Idiopathic siderosis of the CNS: report and review
DOI:
https://doi.org/10.48208/HeadacheMed.2024.29Keywords:
Superficial siderosis, Central nervous system, HeadacheAbstract
Superficial siderosis (SS) of the central nervous system (CNS) is a potentially disabling disorder characterized by the deposition of ferrous iron and hemosiderin, products of hemolysis, in the leptomeninges and superficial layers of the cerebral and cerebellar cortexes, as well as the brainstem and spinal cord. Persisting in the subarachnoid space, accumulation leads to demyelination, axonal loss and subsequent atrophy and neurodegeneration mediated by free radicals. In most cases, a potentially causative spinal or cranial dural abnormality is identified. The classification of SS is based on anatomical distribution, etiology and clinical manifestations, resulting in distinct subtypes: Classical infratentorial (i) SS (type 1), secondary SSi (type 2) and cortical SS (c). The classical clinic manifests with sensorineural hypoacusis, cerebellar ataxia and occasionally myelopathic and radicular signs. Although it is not a classic symptom of SS, infrequently some patients develop chronic intracranial hypertension, which is believed to be associated with obstruction of the interventricular foramen and/or malabsorption of cerebrospinal fluid (CSF). When present, the headache is usually a consequence of intracranial hypotension and its intensity varies according to the type of dural defect and the rate of bleeding or CSF leakage through a fistula. Diagnosis is established by means of magnetic resonance imaging (MRI) of the entire neuroaxis combined with clinical assessment. As alternatives aimed at preventing the progression of the disease and preserving the patient's functional integrity, in addition to controlling the deficits generated by siderosis, surgical closure of the dura mater and chelation are the main therapeutic alternatives.
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Copyright (c) 2024 Isabela da Costa Rodrigues, Mohamad Ali Hussein, Pedro Cougo Samueli, Matheus Kahakura Franco Pedro, Pedro André Kowacs, Emanuel Cassou (Author)
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