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PubMed1992

Superficial siderosis of the central nervous system: report of three cases and review of the literature.

We present 3 cases and a review of the literature to demonstrate the current state of clinical diagnosis and therapy of superficial siderosis of the central nervous system. Typical symptoms were progressive cerebellar ataxia, spasticity and hearing loss. Repeated subarachnoid hemorrhage was indicated by persistent xanthochromia of the cerebrospinal fluid and confirmed by the presence of erythrophages, siderophages and iron-containing pigments. Deposition of free iron and hemosiderin in pial and subpial structures leads to intoxication of the central nervous system and represents the pathophysiological mechanism of superficial siderosis. Hypointensity of the marginal zones of the central nervous system on T2 weighted MR images indicates an iron-induced susceptibility effect and seems pathognomonic for superficial siderosis. In 39 of the 43 previously described cases superficial siderosis was verified by biopsy or autopsy. Today magnetic resonance imaging enables diagnosis at an early stage of the disease. Therapeutic management requires the elimination of any potential source of bleeding. In patients with unknown etiology no proofed therapy is yet available.

Aged↗

PubMed1999

Siderosis bulbi resulting from an intralenticular foreign body.

PURPOSE: To report a case of siderosis bulbi that resulted from a small intralenticular foreign body. METHOD: Case report. RESULTS: A 36-year-old man with normal visual acuity and a peripheral intralenticular iron foreign body in the left eye was treated conservatively. Nine weeks after the injury, he had ocular signs of siderosis bulbi, with changes in the electroretinogram. A clear lens aspiration with removal of the foreign body was performed. After removal of the iron foreign body, no progression or regression of the ocular signs of siderosis bulbi has occurred, and the electroretinogram has not changed over a 2-year period. CONCLUSIONS: Even in the presence of good vision, a patient with an intralenticular ferrous foreign body should be followed closely, and the foreign body should be removed before irreversible siderosis bulbi occurs.

Adult↗

PubMed2000

Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report.

BACKGROUND: To our knowledge, there are only two reported cases of cerebral arteriovenous malformation associated with superficial siderosis. In both cases, the patients were asymptomatic and were discovered on retrospective review of magnetic resonance imaging. CASE DESCRIPTION: We describe a case of superficial siderosis in a 47-year-old male with a remote history of closed head injury that presented with progressive hearing loss, cerebellar ataxia, and urinary incontinence. Lumbar puncture was indicative of active subarachnoid bleeding. Cerebral angiography revealed a small vascular malformation that was resected. RESULTS: Pathological examination confirmed the diagnosis of the arteriovenous malformation. Six months after surgery the patient is neurologically stable with no further progression of clinical signs or symptoms. CONCLUSIONS: We report the first case of surgical resection of an intracranial arteriovenous malformation for the treatment of superficial siderosis. We emphasize the necessity of a detailed evaluation when superficial siderosis is suspected to localize and resect potential bleeding sources, because the disease is progressive and often irreversible.

Brain Diseases↗

PubMed1997

Ocular siderosis. Diagnosis and management.

PURPOSE: To assist clinicians in the diagnosis and management of ocular siderosis. METHODS: The diagnosis and management of three cases of ocular siderosis secondary to a retained iron-containing intraocular foreign body are described. RESULTS: Noteworthy features included: 1) the characteristic features of a tonic or Adies pupil (one case) and 2) the failure of high-resolution computed tomography scanning and plain film radiography to detect the intraocular foreign body (two of three cases). In contrast, ultrasonography demonstrated the intraocular foreign bodies in all cases and accurately localized them to the inferior retinal quadrants. A third noteworthy finding was that a preoperative electroretinogram reduction in amplitudes of as much as 40% compared with that of the uninvolved eye was compatible with excellent vision, and that the electroretinogram returned to normal after the intraocular foreign body was removed. CONCLUSIONS: Ocular siderosis should be considered in the differential diagnosis of a tonic or Adies pupil. To detect an occult intraocular foreign body, clinicians should not rely exclusively on computed tomography scanning or plain film radiography, but should also use B-mode echography with careful study of the inferior quadrants. In ocular siderosis a preoperative electroretinogram reduction in amplitudes of as much as 40% may be reversible after intraocular foreign body removal.

Adie Syndrome↗

PubMed2003

Superficial siderosis of the meninges and its otolaryngologic connection: a series of five patients.

