Siderosis idiopática del sistema nervioso central y asociación con atrofia óptica e infarto cerebral. Caso clínico / Superficial siderosis of the central nervous system. Report of one case

Superficial siderosis of the central nervous system is produced by the deposit of hemosiderin within the subpial layers of the central nervous system and central parts of the cranial nerves, leading to progressive degeneration. We report a 55-year-old male who consulted for hearing loss and long-standing progressive decrease in visual acuity, associated with sudden onset of left hemiparesis. A brain CAT scan showed subacute ischemic lesions in the territory of the right posterior cerebral artery (thalamus and right subcortical temporal regions), old ischemic lesions in the right subcortical occipital regions and cerebellar atrophy. A magnetic resonance confirmed the lesions and the presence of superficial diffuse siderosis. A cerebrospinal fluid analysis showed slight xanthochromia, 26 leukocytes/mm3, glucose 51 mg/dL and proteins 1.23 g/L. He was managed with aspirin in low doses and statins. His motor function improved and was discharged two weeks after admission.

Different etiologies of superficial siderosis / Diferentes etiologias de siderose superficial

Superficial Siderosis (SS) is an uncommon condition caused by hemosiderin deposition into the subarachnoid space. SS is characterized by cerebellar ataxia, progressive sensorineural hearing loss and pyramidal signs, but is often an unrecognized disorder. Magnetic Resonance Imaging (MRI) is the diagnostic procedure of choice due its high sensitivity to hemosiderin deposits in addition to being a non-invasive exam. This paper aims to describe a case of SS and to perform a literature review about SS etiologies, neuroimaging features and clinical characteristics. A 65-year-old man came to a neurology outpatient clinic with seizures and cerebellar ataxia with a history of car accident and severe traumatic brain injury 45 years ago. MRI SWAN showed a hyposignal in the cisterns of the base and on the cerebellar surface and T1-weighted images left hippocampal sclerosis.

A Siderose Superficial (SS) é uma condição rara causada por depósitos de hemossiderina no espaço subaracnóideo. SS é caracterizada por ataxia cerebelar, perda neurosensorial auditiva progressiva e sinais piramidais, mas é frequentemente uma desordem de difícil diagnóstico. A Ressonância Magnética (RM) é o exame de escolha para o diagnóstico devido a sua alta sensibilidade aos depósitos de hemossiderina, além de ser um exame não invasivo. Este artigo tem como objetivo descrever um caso de SS e realizar uma revisão da literatura sobre as etiologias da SS, suas características na neuroimagem e suas características clínicas. Um homem de 65 anos de idade procurou o ambulatório de neurologia com convulsões e ataxia cerebelar. Ele informou histórico de acidente automobilístico e lesão cerebral traumática grave há 45 anos. A RNM SWAN mostrou hipossinal nas cisternas da base e na superfície cerebelar e as imagens em T1 evidenciaram a presença de esclerose hipocampal esquerda.

Asymptomatic Cerebral Small Vessel Disease: Insights from Population-Based Studies / 대한뇌졸중학회지

Cerebral small vessel disease (CSVD) is a common group of neurological conditions that confer a significant burden of morbidity and mortality worldwide. In most cases, CSVD is only recognized in its advanced stages once its symptomatic sequelae develop. However, its significance in asymptomatic healthy populations remains poorly defined. In population-based studies of presumed healthy elderly individuals, CSVD neuroimaging markers including white matter hyperintensities, lacunes, cerebral microbleeds, enlarged perivascular spaces, cortical superficial siderosis, and cerebral microinfarcts are frequently detected. While the presence of these imaging markers may reflect unique mechanisms at play, there are likely shared pathways underlying CSVD. Herein, we aim to assess the etiology and significance of these individual biomarkers by focusing in asymptomatic populations at an epidemiological level. By primarily examining population-based studies, we explore the risk factors that are involved in the formation and progression of these biomarkers. Through a critical semi-systematic review, we aim to characterize “asymptomatic” CSVD, review screening modalities, and draw associations from observational studies in clinical populations. Lastly, we highlight areas of research (including therapeutic approaches) in which further investigation is needed to better understand asymptomatic CSVD.

