J Neurol (2000) 247 : 473
© Steinkopff Verlag 2000

M. Padberg
T. U. Hoogenraad

Cerebral siderosis:
deafness by a spinal tumor
Received: 20 July 1998
Received in revised form:
20 December 1999
Accepted: 4 January 2000

A 50-year-old manager presented
with a 5-year history of progressive
hearing loss and wide-based gait.
The major clinical findings were sensorineural deafness and cerebellar
ataxia. The T2-weighted magnetic
resonance images of the brain
showed striking signal voids on the
surface of the cerebellum, cerebrum
and brainstem, along the 8th cranial
nerves (Fig. 1 A, 1 B, arrowheads).
These hypointense zones on T2weighted images were caused by the
paramagnetic effect of iron and are

A
Fig. 1 A–C

L E T T E R TO T H E E D I TO R S

characteristic of superficial siderosis.
This rare condition is due to chronic
subarachnoid hemorrhage, leading to
the deposition of hemosiderin in the
leptomeninges and the subpial layers
of the brain. The source of bleeding
can be found in about half of patients; it may be a dural abnormality,
a vascular tumor, or other vascular
lesion. In this patient no abnormalities were seen on the cerebral angiogram.
Magnetic resonance imaging of
the vertebral column was performed.
At the level of the first lumbar vertebra, just below the conus medullaris,
a large tumor of the cauda equina
was seen (Fig. 1 C, arrow). At surgery it proved to be an ependymoma.
It was partially resected and then further treated with radiotherapy. In this
case the source of bleeding was a
spinal tumor. Ependymomata bleed
easily. Since 1985 at least five cases
have been reported of ependymoma
associated with superficial siderosis

B

[1]. These include a description of
cerebral superficial siderosis associated with an ependymoma of the
cauda equina [2].
References
1. Gomori JM, Grossman RI, Bilaniuk LT,
Zimmerman RA, Goldberg HI (1985)
High-field MR imaging of superficial
siderosis of the central nervous system.
J Comput Assist Tomogr 9:972–975
2. Friedman DP, Hollander MD (1998)
Neuroradiology case of the day. Myxopapillary ependymoma of the conus
medullaris or filum terminale resulting
in superficial siderosis and dissemination of tumor along CSF pathways. Radiographics 18:794–798
M. Padberg
University Department of Neurology,
Ziekenhuis Leyenburg, Leyweg 275, 2545
CH The Hague, The Netherlands
T. U. Hoogenraad
University Department of Neurology,
UMC, Utrecht

C