[Superficial haemosiderosis of the central nervous system, neuro otological manifestations and images from a magnetic resonance brain scan: a case report].

BACKGROUND

Superficial haemosiderosis of the central nervous system (SHCNS) is an infrequent clinical entity; it is produced by the formation of clinically silent haemosiderin deposits in the leptomeninges, the subpial tissue, the cranial nerves and spinal cord, secondary to chronic bleeding in the subarachnoid space. Aetiology is idiopathic in half the cases or secondary to a vascular malformation or other structural abnormalities. At least 100 cases have been reported in the literature, most of which were diagnosed post mortem. In these descriptions there is a predominance of cerebellar ataxia, progressive hypoacusis, nystagmus, recurring headaches, pyramidal signs, an absence of caloric responses and xanthochromic cerebrospinal fluid. Magnetic resonance (MR) brain scanning in T2 revealed hypointensity in the brain stem, the cerebellum and the Sylvian fissure, with atrophy of the cerebellum and the brain stem.

METHODS

A 64 year old male with bilateral tinnitus and progressive neurosensory hipoacusis, who visited because of loss of balance, urinary incontinence and oscillopsia. The patient s personal history included an episode of unbearable headache at the age of 53. The neurological and neuro otological examination was pathological and the MR brain scan confirmed the diagnosis of SHCNS.

CONCLUSIONS

We wish to highlight how extremely rare this entity is, and especially so when there is a predominance of progressive neuro otological manifestations; we also want to emphasize the importance of carrying out specific imaging studies to enable a diagnosis to be made while the patient is still alive.