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Two remarkable events in the field of intraocular foreign body: (1) The reversal of siderosis bulbi. (2) The spontaneous extrusion of an intraocular copper foreign body.

Two unusual events concerning intraocular foreign bodies are presented. The first patient had an occult or unsuspected intraocular foreign body. He showed iridoplegia with mydriasis, siderosis iridis, and an intraocular piece of iron lying posteriorly near the retina. The foreign body was removed and the patient regained normal iris color and pupillary activity. His vision remains 20/15 six years postoperatively dispite ensuing retinal detachment one year after removal of the foreign body. The second patient was a young boy injured by a blasting cap explosion. He lost one eye from the injury and had a piece of intraocular brass in his left eye. In spite of the development of chalcosis and a mature cataract the lens gradually shrank in the pupillary space permitting a clear aphakic area and 20/25 vision. The brass fragment migrated forward and inferiorly and was finally extruded under the conjunctiva five years later, where it was removed and chemically analyzed by x-ray diffraction.

Adolescent↗

[A comment on siderosis bulbi (author's transl)].

Report on a case of advanced siderosis bulbi. Besides the typical alterations in the anterior segment of the globe, siderotic changes of the retina could be revealed. The ERG showed a reduction of the b-wave. After removal of the foreign body, a partial regression of the siderotic changes took place. In addition, the ERG became normal within 8 weeks postoperatively. The indication for removal of metallic intraocular foreign bodies despite specific alterations of the ERG is emphasized.

Adult↗

[Siderosis of the brain and spinal cord. Report of two cases].

Two cases of superficial siderosis of the brain and spinal cord with cochleovestibular and cerebellar symptoms are diagnosed on brain and spinal MRI scans. Low signal intensity lines are noted on the surface of the brainstem, cerebellum, spinal cord and within the interhemispheric and sylvian fissures. In one case, no brain or vascular malformation is identified; in the second case, two cavernous angiomas are noted on the MRI study. 3D CISS may visualize thickening of the cochleovestibular nerve.

Brain Diseases↗

[Importance of the clinical-radiological complementarity in superficial cerebral siderosis].

We report two cases of superficial siderosis of central nervous system. In one case it was idiopathic and in the other secondary to an unidentified subarachnoid hemorrhage. The symptoms that characterized the clinical picture of both were gait disturbance and hypoacusis. The MRI study showed a superficial rim of hypointensity that covered the cerebellum and brainstem, and extended along the cranial nerves and the brain surface. The findings were clearer in the T2 spin echo series. Due to the high sensitivity for hemosiderin deposits, MRI made it possible to make the final diagnosis of this rare disease.

Aged↗

[A case of superficial siderosis following subtotal removal of pituitary adenoma].

Superficial siderosis of the central nervous system (SS) is a rare clinical syndrome due to repeated intracranial hemorrhage. We report a case of SS occurring 19 years after subtotal removal of a non-functioning pituitary adenoma. The patient was a 37-year-old female, exhibiting progressive bilateral sensory neural hearing loss and cerebellar ataxia. T2 weighted magnetic resonance imaging demonstrated a rim of low intensity signal on the cerebellum, brain stem, and sylvian fissure. Immediate diagnosis based on characteristic symptoms and MRI findings is important for the prevention of irreversible progression of SS.

Adenoma↗

[Pulmonary siderosis caused by inhalation of iron dust or fumes].

Pulmonary Siderosis results from inhalation of iron dust or fumes. It falls into the group of pneumoconioses in which the pulmonary reaction is minimal despite a heavy dust load. Since fibrosis is not caused by inhalation of iron dust, the clinical course is benign and pulmonary function tests and blood gases are within normal limits. We report the first case of sideropneumoconiosis from Israel, in a 39-year-old iron welder. Since this condition is still not recognized as an occupational disease in Israel, the aim of this presentation is to bring it to appropriate medical attention.

Adult↗

Diagnostic pitfall of computed tomography in patients with superficial siderosis of the central nervous system.

