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PubMedDate not supplied

[Superficial siderosis of the central nervous system: report of two new cases].

INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is a rare disease characterized by deafness, ataxia and pyramidal dysfunction. It is due to hemosiderin deposition in the subpial membranes of the brain, spinal cord and cranial nerves. Most cases are secondary to chronic or recurrent bleeding into the subarachnoid space. Diagnosis is permitted by magnetic resonance imaging (MRI). CASE REPORTS: We report two patients with a chronic, slowly progressive cerebellar ataxia and hearing loss. MRI showed T2 hypointense signals in the brain, cerebellum and spinal cord diagnostic of superficial siderosis of the CNS. Xanthochromia was present in one patient. Evoked potentials showed retrochoclear hearing loss. Extensive vascular studies were negative for bleeding sources. One patient, treated with oral anticoagulants, benefited from reduction of the International Normalized Ratio. CONCLUSION: Due to its rarity, clinical suspicion is essential for diagnosis of superficial siderosis of the CNS.

Aged↗
PubMed1991

[Pulmonary siderosis and long-term respiratory risks of arc welders].

Pulmonary siderosis and bronchial cancer are respiratory risks in the long term in arc welders. We report a case of siderosis discovered from radiological abnormalities in a patient of 56 who had been an arc welder for 30 years. An analysis of the broncho-alveolar lavage liquid and transbronchial biopsy confirmed an iron overload and revealed patchy interstitial fibrosis. The toxic risks in arc welders arise from the gaseous fumes produced and inhaled particles at the alveolar level. The pulmonary siderosis is currently considered as a simple pneumoconiosis with a good prognosis. Some recent studies suggest the possibility of a more serious outcome with fibrosis even in the absence of any associated silicosis. The risks of bronchial cancer seem to be principally linked to exposure to chrome. A better understanding of these risks could be furnished by data from epidemiological studies which are still lacking.

Bronchoalveolar Lavage Fluid↗
PubMed1991

Regulation of hepatic transferrin, transferrin receptor and ferritin genes in human siderosis.

Although many studies have examined the regulation of transferrin, transferrin receptor and ferritin subunit gene expression in experimental systems, no molecular biological data in humans have been documented to date. In this study we simultaneously analyzed the hepatic content of transferrin, transferrin receptor and heavy and light ferritin subunit messenger RNAs in tissue samples obtained from subjects with normal iron balance and patients with primary or secondary iron overload. Steady-state levels of transferrin messenger RNA were not depressed by iron overload. On the contrary, they were increased (p less than 0.001) in patients with severe hepatic siderosis (liver iron content greater than 200 mumol/gm dry wt) as compared with the control group. This indicates that, as already suggested by our previous data in experimental siderosis, iron maintains the ability to induce transferrin gene activity even when cellular iron content is significantly increased. Transferrin receptor gene expression was found to respond in the same manner to any cause of iron-tissue load, regardless of the cause. In fact, a lower signal for transferrin receptor messenger RNA was consistently detected in iron-overloaded patients vs. control subjects, particularly in patients with thalassemia major and idiopathic hemochromatosis (p less than 0.001). Ferritin light-subunit messenger RNA accumulation was significantly increased in those patients with severe siderosis (idiopathic hemochromatosis and thalassemia major = liver iron between 200 and 600 mumol/gm dry wt). The fact that no significant change in hepatic ferritin heavy-subunit gene expression was detected in iron-loaded patients confirms preferential production of light-subunit--enriched ferritins in long-term iron overload.(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗
PubMed1986

Transformation and cytotoxicity of iron in siderosis bulbi.

Ocular tissues from two enucleated and one trabeculectomized human eyes with siderosis bulbi were studied by electron microscopy. Ferritin particles were demonstrated in various cell types of the three eyes. Other types of iron were not identifiable. The ferritin particles were scattered throughout the cytoplasm, with a few particles in the nucleus and extracellular space. More ferritin particles were seen in tissues near the iron foreign body. The cells which contained numerous ferritin particles also had siderosomes, most of which were thought to be conglomerates of the ferritin particles in the secondary lysosomes. Cells with numerous ferritin particles, particularly in the siderosomes, showed vacuolar degeneration. The present study indicated that in siderosis bulbi, iron released from the iron foreign body is deposited as ferritin scattered throughout the cytoplasm and is sometimes accumulated as siderosomes, in the affinitive cells. In siderosis bulbi, the cells become damaged by the deposition of ferritin in the cytoplasm, especially in the form of siderosomes.

