Siderosis idiopática del sistema nervioso central y asociación con atrofia óptica e infarto cerebral. Caso clínico / Superficial siderosis of the central nervous system. Report of one case

Superficial siderosis of the central nervous system is produced by the deposit of hemosiderin within the subpial layers of the central nervous system and central parts of the cranial nerves, leading to progressive degeneration. We report a 55-year-old male who consulted for hearing loss and long-standing progressive decrease in visual acuity, associated with sudden onset of left hemiparesis. A brain CAT scan showed subacute ischemic lesions in the territory of the right posterior cerebral artery (thalamus and right subcortical temporal regions), old ischemic lesions in the right subcortical occipital regions and cerebellar atrophy. A magnetic resonance confirmed the lesions and the presence of superficial diffuse siderosis. A cerebrospinal fluid analysis showed slight xanthochromia, 26 leukocytes/mm3, glucose 51 mg/dL and proteins 1.23 g/L. He was managed with aspirin in low doses and statins. His motor function improved and was discharged two weeks after admission.

[Superficial siderosis of the central nervous system. Report of one case]. / Siderosis idiopática del sistema nervioso central y asociación con atrofia óptica e infarto cerebral. Caso clínico.

Superficial siderosis of the central nervous system is produced by the deposit of hemosiderin within the subpial layers of the central nervous system and central parts of the cranial nerves, leading to progressive degeneration. We report a 55-year-old male who consulted for hearing loss and long-standing progressive decrease in visual acuity, associated with sudden onset of left hemiparesis. A brain CAT scan showed subacute ischemic lesions in the territory of the right posterior cerebral artery (thalamus and right subcortical temporal regions), old ischemic lesions in the right subcortical occipital regions and cerebellar atrophy. A magnetic resonance confirmed the lesions and the presence of superficial diffuse siderosis. A cerebrospinal fluid analysis showed slight xanthochromia, 26 leukocytes/mm3, glucose 51 mg/dL and proteins 1.23 g/L. He was managed with aspirin in low doses and statins. His motor function improved and was discharged two weeks after admission.

Programming peculiarities in two cochlear implant users with superficial siderosis of the central nervous system.

OBJECTIVE: To report the audiological outcomes of cochlear implantation in two patients with severe to profound sensorineural hearing loss secondary to superficial siderosis of the CNS and discuss some programming peculiarities that were found in these cases. METHOD: Retrospective review. Data concerning clinical presentation, diagnosis and audiological assessment pre- and post-implantation were collected of two patients with superficial siderosis of the CNS. RESULTS: Both patients showed good hearing thresholds but variable speech perception outcomes. One patient did not achieve open-set speech recognition, but the other achieved 70% speech recognition in quiet. Electrical compound action potentials could not be elicited in either patient. Map parameters showed the need for increased charge. Electrode impedances showed high longitudinal variability. CONCLUSION: The implants were fairly beneficial in restoring hearing and improving communication abilities although many reprogramming sessions have been required. The hurdle in programming was the need of frequent adjustments due to the physiologic variations in electrical discharges and neural conduction, besides the changes in the impedances. Patients diagnosed with superficial siderosis may achieve limited results in speech perception scores due to both cochlear and retrocochlear reasons. Careful counseling about the results must be given to the patients and their families before the cochlear implantation indication.

Hepatitis C, HCV genotypes and hepatic siderosis in patients with chronic renal failure on haemodialysis in Brazil.

BACKGROUND: The aim of this study was to investigate the HCV genotypes, hepatic siderosis, inflammatory activity and fibrosis of the liver in patients with chronic renal failure (CRF) on haemodialysis in Brazil. METHODS: A cohort of 72 CRF patients was compared with a group of 65 candidates for blood donation (CBD). For the subjects selected, who tested positive for anti-HCV antibodies and were HCV-PCR positive, a protocol with epidemiological, clinical and laboratory information was completed. An ultrasound-guided liver biopsy was performed and histological analysis of liver fragments was carried out. The presence of HCV-RNA in plasma was established by nested-RT-PCR. The genotype was determined by Restriction Fragment Length Polymorphism (RFLP) analysis of the PCR product. RESULTS: HCV genotype 1 was predominant in both groups, but genotype 2 was the second most common amongst CRF patients, and there was a significant difference when compared with the CBD group (P=0.016). Regarding inflammation and fibrosis, no significant difference was observed in the histology of the liver between the study groups. Siderosis of the liver was more prevalent in the CRF group (P=0.000). Severe complications of liver biopsies were reported in 10 CRF patients (13.2%). CONCLUSIONS: Genotype 2 was observed more frequently in the haemodialysis group. No statistically significant difference was detected between the CRF and CBD groups with regard to both inflammatory response and liver fibrosis. Hepatic siderosis has been attributed to excessive iron administration. As percutaneous liver biopsy resulted in severe complications, we suggest that other procedures of evaluating liver damage in CRF patients should be looked at thoughtfully.