Superficial siderosis of the central nervous system (SSCN) is a well-described entity with distinct clinical presentation as well as computed tomography (CT) and magnetic resonance imaging (MRI) findings. However, it is critical that when a patient previously diagnosed with SSCN undergoes CT on the brain at a later date, that this scan not be misinterpreted as a new subarachnoid hemorrhage (SAH) so unecessary repeated angiograms are not performed. This report describes such a situation and discusses unique CT findings in SSCN that have been under-recognized and under-emphasized. While conditions such as SAH should not be ruled out, they would be considered atypical. Combined with an adequate prior diagnostic testing history, physicians should proceed to MRI without subjecting the patient to repeat angiography.

Central Nervous System Diseases↗

Lymph node siderosis in trypan blue treated rats.

The accumulation of iron in the lymph nodes of trypan blue treated rats was examined as a possible experimental counterpart of the lymph node siderosis which occurs in patients with Hodgkin's disease. Lymph nodes removed from the hilus of the liver, retrosternal area, axilla and root of the small bowel mesentery were examined histologically for iron in rats receiving 6-20 subcutaneous injections of trypan blue at biweekly intervals and in control rats. An increase in erythrophagocytosis accompanied by a progressive increase in the amount of stainable iron was found in the RE cells of nodes located in the lymphatic outflow tract of the liver. As in patients with Hodgkin's disease, an increase in erythrophagocytosis together with the prolonged retention of iron by RE cells appears to account for the accumulation of iron in the lymph nodes of trypan blue treated rats.

Animals↗

[Pulmonary siderosis in an arc welder].

An arc welder of 32 years of age is presented with a random finding of miliar reticulonodular shadows in the plain film of the thorax. Subjectively and objectively the patient appears healthy. The presence of a pneumoconiosis is confirmed by biopsy. Differential diagnosis had to consider sarcoidosis and pulmonary siderosis in view of the known professional anamnesis. This case report underlines the reped demand to assess x-ray films of the thoracic organs only if the clinical findings and anamnesis are thoroughly known.

Adult↗

Host-associated iron transfer factor in normal humans and patients with transfusion siderosis.

A low molecular weight iron-binding substance that promotes bacterial growth in vitro by increasing iron availability was identified in human blood and urine. Partial purification and physical characterization indicate that this factor is similar to the host-associated iron transfer factor (HAITF) previously isolated from mammalian tissue. HAITF was found to be significantly elevated in the blood of patients with thalassemia who have transfusional siderosis. The level of HAITF in the blood of these patients was also found to correlate with that of serum iron and serum glutamic-oxaloacetic transaminase (SGOT) but not with that of serum ferritin. Thus, elevated blood levels of HAITF may explain the increased susceptibility to infection seen in patients with iron overload. Its physiologic role, however, may involve the transport of iron within cells.

Aspartate Aminotransferases↗

Cerebral siderosis: a complication of anticoagulant therapy?

The authors report a case of cerebral siderosis, a rare disease that generally follows multiple small episodes of subarachnoid hemorrhage from any source, following long-term anticoagulation and minor head injury, and document the features on MR, which demonstrates characteristic hypointensity in the meninges on T2-weighted scans.

Anticoagulants↗

Superficial siderosis--a cause of audiovestibular failure.

Bilateral vestibular end organ failure in adult life is a rare condition with some specific known causes, such as relapsing polychondritis, autoimmune inner ear disease, Lues venerium, and an acute effect of gentamicin. This case report draws attention to a rare condition that is potentially recognizable early in its development. The patient has superficial siderosis, which is iron deposits over the cerebrum, resulting in progressive neurologic failure involving all of the systems. Early in its course before other symptoms appear, there is development of progressive hearing loss and vestibular failure. This case report and literature review are given, including a potential for attempts at therapy if the disorder is recognized early.

Brain Diseases↗

[Two cases of superficial siderosis of the central nervous system. Findings of the cerebrospinal fluid, magnetic resonance imaging and positron emission tomography].

