RESUMO
INTRODUCTION: Superficial CNS siderosis was previously almost unknown but is now diagnosed with increasing frequency owing to magnetic resonance imaging. Patients may present with sensory deafness, gait ataxia, various sensorimotor signs and, eventually, cognitive decline. They typically have a history of traumatic brain or spinal cord injury or previous neurosurgery, or may harbour congenital malformations. However, knowledge about treatment outcomes remains scarce. METHODS: We present a series of nine consecutive patients from a large tertiary neuroscience centre in order to highlight the challenges related to the diagnosis and treatment of superficial siderosis. RESULTS: A potential bleeding aetiology was identified in all patients, but removal of the offending bleeding source was achieved only in three (33%). Symptom progression was halted in just one patient (11%), which suggests that neurodegeneration due to haemosiderin-associated iron toxicity becomes irreversible with time. CONCLUSION: Surgical therapy in superficial CNS siderosis is rarely achieved. We suggest that prospective, large-scale multicentre studies are needed to search for non-surgical therapies that reverse (or prevent) ongoing neurotoxicity due to accumulating iron toxicity. FUNDING: not relevant. TRIAL REGISTRATION: not relevant.
Assuntos
Doenças do Sistema Nervoso Central/terapia , Hemossiderose/terapia , Siderose/diagnóstico , Siderose/terapia , Adulto , Idoso , Doenças do Sistema Nervoso Central/complicações , Doenças do Sistema Nervoso Central/diagnóstico , Feminino , Hemossiderina/toxicidade , Hemossiderose/complicações , Hemossiderose/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Síndromes Neurotóxicas , Siderose/complicações , Hemorragia Subaracnóidea/etiologia , Hemorragia Subaracnóidea/cirurgia , Resultado do TratamentoRESUMO
Superficial siderosis is a rare disease characterized by accumulation of hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves. The first clinicopathological description was reported in 1908. Most patients (95%) experience progressive bilateral hearing loss that begins early in the course of disease. The initial management of a patient with hearing loss secondary to superficial siderosis involves sound amplification using hearing aids. However, cochlear implants can be considered in cases where the patient can no longer gain significant benefit from hearing aids. Only 10 studies have described the use of cochlear implants in superficial siderosis and results have been variable. We report the case of a 65-year-old woman with bilateral progressive sensorineural hearing loss caused by superficial siderosis who benefited significantly from cochlear implantation.
