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At least 109 records · Page 6Linked to original sources
PubMed2006

Superficial siderosis.

BACKGROUND: Superficial siderosis (SS) of the CNS is caused by repeated slow hemorrhage into the subarachnoid space with resultant hemosiderin deposition in the subpial layers of the brain and spinal cord. Despite extensive investigations, the cause of bleeding is frequently undetermined. OBJECTIVES: To review the clinical and imaging features of 30 consecutive patients with SS and provide insights into the underlying causes of subarachnoid bleeding in this disabling disorder. METHODS: The authors reviewed the medical records of 30 consecutive patients with clinical and MRI evidence of SS. RESULTS: The commonest neurologic manifestations included gait ataxia and hearing impairment. A clinical history of subarachnoid hemorrhage was relatively rare. Possible predisposing conditions were identified on history in 22 patients, the commonest being a prior trauma (15 patients). In addition to the characteristic MRI findings of SS, 18 patients had abnormalities on MRI possibly related to chronic bleeding. The most common of these was the presence of a fluid-filled collection in the spinal canal seen in 14 patients. CONCLUSIONS: A history of prior subarachnoid hemorrhage is often absent in patients with superficial siderosis (SS). A past history of trauma is common. Prior intradural surgery may be an additional risk factor. Xanthochromia or the presence of red blood cells in the CSF is a common finding. Only rarely does angiography demonstrate the bleeding source. The presence of a fluid-filled collection in the spinal canal is a common finding on MRI and is likely related to the SS. With longitudinally extensive cavities, a dynamic CT myelogram may help localize the defect and direct the site of laminectomy. Surgical repair of a dural defect, if present, should be considered. Surgical correction of bleeding should be documented by CSF examination months after surgery. Friable vessels in the dural defect are a possible source of the chronic bleeding.

Adult↗
PubMed1993

Superficial siderosis of the central nervous system: magnetic resonance imaging and pathological correlation. Case report.

The authors report a 32-year-old woman who had undergone repair of an occipital encephalocele in infancy and who experienced a 20-year history of progressive hearing loss and intermittent vertigo. After parturition, she developed a rapidly progressive quadriparesis and brain-stem dysfunction associated with persistent intraventricular and subarachnoid hemorrhage. Serial magnetic resonance (MR) images showed progressive deposition of hemosiderin along the surface of the brain, brain stem, and spinal cord, and enhanced thickened membranes at the site of the original encephalocele repair. Posterior fossa exploration disclosed hemorrhagic membranes, which were resected; despite removal of this tissue, the patient deteriorated and died. Postmortem examination confirmed iron-containing pigment along the meninges, cerebral hemispheres, brain stem, spinal cord, and cranial nerves accompanied by atrophy of the superficial cerebellar cortex. It is concluded that superficial siderosis may accompany encephalocele repair. This is believed to be the first report in the literature of superficial siderosis of the central nervous system to correlate in vivo MR images with autopsy results.

Adult↗
PubMed1998

Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature.

Meningeal melanocytoma is a benign melanocytic tumor that originates most frequently from the melanocytes in the posterior fossa or along the spinal cord. This tumor generally occurs as an extraaxial mass that compresses adjacent neural structures to produce various neurological signs. The authors describe an unusual case in which a patient with a meningeal melanocytoma located at the thoracic spinal cord presented with superficial siderosis of the central nervous system (CNS). Extensive neuroradiological studies identified the presence of a spinal cord tumor, and postsurgical histological examination revealed the meningeal melanocytoma as a bleeding source. After surgery, lumbar puncture demonstrated normalization of the patient's cerebrospinal fluid; however, no neurological improvement occurred. The neurological deficits seem irreversible. Meningeal melanocytoma is biologically benign and can be cured by complete surgical resection; therefore, this tumor should be included in the differential diagnosis of pigmented lesions of the CNS. The authors reviewed 14 cases of well-documented meningeal melanocytoma in the literature and discuss the clinical, radiological, and pathological features of the present case to emphasize the importance of early diagnosis and identification of the source of bleeding in patients with superficial siderosis.

Brain Diseases↗
PubMed1999

[Superficial siderosis of the central nervous system].

We report five cases of superficial siderosis of the central nervous system. All patients developed progressive deafness and cerebellar ataxia associated with pyramidal tract signs or mental deterioration. The cerebrospinal fluid examinations usually revealed an elevated protein level, without other abnormalities. Magnetic resonance imaging typically showed a hypointense rim around the cerebral and cerebellar hemispheres, the brainstem and the spinal cord on T2-weighted images. A definite source of bleeding was only found in two patients. The literature on superficial siderosis is reviewed. The etiologies and the pathogenesis are discussed.