Two cases (case 1, a 45-year-old man; case 2, a 68-year-old man) of superficial siderosis of the central nervous system are presented. Main neurological symptoms were anosmia, sensorineural deafness, dysarthria, ataxia, and pyramidal tract signs. Lumbar puncture revealed bloody cerebrospinal fluid (CSF) in both cases. In case 1, the CSF became watery clear after administration of hemostatic medicines. T2-weighted magnetic resonance images showed cerebellar atrophy and marginal hypointensity of the brainstem, cerebellum, and the entire spinal cord. T2-weighted images of the cranial nerves showed hypointensity of the VIII nerves which were clinically impaired as compared with normointensity of the VII nerves which presented no clinical symptom. These findings may reflect difference in the degree of hemosiderin depostion between the VII and VIII nerves. While case 1 had a borderline score of WAIS-R (IQ79), case 2 showed overt dementia (performance IQ65). Positron emission tomography showed that cerebral blood flow and cerebral oxygen metabolism were reduced in the basal temporal lobes in both cases.

Aged↗

[Superficial siderosis of the central nervous system: an electrophysiological study].

Superficial siderosis of the central nervous system (SS) is a rare neurological disorder characterized by symptoms such as neurosensory hearing loss, ataxic gait, and spastic paraparesis. Recently, magnetic resonance imaging (MRI) enables us to make a clinical diagnosis. However, the exact pathophysiological mechanism underlying this disorder remains uncertain. Although iron chelation therapy has been attempted experimentally, it has not been successful and there is no effective medical treatment available. Towards the better understanding of the pathophysiological mechanism underlying SS, we performed electrophysiological studies, in which multiple evoked potential studies were included, in 3 patients with SS. Somatosensory evoked potentials (SEPs) evoked by median nerve stimulation were all normal, but those evoked by the posterior tibial nerve stimulation showed a significant delay of the latency of P40. In the auditory brainstem response (ABR) studies, there were no reproducible responses of the brainstem origin. In the blink reflex studies, R2 latency was delayed in one patient. In visual evoked potential (VEP) studies, the latency of P100 was delayed in two of three patients, unless all the patients clinically showed no visual symptom. The nerve conduction velocity studies performed in peripheral nerves of upper and lower extremities were all normal. The abnormal findings of ABR and SEP may suggest that the acoustic nerve and the posterior funiculus of the spinal cord are involved, respectively. These findings are also in a good agreement with pathological findings of SS reported in the literature. In SS, the hemosiderine accumulation is usually less severe in the visual tract; however, the delay of VEPs may suggest the latent dysfunctioning of the visual system in SS. It is suggested that multiple evoked potential study is useful for clinical evaluation of SS.

Aged↗

[Long-term follow-up study with PET in a case of superficial siderosis].

Long-term follow-up study with positron emission tomography (PET) has been conducted in a patient with superficial siderosis for ten years. A 63-year-old right-handed woman began to exhibit a cerebellar ataxia when she was 52 years old. Thereafter she has been exhibiting a slowly progressive course of pyramidal signs, hearing loss, anosmia, bilateral sciatica and memory disturbance in addition to the cerebellar ataxia. Series of x-ray CT and MRI disclosed a progressive atrophy of the cerebellum, specifically in the superior vermis, and a mild diffuse atrophy in the cerebral hemispheres, whereas no significant atrophy was seen in the brainstem. A marked hypointensity was seen along the rim of the brain structures including brainstem, cerebellum, sylvian fissures and the medial temporal lobes on MR T 2-weighted images. This hypointensity was also found at the edges of the third and fourth ventricles. These findings were regarded as haemosiderin deposit. By using oxygen-15, PET was carried out repeatedly with ten-year interval. In the initial PET study, both cerebral blood flow (CBF) and cerebral metabolic rate of oxygen (CMRO2) were mildly decreased in the cerebellar hemispheres and the occipital cortices. The follow-up PET study revealed a progressive reduction of CMRO2 in the brainstem, cerebellar hemispheres and temporal cortices including the hippocampus in which the haemosiderin deposition was marked on MRI, whereas the reduction of CBF was not advanced as compared with CMRO2. It was suggested that the progressive reduction of CMRO2 in the brain sites where the haemosiderin deposition was observed on MRI reflected the clinical course of neurological deterioration.

Brain↗