Last update:
24-Mar-2005

Superficial siderosis is a rare disease of the central nervous system, caused by destructive deposition of hemosiderin in the leptomeninges and the subpial layers of the brain and the spinal cord. This is the result of continuous or recurrent, often clinically silent, hemorrhage in the subarachnoid space, often without an evident bleeding source. The cases are described of two male patients with severe cerebellar ataxia, progressive bilateral sensorineural hearing loss, pyramidal tract signs and dementia. The diagnosis of superficial siderosis is supported by the characteristic symptom constellation, the xanthochromic cerebrospinal fluid (in one patient) and the typical MRI findings on the surface of the brainstem, cerebellum, cortex and spinal cord. Early recognition and treatment of this rare clinical entity may be of importance for the further prognosis of patients.

Key words: Central nervous system, Hemosiderin, Magnetic resonance imaging, Subarachnoid hemorrhage, Superficial siderosis Intestinal bleeding of obscure origin in childhood: Diagnosis and treatment by means of laparoscopy.