DESCRIPTION
A 65-year-old man was seen at the ataxia clinic with progressively worsening balance over the past 8 years. He had also had a gradual decline in hearing and tinnitus to the extent of using a hearing aid. He had a history of a left cerebellar haemangioblastoma removed 20 years ago without any residual balance problems. He had worked for 42 years as a lathe operator and had been exposed to loud noise, and hence the cause of deafness was previously thought to be industrial. Neurological examination revealed gaze evoked nystagmus, severe bilateral sensory–neural deafness, and dysarthria with marked limb and gait ataxia. Given the history of intracranial surgery, “superficial siderosis” was suspected that could account for the deafness as well as the progressive ataxia.1 MRI of the brain confirmed superficial deposition of haemosiderin over the brain stem and superior cerebellum (figs 1–3). Cerebrospinal fluid (CSF) examination further confirmed ongoing subarachnoid bleeding with xanthochromia and a high red cell count. Attempts to identify the source of bleeding failed and hence a ventriculoperitoneal shunt was inserted with the hope of arresting the progression of ataxia. Putting in a shunt is not a well recognised way of treating this condition and there is no medical treatment of any benefit, but logic suggests that if the amount of blood products in the CSF is reduced it might slow the progression. However, on review the patient did not show any neurological improvement.
Figure 1.
T2 diffusion-weighted images of basal brain showing extensive haemosiderin deposition on the meninges, cerebellum and around the brain stem.
Figure 3.
Coronal section showing blackening of the surface of cerebrum and brain stem.
Figure 2.
T2 diffusion-weighted images of basal brain showing extensive haemosiderin deposition on the meninges, cerebellum and around the brain stem.
Superficial siderosis of the central nervous system (CNS) is a rare disease. Known sources of bleeding are vascular CNS tumours, CSF cavity lesions, vascular malformations and neurosurgical interventions. It is a rather progressive illness, which usually leads to premature death. Detection of the source of bleeding is successful in only about 50% of cases. The elimination of the source of bleeding alone might prevent the progression of the disease; therefore, an early and extensive search for this source is highly recommended.2
Acknowledgments
We thank the patient for giving his consent.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
REFERENCES
- 1.McCarron MO, Flynn PA, Owens C, et al. Superficial siderosis of the central nervous system many years after neurosurgical procedures. J Neurol Neurosurg Psychiatry 2003. September; 74: 1326–8 [DOI] [PMC free article] [PubMed] [Google Scholar]
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