OBJECTIVE: To study the otolaryngologic disorders in patients with superficial siderosis of the meninges. BACKGROUND: Superficial siderosis of the central nervous system is a rare disorder characterized by progressive bilateral hearing loss and ataxia caused by recurrent bleeding into the subarachnoid space. The cerebellum, eighth cranial nerve, and olfactory nerve are particularly susceptible to the deposition of hemosiderin, which is responsible for the symptoms. The diagnosis is confirmed by magnetic resonance imaging. METHODS: The clinical notes of five patients with superficial siderosis of the meninges were reviewed with the intent of reporting the otolaryngologic symptoms and signs, the clinical investigations, and treatments. RESULTS: Four of the five patients had sensorineural deafness, two had smell disturbances, and three had gait abnormalities. Magnetic resonance imaging was the most important investigation used to identify the condition. CONCLUSIONS: Superficial siderosis of the meninges is an important differential diagnosis in patients with progressive sensorineural deafness.

Adolescent↗

PubMed1991

The cytological recognition of African siderosis on bone smears.

Eight cases of African siderosis primarily recognized on cytological smears are presented and discussed. All the smears were obtained from Jamshidi needle biopsies of vertebral bodies. Six cases showed siderosis only, while a seventh showed two pathological processes on one slide, namely metastatic keratinizing squamous carcinoma and siderosis. The remaining case showed cytological evidence of tuberculosis and siderosis. All cases were histologically confirmed, an additional feature in two cases being osseous tuberculosis which was not evident on the cytological smears. A search of the literature failed to reveal any report on the cytological recognition of this disease, or its association with tuberculosis.

Adult↗

PubMed2003

Superficial siderosis of the central nervous system many years after neurosurgical procedures.

Recurrent haemorrhage into the subarachnoid space causes superficial siderosis, which clinically manifests as cerebellar ataxia, sensorineural hearing loss, and myelopathy. Two patients developed clinical, radiological, and biochemical evidence of superficial siderosis many years after surgery. One had two posterior fossa operations, a left temporal craniectomy, and radiotherapy for a presumed brain tumour before developing clinical evidence of superficial sidersosis 37 years later. The other had small bilateral subdural collections from recurrent shunt revisions following posterior fossa surgery for a Chiari malformation, and then developed deafness and ataxia. The first patient currently has the longest recorded delay between presumed subarachnoid bleeding and clinical manifestations of superficial siderosis. Both patients provide further evidence that superficial siderosis of the central nervous system, a progressive neurodegenerative vascular condition, may be a delayed complication of neurosurgical procedures.

Adult↗

PubMed1999

[A case of superficial siderosis of the central nervous system--findings of the neuro-otological tests and evoked potentials].

We report a 57-year-old woman with superficial siderosis of the central nervous system. On physical examination, sensorineural hearing impairment, cerebellar ataxia, pyramidal tract signs, dysarthria, and neurogenic bladder were seen. Brain and spinal cord MRI (T2 and proton weighted images) revealed cerebellar atrophy and marginal hypointensity of the Sylvian fissure and brain stem, cerebellum and spinal cord. We diagnosed this patient as superficial siderosis because of the clinical course and specific findings on MRI. We consider this case idiopathic superficial siderosis because the source of the bleeding was unknown. Neuro-otological tests and BAEP disclosed retrocochlear deafness. Her central motor conduction time on MEP was delayed. We discussed the pathogenic mechanism of these conditions, and we concluded that we must take notice of myelinopathy in superficial siderosis and that MEP was useful for detecting them.

Audiometry, Evoked Response↗

PubMed1975

Gastrojejunostomy inhibits postshunt siderosis.

Thirteen of fourteen dogs developed marked increases in hepatic hemosiderin (postshunt siderosis) after construction of portacaval shunt, whereas only three of 12 dogs developed such siderosis after construction of a portacaval shunt and end-to-side gastrojejunostomy with duodenal exclusion. Splenic hemosiderin was increased markedly in dogs with portacaval shunt but increased minimally in dogs with portacaval shunt and gastrojejunostomy. Rib marrow fat increased slightly in dogs with portacaval shunt and decreased slightly in dogs with portacaval shunt and gastrojejunostomy. Thus continued iron absorption appears to account for the development of postshunt siderosis and the addition of gastrojejunostomy ameliorates postshunt siderosis by diminishing iron absorption.

Adipose Tissue↗

PubMed2006

[The characteristics of ultrasound biomicroscopy images of siderosis].

