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From the Department of Medicine (Dr. Anderson), Faculty of Medicine and Health Science, University of Auckland; and the Departments of Neurology (Drs. Sheffield and Anderson) and Radiology (Dr. Hope), Auckland Hospital, Auckland, New Zealand.
Address correspondence and reprint requests to Dr. Neil Anderson, Department of Medicine, Faculty of Medicine and Health Science, University of Auckland, Private Bag 92019, Auckland, New Zealand.
OBJECTIVE: To describe the clinical and radiologic features of superficial siderosis of the CNS after treatment of a cerebellar tumor.
METHODS: Clinical assessment and MRI in four patients with superficial siderosis were performed.
RESULTS: Four patients with superficial siderosis had been treated for a primary cerebellar tumor (astrocytoma in three patients, medulloblastoma in one patient) during childhood. All patients were treated with surgery and three received radiotherapy. Slowly progressive bilateral sensorineural hearing loss, gait ataxia, and limb ataxia appeared 8 to 22 years after diagnosis of the cerebellar tumor. Other clinical features were mild cognitive impairment, dysarthria, nystagmus, optic neuropathy, anosmia, and upper motor neuron signs. The CSF contained erythrocytes and increased protein. MRI with fast spin-echo T2-weighted and gradient-echo T2* sequences showed a hypointense rim of iron coating the surface of the cerebellum and brainstem. Twenty-one other patients who had survived more than 5 years after treatment of a primary cerebellar tumor did not have symptoms or signs suggestive of superficial siderosis.
CONCLUSIONS: Superficial siderosis is an uncommon late complication of the treatment of a childhood cerebellar tumor, but it is probably underrecognized. The diagnosis should be suspected in patients who present with slowly progressive sensorineural hearing loss and ataxia many years after eradication of a childhood cerebellar tumor.
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