Adult↗
PubMed1993

[Pulmonary siderosis: report of a case].

Pulmonary siderosis is one kind of pneumoconiosis caused by the long term inhalation of iron dust. It occurs in a number of occupations including steel rolling and grinding, welding, polishing, casting, boiler scaling, iron ore mining and emery working. Here we report a case of pulmonary siderosis. A 49-year-old male who had worked in an iron foundry for 30 years was admitted because diffuse micronodular lesions were seen in both lung fields on a routine chest radiographic study. A pulmonary function test disclosed a mildly restrictive ventilatory defect. Transbronchial lung biopsy revealed a significant amount of iron dust deposited within a fibrous nodule by which confirmed the diagnosis of pulmonary siderosis.

Humans↗
PubMed1993

Occupational siderosis and welders' lung: a review.

Siderosis of the lung is generally assumed to be a benign condition, not associated with respiratory symptoms. A review of the literature suggests that this assumption may be incorrect, and that siderosis may lead both to symptomatic and functional changes. It is known that iron ore miners have a raised lung cancer mortality, but this has been attributed to smoking, or exposure to tars or radon. Mortality studies among iron workers (haematite miners, welders, iron foundry and steel workers) show, however, that an association exists between working with iron and death, both from lung cancer and other respiratory causes. A number of surveys have examined respiratory function and symptoms among welders. These indicate that welding is associated with obstructive airways disease. The effect of the welding fume on respiratory function and symptoms can be as great as that of smoking. Iron has also been shown to cause fibrosis in some cases. Small functional changes of restriction and loss of lung compliance are often due to iron alone. The fibrosis may be enhanced by associated silica exposure. A number of constituents of welding fume could, along with iron, contribute to pulmonary changes. The presence of siderosis may act as a good marker of exposure to fume and any resulting disability.

Adult↗
PubMedDate not supplied

Hepatic siderosis, fibrosis and cirrhosis: the association with hepatocellular carcinoma in high-risk population.

Iron overload has been shown to impair the immune response of the liver, and induce hepatic fibrosis and cirrhosis. Opinions differ concerning the relative risk of developing hepatocellular carcinoma (HCC) in siderotic patients as compared with patients with hepatic fibrosis and cirrhosis and the possible mechanism of liver carcinogenesis in genetic hemochromatosis is still unknown. The purpose of this study is to assess hepatic iron overload, fibrosis and cirrhosis in liver tissue adjacent to hepatocellular carcinoma and in liver tissue of controls in population at risk for hepatocellular carcinoma. Liver tissue was available for examination in 147 biopsies with HCC collected in South Africa. As controls we used liver samples from 211 age and sex matched Africans who died in accidents. Tissue samples were processed routinely, stained with H and E, Sweet's reticulin, Masson's trichrome for fibrous tissue, Prussian blue for iron stain and immunohistochemically for HBsAg. Iron content was assessed with the method described by Brissot. Iron overload was detected in 42.1% of cancerous livers and in 43.7% of livers from controls. The presence of siderosis and iron content gradually increased with the age of studied similarly in cases and in controls. Cirrhosis was present in 32% of cancerous livers and was associated with iron overload in 13%. No cirrhosis and 6% of mild periportal fibrosis not related with siderosis was observed in controls. HBsAg was stainable in 80% of cancerous livers of patients below 25 years of age and in 40% of patients over 35 years. HBsAg in controls was positive in 9%. No relationship of HBsAg and amount of stainable iron in cancerous and livers of controls was found. In conclusion, African siderosis can not play important role in the etiopathogenesis of HCC.

Adult↗
PubMedDate not supplied

[A case of occupational pulmonary siderosis: the pathogenetic and prognostic considerations].

Pulmonary siderosis is a disease which can be considered with the pneumoconioses of the so-called inert dust (either by deposit or by accumulation) and, from the earliest nosographic descriptions dating back to the thirties and forties, it has been substantially considered a benign form because of the scarce sclerogenic evolution and the preservation of discrete respiratory function. We present a case of pulmonary siderosis characterized on one hand by the importance and clarity of the cytopathological picture and on the other by the presence of changes, although modest, in lung function which confirm the most recent hypotheses in the literature on the potential evolution of pneumosiderotic diseases and their relatively less benign prognosis. From a preventive point of view the case draws attention to the need for greater control of environmental hygiene conditions in small factories. From an insurance and medico-legal point of view siderosis has recently been explicitly added to the occupational diseases which can be declared to the Italian state department dealing with occupational disease and disability payments (I.N.A.I.L.) (DPR of the 13.04.1994, section 47).

