Superficial siderosis following trauma to the cervical spine: Case series and review of literature

Superficial siderosis is a rare progressive disease associated with chronic hemosiderin deposition on the surfaces of the central nervous system (CNS). It typically manifests clinically in sensorineural hearing loss, cerebellar ataxia, and pyramidal signs. Recurrent or continuous bleeding into the cerebrospinal fluid is implicated in the disease process. The magnetic resonance imaging gradient-echo T2-weighted images have high sensitivity for hemosiderin deposits that bathe the CNS, giving the characteristic black rimmed area of hypointensity apparent on these images. The natural history and its treatments are still not clearly defined in literature. Our report details the clinical course and management of three cases of superficial siderosis following either cervical spine or brachial plexus injury. All of them underwent surgical intervention. In two of the cases, positive cessation of the intradural bleeding was achieved through surgery but clinical and radiological improvement occurred in only one of the cases. One patient had a negative intradural exploration. To date, 30 cases of superficial siderosis reported in the literature have undergone surgical intervention. Cessation of disease progression or neurological improvement has been documented in 18 of these cases. Our cases reveal that patients with superficial siderosis often develop severe functional impairment due to the progressive nature of the disease. On balance, we are of the opinion that early craniospinal imaging and surgical exploration should be undertaken, at least to attempt to halt neurological deterioration.