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At least 145 records · Page 8Linked to original sources
PubMed2003

[Siderosis bulbi. Is it still a problem?].

BACKGROUND: Siderosis bulbi (SB) is caused by un-removed intraocular iron-containing foreign bodies. Our retrospective study was designed to establish the number of patients treated for SB at our ophthalmology department within the past 10 years, and to find out why SB occurred. PATIENTS AND METHODS: Retrospectively, we analyzed the documentation of every patient treated at our department for ocular injury due to an intraocular metal foreign body between 1.1.1992 and 1.1.2002. Particular attention was focussed on the documentation of patients exhibiting symptoms of SB. RESULTS: During this period of time, 48 eyes with an intraocular iron-containing foreign body were treated operatively. In 45 cases, the foreign body was removed within 3 days after injury without any symptoms of SB. Of these, three patients (6.2%) came so late for treatment that various clinical signs of SB were already present. CONCLUSIONS: There are three major reasons for the development of SB: some patients do not come for an examination because there is no discomfort, the second reason is that the medical treatment at the first visit was too superficial and thirdly a small foreign body may also be overlooked due to deficient or inappropriate methods of examination. Therefore, sporadic cases of SB are still to be expected in the future.

Adult↗
PubMed2005

Superficial siderosis of the central nervous system following cervical nerve root avulsion: the importance of early diagnosis and surgery.

Superficial siderosis (SS) of the central nervous system is an insidious, progressive, irreversible and debilitating neurological disorder caused by recurrent haemorrhage within the subarachnoid space. The subsequent deposition of haemorrhagic breakdown products in the spinal cord and nervous tissues leads to the loss of neurones and myelin, and to the development of a neurological deficit. In a small number of patients, the source of haemorrhage is related to traumatic cervical nerve root avulsion occurring several years prior to the onset of symptoms. Surgical ablation of the source has been shown to halt the progression of the disease, at least in the short term. We review the literature on SS secondary to cervical nerve root avulsion and report a further case in which surgical management was successful in halting disease progression. We emphasize that early detection and recognition of the initial non-progressive symptoms related to this poorly known disease, coupled with timely surgical management, minimizes the degree of neurological disability.

Adult↗
PubMedDate not supplied

Hearing loss and vertigo in superficial siderosis of the central nervous system.

PURPOSE: Otoneurologic findings in patients with superficial siderosis (SS) of the central nervous system are described. A 20-year-old man with acute vertigo, dizziness, with a history of head trauma in childhood; an 87-year-old woman with severe disequilibrium appearing after peridural anesthesia; and a 55-year-old woman with recurrent episodes of positional vertigo and progressive ataxia, suffering from a lumbar ependymoma are described; all patients complained of progressive bilateral hearing loss and tinnitus. Methods and results Otoneurologic examination showed bilateral sensorineural hearing loss, disturbed ocular pursuit and optokinetic nystagmus, incomplete visual suppression of the vestibulo-ocular reflex during rotatory pendular testing, right hyporeflexia, and bilateral caloric areflexia. Cerebral magnetic resonance imaging (MRI) revealed rims of hypointensity surrounding the brainstem and linear hypointensities following the surface of the cerebellar folia. CONCLUSIONS: The etiology stems from subarachnoid hemorrhage, but the source of bleeding may remain obscure. Bilateral hearing loss is described in 95% and disequilibrium in 90% because of peripheral vestibular deficit and cerebellar ataxia. In patients with progressive bilateral cochleo-vestibular deficit of unknown etiology, MRI is the examination of choice to confirm SS.

Adult↗
PubMed1993

Superficial siderosis of the central nervous system--diagnosis by magnetic resonance imaging.

Superficial siderosis of the central nervous system is a rare disorder characterized by the deposition of haemosiderin in the leptomeninges, cranial nerves and spinal cord. The clinical features include sensorineural deafness, ataxia and pyramidal tract signs. We describe two cases demonstrating the characteristic findings on magnetic resonance imaging of widespread peripheral low signal on T2-weighted images.

Adult↗
PubMed1993

Periportal fibrosis secondary to transfusion siderosis in two patients with acquired immune deficiency syndrome (AIDS) treated with zidovudine--a confusing ultrasound appearance.

