Idiopathic superficial siderosis of the central nervous system: Experience of the neurology department of UHC Hassan II of Fez and review of the literature

Abstract

The superficial siderosis (SS) of the CNS is an acquired neurological disorder resulting from hemosiderin deposition in the subpial layers of the brain, cranial nerves and spinal cord producing a progressive white matter and neuronal damage. To compare the neurological results of three patients suffering from progressive ataxia due to superficial siderosis with the magnetic resonance imaging (MRI) findings. This is a retrospective study of 3 patients whose main symptom was progressive ataxia, 2 of them also had a hearing loss. Cerebral MRI showed SS-CNS. These 3 patients are the only cases diagnosed in the neurological department in Fez since September 2014 until Mars 2019. The average age of our patients was 59 years old. Gait instability was the main symptom of all patients, two of them presented also a hearing loss and only one patient had spasticity of the 2 lower limbs. All patients underwent cerebral MRI T2 gradient echo showing a siderosis of the central nervous system. The etiological assessment for a source of bleeding was performed, including: a medullary MRI and arteriography. Not objecting any obvious cause of bleeding. A neuropsychological assessment was performed revealing a deterioration of cognitive functions for one patient. The diagnosis of idiopathic SS-CNS was retained for all patients. SS-CNS should be highly suspected in patients with progressive sensorineural hearing loss, ataxia, and signs of pyramidal tracts, MRI of brain and whole spinal cord should be undertaken to confirm the diagnosis. The cause of hemorrhage is not identified in most cases.