PURPOSE: To evaluate the characteristics of ultrasound biomicroscopy (UBM) image and the rate of positiveness of diagnosis for foreign bodies in siderosis. METHODS: Sixteen cases of siderosis were examined with UBM and the data ware analyzed, compared with the data of X-ray and the record of operation. RESULTS: The data of UBM showed deposition of rust in different tissues of eye-balls in the 16 cases of siderosis. Within the 10 cases of foreign bodies found by X-ray, only 4 cases were positive by UBM. X-ray data showed that the remaining 6 cases were negative of foreign bodies, but 1 case was found positive by UBM. The foreign bodies of the 11 cases were removed by operation. The characteristics of UBM images of foreign bodies displayed high and strong reflectivity with ultrasonic shadow, while the UBM image of siderosis showed multiple high and strong spot-like, speckled or thread-like shadows without "tails". CONCLUSION: UBM could detect the exact location of intraocular foreign bodies and rust. The positive rate of diagnosis for foreign bodies of UBM was lower than X-ray.

Adolescent↗

PubMed1984

[Determination of serum ferritin in porphyria cutanea tarda. A reliable sign of hepatic siderosis].

Some parameters of iron metabolism in 26 patients with porphyria cutanea tarda (PCT) which is often associated with mild iron overload and hepatic siderosis, are studied. Serum iron, percent transferrin saturation and ferritin were pathologically increased. Statistical comparisons were performed between PCT patients and healthy controls, liver disease patients (cirrhosis, chronic active hepatitis) and patients with associated liver siderosis (alcoholic cirrhosis, cirrhosis and chronic active hepatitis in thalassemia). Ferritin levels are higher in patients with porphyria than in healthy controls (p less than 0,001) and in patients without liver siderosis (p less than 0,001). No statistical difference is observed between patients with porphyria and patients with siderosis. A significant decrease in ferritin levels is registered after venesection therapy. The conclusion is drawn that serum ferritin increase in PCT is related to hepatic iron store amounts rather than hepatic necrosis. It is assumed that ferritin follow-up during phlebotomy therapy and also during remission is useful to indicate the exhaustion or an early replenishment of hepatic iron stores.

Adult↗

PubMed1998

[Superficial siderosis appeared in a case of suprasellar embryonal carcinoma].

A case of superficial siderosis that appeared in a case of suprasellar embryonal carcinoma is reported. A 24-year-old man presented polydipsia and vertigo. MRI revealed a suprasellar tumor. The tumor contained high intense spots on T1-weighted images, suggesting intratumoral hemorrhage. He underwent a surgery, which proved it as embryonal carcinoma pathologically. Cerebrospinal fluid was xanthochromic at surgery, suggesting terminal hemorrhage. After surgery, he received a total dose of 56 Gy of irradiation. Tumor decreased in size and symptoms improved. However, he presented occipital headache 7 months after surgery. MRI showed disseminated tumors in the subarachnoid spaces. He received irradiation for the whole spine and adjuvant chemotherapy. During treatment, MRI demonstrated low signals on the surface of the brain stem, suggesting the superficial siderosis. The lesions spread to the surface of the cerebellum and tentorium cerebelli. Superficial siderosis is characterized by the deposition of hemosiderin in the leptomeninges, cranial nerves and spinal cord. The etiology of the hemosiderin deposition is thought to be chronic or recurrent bleeding into subarachnoid space. Experimentally, similar lesions have been produced in the animals following intrathecal injection of blood or hemolysed red cells. In the literature, MRI demonstrated a rim of marked hypo-intensity on T2-weighted images, consistent with hemosiderin deposits, on the surface of cerebellum and brain stem. Gradient-echo sequences have been more sensitive than T2-weighted images of spin echo sequences. In the present case, the superficial siderosis seems to be due to chronic tumoral hemorrhage. This phenomenon could be related to chemotherapy using CDDP and etoposide.

Adult↗

PubMed2000

Diagnostic accuracy of abdominal ultrasonography compared to magnetic resonance imaging in siderosis of the spleen.

A prospective study to compare the diagnostic performance of ultrasonography with magnetic resonance imaging using gradient-recalled echo technique in cases of siderosis of spleen was conducted in 53 cirrhotic patients with endoscopic proof of gastroesophageal varices. Of the 34 patients with splenic siderosis on MR imaging, punctate hyperechoic foci were detected in the spleen on ultrasonography in 24 patients. Using MR imaging as the reference standard for the diagnosis of splenic siderosis, the sensitivity of ultrasonography is 70.6%; specificity is 78.9%; positive predictive value is 85.7%; and negative predictive value is 40%. We conclude that ultrasonography is a fairly accurate technique in the diagnosis of splenic siderosis.