We present the confusing sonographic features of periportal fibrosis in two AIDS patients who had zidovudine-induced transfusion siderosis of the liver. To our knowledge this has not been previously reported in the literature. The clinical, pathological and sonographic features are described.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Trochlear nerve palsy associated with superficial siderosis of the central nervous system.

A 56-year-old man with superficial siderosis of the central nervous system (SSCN) presented with complaints of trochlear palsy, visual field defects, gait ataxia, and hearing loss. He had no history of trauma and there were no signs of tumors or aneurysms. T2-weighted magnetic resonance imaging demonstrated characteristic hypointensity in the meninges. We believe that SSCN should be added to the differential diagnosis of trochlear nerve palsy.

Brain Diseases↗
PubMedDate not supplied

Superficial siderosis of the central nervous system. Its MRI manifestations.

Three cases of superficial siderosis of the central nervous system are reported here. Using a 1.0-T magnetic resonance (MR) unit, typical hypointense rims were observed under the brain surface on T2-weighted images. In one patient, marked atrophy of the superior cerebellar vermis and cerebellar parenchymal hyperintensity were also detected. The spinal cord was involved in two of the three patients. On T1-weighted images, hyperintense rims were demonstrated over the brain surface in two of the three patients. This finding has not been previously reported.

Aged↗
PubMed1995

Superficial siderosis of the central nervous system: a case with an unruptured intracranial aneurysm.

We present a case of superficial siderosis (SS) of the central nervous system (CNS) with an unruptured intracranial aneurysm to illustrate that the commonly encountered unexplainable progressive sensorineural hearing loss (SNHL) can be an important sign for the early awareness of this rare disorder. The literature on SS is reviewed and the pathogenesis of SS is discussed.

Central Nervous System Diseases↗
PubMed1997

Superficial siderosis of the central nervous system: an underestimated cause of hearing loss.

Superficial siderosis of the central nervous system (CNS) is a rare disease resulting in the accumulation of haemosiderin in the meninges, the brain surface, the spinal cord and the cranial nerves. The pigment is deposited as a result of chronic bleeding in the subarachnoid space. This produces a clinical picture of deafness, ataxia, cranial nerve deficits and in the latest stages dementia. In some cases the source of bleeding can be identified, whilst in others it can not. Despite its rarity the disease should be considered in the differential diagnosis of sensorineural deafness, particularly as it is a progressive and in some cases curable disease which is easily diagnosed by magnetic resonance imaging (MRI). In this case report the haemosiderin was derived from an ependymoma of the fourth ventricle with extension into the cerebello-pontine angle. The first symptom was a worsening sensorineural hearing loss.

Brain Neoplasms↗
PubMed1993

Ocular siderosis.

The authors report their experience in the management of 8 patients with ocular siderosis due to a retained intraocular foreign body (IOFB). All patients were male, aged between 19 and 39 years. Seven had a definite history of trauma; 3 had presented at the time of injury to a casualty department, and the diagnosis had been missed. The interval between injury and diagnosis ranged from 2 to 24 months. IOFB removal was performed in 7 patients: through a sclerotomy and magnet or foreign body forceps in 4 eyes and via a pars plana vitrectomy and intraocular foreign body forceps in 3 eyes. Cataract extraction was performed in 4 patients. Histological examination of specimens removed at the time of surgery showed iron deposition in the conjunctiva, anterior lens capsule and pars plana. Transmission electron microscope X-ray microanalysis showed that iron was contained in siderosomes, intracytoplasmic membrane-bound dense bodies. Final visual acuity was 6/12 or better in 6 patients and reduced to light perception in the remaining 2 due to proliferative vitreoretinopathy.

Adult↗
PubMed2007

Superficial siderosis: a case report and review of the literature.

BACKGROUND: A 48-year-old man with a past history of a car-accident-induced coma at the age of 3 years and spontaneous bilateral subdural hematomas at the age of 34 years presented with a 2-year history of worsening gait difficulties, clumsiness and dysarthria. CT scans of the brain taken during the 6 months leading up to his presentation had revealed chronic subarachnoid hemorrhage, but angiograms had not been able to identify the source of bleeding. INVESTIGATIONS: Neurological examination, brain MRI scan, lumbar puncture, four-vessel cerebral angiogram and spinal angiogram. DIAGNOSIS: Superficial siderosis; a complete review of the current literature on the condition is provided. MANAGEMENT: Iron chelating agents.