Bronchoalveolar Lavage Fluid↗
PubMed1976

[Siderosis of the lens (author's transl)].

Incipient siderosis of the lens is reflected by an extremely fine granular, almost homogenous closely subcapsular brown discoloration between the anterior capsule of the lens and the epithelium. Advanced siderosis of the lens leads to subcapsular "rust spots" of varying size, especially in the region below the pupil; to increasing permeability cataract with protein breakdown and also to brown discoloration of the developing hollow spaces and clefts containing protein. In extensive siderosis of the lens, there was the following histological evidence of iron infiltration colour reactions: Between epithelium and capsule: (fusiformly) changed epithelia, intercellular substance formed by metaplasia of epithelia with connective tissue fibrillae (birefractive capsular cataract), in large, balloon-like epithelial cells freely occurring in the liquefied capsule, by decomposition of these cells in all fluid cavities containing protein (water clefts, etc.). On the other hand, there were no siderous granulations of protein: in normal epithelial cells of the lens, in unchanged fibres of the lens, in Wedl's cells and in Morgagni's or myelin droplets.

Adult↗
PubMed1993

Posterior fossa surgery: an unusual cause of superficial siderosis.

With the widespread use of magnetic resonance imaging, an increasing number of cases of superficial siderosis are being discovered. However, the cause remains uncertain in almost half the cases. We report a case where previous surgery for a pontine hematoma established a pathway for extravasation of blood into the 4th ventricle, resulting in superficial siderosis that was demonstrated on a follow-up magnetic resonance imaging scan 8 years later. This case presents an unusual iatrogenic cause of superficial siderosis that has not been reported previously.

Brain Neoplasms↗
PubMed1983

Early receptor potential measurements in human ocular siderosis.

There is electrophysiologic evidence that photoreceptors have normal visual pigment density (outer segment length) at a stage of ocular siderosis when the photoreceptor membrane function is defective. The early receptor potentials (ERPs) from two patients showed normal amplitudes, although the electroretinograms (ERGs) had reduced a- and b-waves for both cone and rod ERG responses. Since the ERP is generated by photolysis of visual pigment and requires orientation of the pigment molecules by outer segment disc membranes, the normal ERP amplitudes suggest that the photoreceptor outer segments have normal photopigment density, are of normal length, and are properly oriented. However, the transduction mechanism that converts visual pigment photolysis into membrane electrical potentials (the ERG a wave) is defective at this stage of ocular siderosis.

Adult↗
PubMed2004

Superficial siderosis: a potentially important cause of genetic as well as non-genetic deafness.

Superficial siderosis is an important disease that is increasingly being recognized as a cause of sensorineural hearing loss. Hemosiderin, resulting from repeated episodes of subarachnoid bleeding, is deposited preferentially on the surface of the eighth nerve, cerebellum, and brain stem as a consequence of glial catabolism of ferritin within those structures. This deposition eventually results in destruction and demyelination within the central nervous system, leading to the cardinal clinical findings of superficial siderosis: hearing loss, ataxia, and myelopathy. This mechanism may contribute to the pathogenesis of several forms of genetic deafness, and should be considered as a diagnostic possibility in cases of late onset deafness even in the absence of an overt history of subarachnoid bleeding.

Adult↗
PubMed1977

The relationship between Parkinson syndrome and vascular siderosis: an electron microprobe study.

Vascular siderosis (VS) refers to the presence of mineralized small and medium-sized arteries in the globus pallidus, usually regarded as reflecting an aging process. The electron probe analysis of deposits in vascular siderosis in 10 patients dying of parkinsonism and in 8 other patients without parkinsonism is reported here. The microprobes identified a total of 11 elements in the VS in these 18 patients. Five elements--lead, aluminum, sulfur, manganese, and barium--were present only in VS associated with parkinsonism. Statistically, the association of lead was highly significant while that of aluminum and sulfur in 4. The significance of the presence of sulfur is difficult to assess since its compounds are normally present in large quantities in the brain. These findings raise the question whether lead and aluminum may be associated in some way with the pathogenesis of certain forms of Parkinson disease.

Aged↗
PubMed1993

The pathogenesis of superficial siderosis of the central nervous system.