Adult↗
PubMed1999

Superficial siderosis of the CNS associated with multiple cavernous malformations.

Superficial siderosis of the CNS due to chronic, recurrent subarachnoid hemorrhage is an uncommon and potentially debilitating disorder. The classic clinical manifestation is progressive bilateral sensorineural hearing loss (SNHL), although ataxia and pyramidal signs also are observed frequently. Cavernous malformations rarely present with subarachnoid hemorrhage. We describe an unusual case of a young patient who presented with progressive, bilateral SNHL who was found to have superficial CNS siderosis associated with multiple cavernous malformations.

Adult↗
PubMed2002

MRI findings in a case of a superficial siderosis associated with an ependymoma.

We present the imaging findings of superficial siderosis of the central nervous system associated with an ependymoma of the posterior fossa in a patient who presented with progressive bilateral sensorineural hearing loss and cerebellar ataxia. The ependymoma was a homogeneous well-defined mass of the fourth ventricle without hydrocephalus. Secondary siderosis due to chronic bleeding from the ependymoma appeared as linear hypointensity delineating the surface of the cortex, thin and subtle on spin-echo T2-weighted images, thick and obvious on gradient-echo T2-weighted images.

Adult↗
PubMed2004

Idiopathic superficial siderosis: a case report.

Superficial siderosis of the central nervous system (SSCN) is a very rare disorder. The clinical syndrome of SSNC consists of sensorineural hearing loss, cerebellar ataxia and myelopathy. The clinical syndrome together with the typical appearance on magnetic resonance imaging (MRI) of hyposignal intensity along the leptomeninges in T2 sequence permit the diagnosis of SSCN. A 58 year-old man who has a history of chronic progressive hearing loss and gait instability for 5 years is presented. The neurological examination revealed bilateral sensorineural hearing loss, cerebellar ataxia and mild spasticity of the lower extremities. MRI showed classical superficial siderosis in the form of hyposignal intensity along the leptomeninges in T2 sequence. The prominent sites of hemosiderin deposition in this case were cerebellar vermis, trigeminal nerves, vestibulocochlear nerves, around the brain stem and spinal cord surface. Cerebrospinal fluid findings confirmed chronic subarachnoid hemorrhage but bleeding site could not be demonstrated. There is no specific treatment available for idiopathic SSCN.

Chronic Disease↗
PubMed1987

[Functional significance of lung siderosis in electrode welders].

56 electric arc welders found in a cross-sectional study to show pulmonary siderosis in full size x-rays were clinically examined. Looking for the discussed functional, relevance of such lung diseases in welders the diagnostic program included spirometry, flow-volume-curves, body-plethysmography, single breath diffusion capacity, pulmonary compliance and ergooxytensiometry. The results are supporting that "lung siderosis" in welders is associated with slight functional deterioration in the type of interstitial lung diseases.

Electrodes↗
PubMed1978

Angiosarcoma with pulmonary siderosis and persistent reticulocytosis. Steroid responsiveness suggests an immune basis.

A 35-year old man with cough, hemoptysis, and dyspnea was found to have diffuse pulmonary infiltrates and iron-laden macrophages in the sputum. Pulmonary siderosis was confirmed by transbronchial biopsy. An associated hypochromic anemia required frequent transfusion. Though marrow iron stores were absent, reticulocytosis was maintained. Corticosteroid therapy resulted in cessation of hemoptysis, clearing of the pulmonary infiltration, and a substantial reduction in transfusion requirement. Splenectomy was of no benefit. The patient developed cerebral symptoms with seizures, and rapid deterioration led to cerebral symptoms with seizures, and rapid deterioration led to death. Disseminated hemangiosarcoma was found at autopsy. Steroid responsiveness of the associated pulmonary siderosis suggests that it had an immune basis.

Adrenal Cortex Hormones↗
PubMed1978

[Siderosis bulbi et lentis produced by intralenticular rust (author's transl)].

A 17-year-old apprentice affected with siderosis bulbi and lentis of his right eye, was examined by biomicroscopy, ultrasonography and x-rays without evidence of an i.o. foreign body. After extraction of the cataractous lens all signs of siderosis disappeared within six weeks. The histologic examination of the lens showed a small particle of subcapsular rust.