Chronic Disease↗
PubMed2002

Uroporphyria in the uroporphyrinogen decarboxylase-deficient mouse: Interplay with siderosis and polychlorinated biphenyl exposure.

Several methods have been used to develop rodent models with the hepatic manifestations of porphyria cutanea tarda (PCT). Acute iron administration or mutations of the hemochromatosis gene (Hfe) have been used to generate hepatic siderosis, a nearly uniform finding in PCT. Heterozygosity for a null mutation at the uroporphyrinogen decarboxylase (Uro-D+/-) locus has been developed to mimic familial PCT in humans. This study examines the interplay of these 2 genetic risk factors and their influence, alone and combined with polychlorinated-biphenyl exposure. Neither an Hfe-null mutation nor iron-dextran administration alone or in combination with polychlorinated biphenyl exposure was porphyrinogenic in a 3-week model using mice wild-type at the Uro-D locus. Homozygosity for an Hfe-null mutation significantly elevated hepatic iron but not to the extent seen with parenteral iron-dextran administration. Homozygosity for an Hfe-null mutation but not iron-dextran administration was porphyrinogenic in animals heterozygous for the Uro-D mutation. Polychlorinated biphenyls were also porphyrinogenic in these animals. Uroporphyria in Uro-D+/- animals was exacerbated by combinations of the homozygous Hfe-null mutation and polychlorinated biphenyls and iron-dextran and polychlorinated biphenyls. In all cases in which uroporphyria developed, a greater degree of experimental uroporphyria was seen in female animals. All elevated hepatic uroporphyrin concentrations were accompanied by depressed uroporphyrinogen decarboxylase activity and the presence of a factor in cytosol that inhibits recombinant human uroporphyrinogen decarboxylase. In conclusion, the expression of the uroporphyric phenotype, dependent on the susceptibility imparted by a genetic mutation, provides a uniquely facile model for dissecting the molecular pathogenesis of the disease.

Animals↗
PubMed1978

[Controlled desferrioxamine treatment of congenital anaemia and transfusion siderosis (author's transl)].

A 19-year-old patient with congenital Blackfan-Diamond anaemia has been maintained on a regular red cell transfusion schedule since he was two months old. From the age of four he has received desferrioxamine injections at regular intervals with different dose levels. In spite of the treatment with the chelating agent secondary siderosis developed with typical endocrinological abnormalities and cardiac arrhythmias. Increasing the dose of desferrioxamine to 16 g/24 h resulted in an iron excretion of 184 mg/24 h. Such an intensification of treatment finally produced a negative iron balance and the cardiac arrhythmias disappeared. Desferrioxamine therapy should be done under controlled circumstances only, an iron balance is mandatory, and the dose should be adjusted to the results of the balance.

Adolescent↗
PubMed2001

Hepatic failure with neonatal tissue siderosis of hemochromatotic type in an infant presenting with meconium ileus. Case report and differential diagnosis of the perinatal iron storage disorders.

We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth. Postmortem examination showed a wrinkled liver with extensive collapse of the hepatic architecture and regenerating nodules as well as hepatic and extrahepatic iron accumulation of hemochromatotic type, sparing the reticuloendothelial system. Atomic absorption spectroscopy confirmed an increase in the iron content of various organs: liver, heart, pancreas, oral salivary gland, kidney, and adrenal gland. The increase in the iron content of various organs was determined by comparing the analysis of the propositus with those of 5 gestationally age-related preterm infants who had died in the intensive care unit: 2 died of meconium aspiration syndrome, the other 3 of hyaline membrane disease, bronchopulmonary dysplasia, and immaturity, respectively. We also compared the analysis of 15 fetuses having a a condition predisposing to iron accumulation (trisomy 21, trisomy 18, cytomegalovirus, amnion infection syndrome, Rhesus- and ABO-incompatibility, congenital hemolysis, anti-phospholipid syndrome, congenital heart disease). Delta F508, the most frequent mutation seen in cystic fibrosis patients, was excluded by gene sequencing. Different noxae causing iron accumulation in the neonatal period have led to the statement that neonatal hemochromatosis may collect different etiologies, such as metabolic disorders, infections, chromosomal aberrations, and immunological disorders. In this study, we report the singular evidence of neonatal iron accumulation of hemochromatotic type in an infant presenting with meconium ileus and propose a classification of the neonatal disorders associated with iron accumulation.

