis of the central nervous system.
Acta neuropathologica.
2008;116(4):371-82.
4.
McCarron MO,
McKinstry CS,
Gibson JM.
Superficial siderosis 20 years after brain tumour.
The Lancet Neurology.
2002;1(5):326.
5.
Kumar N.
Superficial siderosis: associations...