In advanced cases of superficial siderosis of the human central nervous system, the clinical triad of hearing loss, cerebellar ataxia, and myelopathy permits the diagnosis at the bedside, and magnetic resonance imaging readily confirms the hemosiderin deposits in brainstem, cerebellum, and spinal cord. To study the pathogenesis of this condition and explain the selective vulnerability of the cerebellum, experimental siderosis was induced in rabbits by the repeated intracisternal injection of autologous red blood cells. The earliest cellular response in the cerebellar molecular layer was hyperplasia and hypertrophy of microglia as displayed by immunocytochemistry for ferritin. Microglia also contained iron, but ferritin biosynthesis appeared to proceed without commensurate iron accumulation. This early apoferritin response probably occurred due to the presence of heme, rather than iron, in the cerebrospinal fluid and subpial tissue. Ferritin biosynthesis is accelerated when the ferritin repressor protein is dissociated from ferritin messenger ribonucleic acid. A specific antiserum localized ferritin repressor protein predominantly to astrocytes including Bergmann glia. It is proposed that abundance and proximity of ferritin repressor protein--immunoreactive Bergmann glia and ferritin-containing microglia in the cerebellar molecular layer permit prompt cellular interaction in the conversion of heme to ferritin and ultimately hemosiderin.

Animals↗
PubMed1996

Surgical management of superficial siderosis following cervical nerve root avulsion.

Neurosensory hearing loss, ataxia, spastic paraparesis, sphincter dysfunction, somatosensory disturbances, and cognitive effects are associated with superficial siderosis of the central nervous system caused by chronic intrathecal bleeding. We describe superficial siderosis that developed more than a decade following traumatic brachial nerve root avulsion. While cerebrospinal fluid analysis indicated chronic intrathecal bleeding, angiography did not localize a specific source. Surgical intervention that included repair of a meningeal diverticulum and venous cauterization resulted in overall reduction, but not complete elimination, of central nervous system bleeding.

Brachial Plexus↗
PubMed1987

High-field spin-echo MR imaging of superficial and subependymal siderosis secondary to neonatal intraventricular hemorrhage.

Two cases of superficial siderosis with subependymal siderosis, secondary to neonatal intraventricular hemorrhage, are presented. High-field spin-echo MR imaging (1.5 Tesla) showed marginal hypointensity of the ventricular walls as well as of the subpial regions. These findings were most evident on T2 weighted images, characteristic of hemosiderotic deposits.

Brain Diseases↗
PubMedDate not supplied

Superficial siderosis of the central nervous system.

Two patients with superficial siderosis of the central nervous system are reported. Both developed progressive deafness over many years; one with associated anosmia and partial seizures; the other with progressive ataxia and diplopia. The cerebrospinal fluid was xanthochromic in one and the protein was raised in both. Magnetic resonance imaging revealed a hypodense rim around the eighth cranial nerve, cerebellum, brain stem and spinal cord. Despite extensive investigations the cause of the superficial siderosis in both patients remains undetermined.

Adult↗
PubMed2002

MRI of the liver and the pituitary gland in patients with beta-thalassemia major: does hepatic siderosis predict pituitary iron deposition?

Our objective was to study, in thalassemic patients, if hepatic siderosis evaluated by MRI could predict the pituitary iron overload. In 36 thalassemic patients (age range 6-44 years, mean age 21.7 years) the liver/fat ratio (L/F), the pituitary/fat ratio (P/F), the liver and pituitary T2 relaxation times were evaluated, by using a multiecho spin-echo sequence. Serum ferritin levels were measured and an extensive endocrine evaluation was performed. The L/F, the P/F and pituitary T2 showed a good correlation with serum ferritin ( r=-0.55, r=-0.55 and r=-0.53, respectively; p<0.01). Liver T2 did not show significant correlation with serum ferritin. The variability of L/F explained only the 10.8% of the variability of pituitary T2 and of the P/F. When ferritin was added to the model it predicted only the 26.85% and the 30.8% of the variability of pituitary T2 and of the P/F, respectively. The P/F and pituitary T2 were lower in patients with hypogonadotropic hypogonadism (group 1) compared with those without pituitary dysfunction (group 2). No significant differences of L/F were found between the two groups. Hepatic iron overload evaluated by MR is a poor predictor of pituitary siderosis. The MR studies of the pituitary gland might be necessary to evaluate the pituitary iron overload.

Adolescent↗