Infratentorial superficial hemosiderosis

Abstract

   Introduction. Superficial hemosiderosis of the central nervous system is a chronic, progressive disease caused by continuous low-volume bleeding into the subarachnoid space and, as a result, subpial deposition of hemoglobin de-tritus. Infratentorial superfi cial hemosiderosis (ISS) is characterized by hemosiderin accumulation primarily on the surface of the cerebellum, brainstem and spinal cord. Early detection of ISS may prevent severe disability and raise the chance of successful medical treatment.   The purpose of this study was to analyze clinical, radiological, instrumental, and laboratory findings, based on a series of ISS cases diagnosed at the Research Center of Neurology.   Material and methods. Seven patients diagnosed with ISS were included in this study (4 men, 3 women). Evaluation of the clinical findings, disease history, brain and spinal cord MRI, MR-angiography, brainstem auditory evoked potentials, audiometry, abdominal ultrasound, and laboratory iron metabolism tests was performed.   Results. The median age of subjects was 63 years, and the median duration of disease was 3 years. We identified the cause of ISS in four patients. The most common clinical symptoms were cerebellar ataxia and sensorineural hearing loss. All patients had a typical ISS pattern on brain MRI images.   Conclusion. With the development of neuroimaging, iSS is becoming a more frequent finding. Clinicians should be aware of its causes and optimal management strategy. Further studies of possible iSS treatments are needed to reduce free iron neurotoxicity and minimize clinical manifestations of the disease.