DNA↗
PubMed2006

Superficial siderosis causing retrolabyrinthine involvement in both cochlear and vestibular branches of the eighth cranial nerve.

Although superficial siderosis (SS) has been clinically characterized as a combination of sensorineural hearing impairment, cerebellar ataxia and pyramidal signs, precise evaluation of the function of the eighth cranial nerve has rarely been reported. The purpose of this study was to evaluate the audiological and vestibular function. We present a patient with complaints of progressive bilateral hearing loss and gait difficulty. We evaluated the audiological and vestibular functions with auditory brainstem responses and vestibular evoked myogenic potentials (VEMPs) by clicks and galvanic stimuli. The patient showed linear hypointensities surrounding the brainstem, cerebellum and the eighth cranial nerve on T2-weighted MRI images, which is characteristic of SS. Auditory brainstem response showed only wave I in the right ear and no response in the left ear. Click VEMPs and galvanic VEMPs showed no response on either side. The results of a neuro-otological examination suggested that both audiological and vestibular dysfunction in the patient with SS is of retrolabyrinthine origin.

Ataxia↗
PubMed2002

Subacute hepatic failure after the perinatal period with haemochromatotic siderosis at the age of 11 months: an unusual perspective on neonatal haemochromatosis.

UNLABELLED: Neonatal haemochromatosis is a rare disease characterized by the early onset of liver failure, a rapidly deteriorating clinical course and high mortality. An unusual case is presented of subacute hepatic failure in which autopsy findings at the age of 11 mo showed a haemochromatotic pattern of siderosis. Despite the similarity in the pattern of iron distribution to neonatal haemochromatosis, the clinical course was highly atypical in that the infant had later and milder presentation at 1 mo of age and slow progression into liver failure. This case illustrates the heterogeneous nature of the haemochromatotic phenotype in newborns and infants. CONCLUSION: The entity "neonatal haemochromatosis" may reflect a spectrum of diseases with diverse aetiologies. Fulminant liver disease in the newborn period can also result in similar pattern of iron distribution.

Disease Progression↗
PubMed1998

Decreased bone mineral density in prepubertal children with sickle cell disease: correlation with growth parameters, degree of siderosis and secretion of growth factors.

Patients with sickle cell disease (SCD) frequently have bone disorders of multifactorial aetiology. We attempted to analyse the relationships between bone mineral density (BMD) on the one hand and auxologic parameters, degree of siderosis, function of the growth hormone (GH)/insulin-like growth factor-I (IGF-I)/IGF-binding protein 3 (IGFBP3) axis, and calcium-phosphate balance in 28 prepubertal children with SCD and 15 age-matched children with constitutional short stature (CSS). Children with SCD had significantly decreased BMD (77.9 +/- 11.9 per cent of normal BMD for age and sex) and circulating concentrations of IGF-I (91 +/- 31 ng/ml) and IGFBP3 (1.7 +/- 0.44 mg/l) compared with the control group (BMD = 93.5 +/- 8.2 per cent of normal BMD for age and sex, IGF-I = 221 +/- 48 ng/ml, and IGFBP3 = 2.3 +/- 0.34 mg/ml). GH response to provocation was defective (peak below 10 micrograms/l) in 40 per cent of children with SCD. Those with SCD with defective GH secretion had significantly lower circulating IGF-I concentration and BMD than those with normal GH secretion. Serum calcium, phosphate and alkaline phosphatase concentrations were normal in all children with SCD. BMD was correlated significantly with height, weight, and body mass index as well as with the circulating concentrations of IGF-I and IGFBP3. It is suggested that increasing the circulating IGF-I concentration, either through increasing the caloric intake of subjects and/or via GH/IGF-I therapy, may improve growth and bone mineralization in these patients.

Anemia, Sickle Cell↗
PubMedDate not supplied

High-field MR imaging of superficial siderosis of the central nervous system.

A case of superficial siderosis of the central nervous system secondary to bleeding from an ependymoma is presented. High-field magnetic resonance imaging showed marginal hypointensity of the cervical cord, medulla oblongata, pons, mesencephalon, anterior cerebellar and basal cerebral surfaces, and cranial nerves (II, V, VIII). These findings were evident in the T2 weighted images.

Adult↗