Adolescent↗
PubMed1978

[Disturbancies of iron utilization: chronic anaemia, sideroblastic anaemia, and residual endothelial siderosis (author's transl)].

Knowledge of disturbancies of iron utilization has been considerably extended by histochemical-ultrastructural findings and the results of immunoradiometric assays for serum ferritin. -- In chronic anaemia due to infections or neoplastic diseases hyposideraemia and normal unsaturated iron binding capacity were associated with increased iron retention in macrophages and slightly to highly increased serum ferritin (500--4000 ng/ml). -- 117 patients with sideroblastic anaemia formed a heterogenous group of diverse aetiology. The iron granules of ringed sideroblasts contained nonferritin iron in mitochondria. At diagnosis, a normal iron status was found in single cases. More frequently, praelatent and latent iron overload with ferritin levels up to more than 2000 ng/ml were observed. Manifest iron overload with tissue damage was mostly the result of numerous transfusions (ferritin 4700 bis 9500 ng/ml). -- After i.v. application of colloidal iron endothelial siderosis was a regular finding. The typical uniform granules representing nonferritin-iron in lysosomes disappeared in the course of 1--3 years completely. In contrast, the colloidal iron taken up simultaneously by the macrophages was rapidly transformed into ferritin and easily used up for haemoglobin synthesis when required. The corresponding increase of serum ferritin up to maximal 4000 mg/ml was dose related. Continued blood losses lead to residual endothelial siderosis after exhaustion of macrophageal iron and recurrence of iron deficiency anaemia. The serum ferritin fell to low levels (0--12 ng/ml) as observed in untreated cases.

Anemia↗
PubMed1994

[A clinical analysis of subclinical siderosis and secondary glaucoma].

In this study, we reported 6 cases of retained iron-containing intraocular foreign body with no signs of siderosis but manifestations of secondary open angle glaucoma. The clinical characteristics and the possible mechanism of this form of glaucoma were described. It is proposed to name this special and easily neglected glaucoma "subclinical siderosis secondary glaucoma".

Adolescent↗
PubMed1994

[A case of idiopathic superficial siderosis of the central nervous system].

A 59-year-old man developed a staggering and wide based-gait in July 1990. Dysarthria, hearing loss, vexation and disturbance of memory appeared in January 1991. He consulted our clinic in May 1991, and cerebellar ataxia, neurogenic bladder, and cerebellar atrophy on brain CT were noted. Subsequently, he was followed as OPCA. Brain and spinal cord MRI (T2 and proton weighted images) revealed hypointensity on the surface of the Sylvian fissure, cerebellum, brainstem and spinal cord. We diagnosed this case as superficial siderosis because of the clinical course, i.e. cerebellar ataxia, dementia and sensorineural hearing impairment, and specific findings on MRI. We consider this case idiopathic superficial siderosis because the origin of the bleeding source was unknown. IMP-SPECT showed low perfusion in the cerebellum and frontal lobe where hemosiderin was heavily deposited. RI cisternography revealed a disturbance of CSF absorption even after 48 hours. The basic rhythm on EEG was slow alpha band with sporadic theta waves dominantly in the frontal lobe. His central conduction time on ABR and SEP was delayed, OKN was poorly elicited and ETT exhibited a staircase pattern. The physiological results as well as the clinical manifestations of the present case suggest that hemosiderin deposit on the surface of brain and spinal cord caused serious damage to the underlying structures.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain Diseases↗
PubMed1994

[Magnetic resonance and hepatic siderosis].

The principles of generation of magnetic resonance imaging (MRI) are resumed by briefly explaining the effects of an external magnetic field (EMF) on hydrogen nuclei and of pulses of radiofrequency (RF) radiation. The latter creates a resonant effect, and the same nuclei, moved from the external field axis, when RF pulse is stopped, will "relax" to their original alignment in the magnetic field and in so doing radiate the absorbed energy to their surroundings. This energy provides a signal that can be detected and spatially resolved by the receiver coil wrapped around the patient, through a computerized system. After briefly explaining also the distinctive parameters T2 and T1, the author presents his experience in the MRI detection of different degrees of siderosis of the liver--ranging from idiopathic and secondary haemochromatosis to milder siderosis of alcoholic liver disease and porphyria cutanea tarda. The results were accomplished by employing an equipment operating at an enhanced field strength (1.5 Tesla). Previous reports have validated this technique in order to distinguish idiopathic from secondary haemochromatosis. Furthermore, the present study shows that even low to moderate degrees of liver iron deposition can be appreciated and roughly quantitated by the decrease of the transverse relaxation time (T2), which resulted proportional to the amount of liver iron, under these operating conditions. Thus, MRI is proposed as an useful and non-invasive way to detect iron deposition and to follow up iron depletion treatments.