Adult↗

PubMed1993

[Differential liver siderosis diagnosis using quantitative iron determination in the biopsy specimen].

Quantitative iron investigations were carried out in the leavings of liver biopsy specimens from 92 patients with liver siderosis, detected by histochemical methods. A discrimination between primary and secondary liver siderosis were tried by the liver iron index. Idiopathic hemochromatosis was not found in liver iron indices below 2, both hemochromatosis and secondary liver siderosis were found with an index above 2. Only idiopathic hemochromatosis was found with an index above 5 with the exception of one transfusional siderosis in refractory anemia.

Biopsy↗

PubMed1999

Superficial siderosis of the central nervous system and anticoagulant therapy: a case report.

Superficial siderosis of the central nervous system is a rare condition characterized by deposition of haemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. With the widespread use of magnetic resonance imaging, an increasing number of cases of superficial siderosis are being discovered, secondary forms being more frequent than idiopathic ones. We report a 78-year-old man in oral anticoagulant therapy, who presented neurosensory hearing loss, gait ataxia and spastic paraparesis. Magnetic resonance imaging suggested the diagnosis of superficial siderosis of the central nervous system, without an evident bleeding source.

Aged↗

PubMed1990

Fetal liver disease may precede extrahepatic siderosis in neonatal hemochromatosis.

Three children of a mother with biopsy-confirmed posttransfusional hepatitis of undetermined etiology (non-A, non-B hepatitis) died in utero or in infancy. All had liver disease of intrauterine onset. The two liveborn children died of the consequences of severe hepatic insufficiency manifest at birth and met clinicopathologic criteria for neonatal hemochromatosis. Although hepatic architecture in the stillborn fetus was markedly disordered, with hepatocyte giant cell transformation, extrahepatic siderosis was not present and hepatic siderosis was minimal. These findings indicate that in some cases of neonatal hemochromatosis, extrahepatic siderosis may be caused by hepatic injury rather than primarily due to excessive transport of iron from mother to fetus and support speculation that in some instances an infective agent may be responsible.

Adolescent↗

PubMed1992

Experimental superficial siderosis of the central nervous system: biochemical correlates.

The pathogenesis of superficial siderosis of the central nervous system (CNS) may be examined by the repeated intracisternal injection of washed autologous red blood cells (RBC). In rabbits, the injections cause the accumulation of iron in the cytoplasm of microglial cells and astrocytes of cerebellar and cerebral cortices. Immunocytochemistry for ferritin reveals enhanced reaction product mainly in microglia but hemosiderin occurs only after extending the injections to 6 months. In an effort to determine the biochemical correlates of these morphological changes, iron, ferritin, ferritin subunits and the ferritin repressor protein (FRP) were quantitated. There was no increase of total iron or ferritin in the exposed cortical areas. However, the injections of RBC caused dramatic shifts of the relative contributions by heavy (H-) and light (L-) ferritin subunits. The initial response was a prompt increase of the H/L ratio to over 4.0 from the normal ratio near 1.0. Extended injections caused the ratio to drop to below unity, and the predominance of L-ferritin at 6 months coincided with the appearance of granular hemosiderin. This investigation also confirmed the presence of FRP in rabbit brain cytosols but the induction of experimental superficial siderosis did not change its levels or in vitro affinity for the iron-responsive element in ferritin messenger ribonucleic acid. It is proposed that the incrustation by hemosiderin which characterizes superficial siderosis of the CNS in humans occurs when prolonged exposure to hemoglobin produces persistent shifts of the H/L-ratios by accumulation of L-ferritin.

Animals↗

PubMed2005

CNS siderosis and dandy-walker variant after neonatal alloimmune thrombocytopenia.

This report presents a case of superficial siderosis of the central nervous system secondary to repeated intraventricular and subarachnoid bleeding of a newborn infant with neonatal alloimmune thrombocytopenia. In addition, this infant manifested Dandy-Walker variant. To date, the few known cases of superficial siderosis in neonates have not been associated with neonatal alloimmune thrombocytopenia or Dandy-Walker complex. We believe that repeated bleeding in the central nervous system from early fetal life, especially in the posterior fossa, may produce cerebellar atrophy as occurs in adults with superficial siderosis of the central nervous system.

Cerebellum↗