Superficial intraventricular surface siderosis brain

Abstract

Superficial siderosis of the central nervous system is a chronic condition characterized by hemosiderin deposition in the brain and spinal cord. It's diagnosed by brain MRI. It can be caused by low-grade extravasation of blood into the subarachnoid space of the brain. There are 2 types of superficial siderosis cortical and infratentorial. Although asymptomatic in many cases; Cerebellar-predominant siderosis, a subtype of infratentorial, can affect hearing, gait, and even muscles. In this report, we present a case of a 51-year old female with complaints of hearing loss, unsteadiness in his lower limb, and spastic paresis. During MRI neuroimaging, we noticed findings of hypointensity areas within the brainstem and cerebellum, probably due to hemosiderin deposition. Based on the MRI findings, the patient was diagnosed with superficial siderosis. The patient was started on deferiprone and followed for the consecutive 18 months. Moderate improvement of the hearing loss and ataxia was noted while no change in muscle force. However, the repetitive MRI did not reveal any changes compared to the previous one.

Fig. 1

Typical magnetic resonance imaging (MRI) findings in superficial intraventricular surface siderosis. T2-weighted axial images present hemosiderindeposits along the cerebellar peduncles, around the IV ventricle (A) (green arrow) mesencephalon) (B) (blue arrow). T2-weighted images GRE presents hemosiderin deposits along the peripontine cistern, pons, Sylvi Aquedactus, tentorium, temporal horn of the left lateral ventricle (C) (red arrows), mesencephalon, suprasellar pons cistern (D) (white arrow), III ventricle (E) (yellow arrow), and frontal horns (F) (black arrow).