Hemochromatosis↗
PubMedDate not supplied

Superficial siderosis of the CNS: MR diagnosis and clinical findings.

PURPOSE: To report the clinical and neuroradiologic findings of superficial siderosis of the CNS, due to chronic subarachnoid bleeding of unknown origin. MATERIALS AND METHODS: We observed seven cases. The main clinical manifestations were progressive deafness and ataxia. Four patients had had previous cranial or cervical trauma, with root avulsion in two, many years before onset of deafness and ataxia. Neuroradiologic studies included MR (0.5 T in four and 1.5 T in three) and angiography of the brain in all cases, CT in six cases, MR of the spine in six, and myelography in four. RESULTS: MR demonstrated a rim of marked hypointensity in T2-weighted images, consistent with hemosiderin deposits, on the surface of cerebellum, brain stem, inferior part of cerebral hemispheres, and spinal cord. CT showed cerebellar atrophy in five cases, and a rim of mild hyperdensity around the brain stem in two. Angiographic studies were negative. Myelography showed cervical nerve root avulsion in two cases and a cervicodorsal extradural cyst in one. Cerebrospinal fluid contained RBCs in all the six examined cases. CONCLUSION: Although CT may occasionally suggest the diagnosis of superficial siderosis, MR demonstrates this abnormality to better advantage.

Adult↗
PubMed1994

Superficial siderosis of the central nervous system: an underdiagnosed cause of sensorineural hearing loss and ataxia.

Superficial siderosis of the central nervous system (CNS) is a rare but ultimately fatal disorder characterized by progressive hearing loss, ataxia, and dementia. Symptoms are caused by the deposition of hemosiderin in the CNS as a result of recurrent or persistent extravasation of blood into the cerebrospinal fluid. Sites most commonly involved include the cerebellum, brain stem, and eighth cranial nerve. In the past, diagnosis was most often made at autopsy, and this may account for the lack of reported cases in the otolaryngologic literature and the presumed rarity of the disorder. Now, diagnosis is confirmed by characteristic magnetic resonance imaging findings and treatment is targeted toward the source of bleeding. The diagnosis of superficial CNS siderosis should be entertained in all patients presenting with sensorineural hearing loss and ataxia.

Aged↗
PubMed1996

[A case with superficial siderosis of the central nervous system accompanied by sensory high tone hearing loss and recurrent headache and vomiting].

A 13-year-old boy with superficial siderosis of the central nervous system was reported. There were many members, including the proband, with sensory high tone hearing loss in his maternal family, but they did not have other neurological symptoms. Paroxysmal and pulsatile severe headache, and vomiting without aura appeared recurrently at the age of 8. His consciousness was alert and no other abnormal sign or symptom was seen during the attacks. The electroencephalogram and cranial computed tomogram revealed no abnormality. The T2 weighted magnetic resonance image of the cranium showed a superficial low intensity zone on the cerebellar vermis, frontal and parietal lobes of the cerebrum, and cervical and upper portion of the thoracic spinal cord at the age of 12, when he had severe headache and vomiting. Cerebrospinal fluid showed xanthochromia with mild elevation of the protein level during an attack, and a light bloody appearance during the asymptomatic state. The diagnosis of superficial siderosis of the central nervous system was made by these findings of magnetic resonance image and cerebrospinal fluid. The bleeding sources to the subarachnoid space could not be detected by cerebral angiography.

Adolescent↗
PubMed1996

Superficial siderosis and episodic fourth nerve paresis. Report of a case with clinical and magnetic resonance imaging findings.

We describe a patient with superficial siderosis who had an episodic unilateral fourth nerve paresis. The superficial siderosis was caused by small repeated intraventricular hemorrhages from a periventricular cavernous angioma. T2-weighted magnetic resonance images demonstrated a rim of low signal intensity at the brain surface, characteristic of hemosiderin deposition. These low-signal-intensity deposits included the dorsal brain stem around the anterior medullary velum. We suggest that the hemosiderin deposits affected the proximal portion of the fourth nerve where it contains central myelin and that this in some way caused unstable conduction of nerve impulses through the nerve.

Adult↗