Superficial siderosis of the central nervous system caused by myxopapillary ependymoma of conus medullaris and cauda equine: a case report and literature review / 北京大学学报(医学版)

Superficial siderosis of the central nervous system (SSCNS) is a rare disorder caused by hemosiderin deposits in the subpial layers of the brain and spinal cord due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). Central nervous system tumor could be one of the sources of bleeding. Some problems exist at present regarding the diagnosis and treatment of SSCNS in China. On account of fewer cases, the insufficient awareness of the condition, and the lack of long-term follow-up data, enough attention has not been paid to etiological diagnosis. The speculative high rate of missed diagnoses of SSCNS indicates a great disparity in the treatment from the world's advanced level. Related data of clinical and basic research need to accumulate as soon as possible to promote the clinical diagnosis and treatment of the disease. The progressive neurological deficits are involved in the typical clinical manifestations of SSCNS with a triad of bilateral symmetrical sensorineural hearing loss, cerebellar ataxia and signs of corticospinal tract dysfunction. Nevertheless, there are few patients with the triad signs at the same time, which lead to a delayed diagnosis or misdiagnosis. Detection of this disease was commonly post-mortem until the advent of MRI with signal and location characteristics, which made diagnosis easier. Siderosis appears as a hypointense rim covering the surface of the cerebellum, the brain stem, the spinal cord, similar to a black pencil line, thin on SE-T2-weighted images, thick and conspicuous on GE-T2-weighted images or on susceptibility-weighted imaging (SWI). The only effective way of treating the disorder is to identify the source of bleeding and remove it. MR examination is useful for seeking a source of bleeding too. Therefore, once superficial siderosis is considered, lesions of the central nervous system must be searched using MRI of the brain and spine. We report here a 37-year-old male diagnosed of SSCNS with the classical clinical symptoms of cerebellar ataxia, sensorineural hearing loss and myelopathy. T2-weighed MRI showed characteristic marginal hypo-intensity around the central nervous system. Etiological explorations revealed a large conus medullaris / cauda equina ependymoma filling the lumbosacral spinal canal, a myxopapillary ependymoma (MPE) confirmed by surgical resection and histopathological examination. The related literature was reviewed to ascertain the mechanism of SSCNS secondary to MPE, and to discuss the pathogenesis, clinical features, diagnosis and treatment of SSCNS. This paper aims to improve the awareness of SSCNS and diagnostic level, and to lay stress on the etiological explorations that is beneficial to the development of exact treatment plan.

Pathologically Confirmed Cerebral Amyloid Angiopathy with No Radiological Sign in a Patient with Early Onset Alzheimer's Disease

Cerebral amyloid angiopathy (CAA) is associated with perivascular disruption, which is caused by progressive amyloid-beta (Aβ) deposition in vessels. Previous autopsy studies have shown that the prevalence of CAA in Alzheimer's disease (AD) is 70% to 90%. CAA is principally characterized by restricted lobar microbleeds (MBs), which can be detected by gradient-echo T2* (GRE) and susceptibility-weighted imaging (SWI). We herein report on a 62-year-old man who presented with 8 years of memory impairment. The apolipoprotein E (APOE) genotype was ε4/ε4, and a brain GRE performed 28 months before death revealed mild atrophy and no MBs. At autopsy, the patient scored “A3, B3, C3” according to the National Institute on Aging-Alzheimer's Association guidelines; the patient thus exhibited a high level of AD neuropathological changes. Furthermore, immunohistochemical staining for Aβ showed antibody accumulation and severe cerebral amyloid angiopathic changes in numerous vessels with amyloid deposits. Our case suggests that radiological CAA markers, such as cerebral microbleed (CMB) or cerebral superficial siderosis, may not suffice to detect amyloid angiopathy in cerebral vessels. CAA should therefore be considered as a combined pathology in APOE ε4 homozygotes with AD, even if such patients do not exhibit CMB on MRI.

Craniopharyngioma with Intratumoral Hemorrhage and Superficial Siderosis

Superficial siderosis of the central nervous system (CNS) is a progressive and debilitating neurological disease manifesting sensorineural hearing loss, cerebellar ataxia, and pyramidal tract signs. Chronic extravasation of blood into the subarachnoid space results in the accumulation of hemoglobin derivate in the subpial layer of the CNS, which is toxic to the neural tissues. Craniopharyngioma is a benign third ventricle tumor, which rarely presents with tumor bleeding. We report a rare case of superficial siderosis associated with craniopharyngioma with intratumoral hemorrhage in a patient with no history of prior trauma or CNS surgery.

Symptomatic Relief of Idiopathic Infratentorial Superficial Siderosis with Maintaining Supine Position

Superficial siderosis results from the deposition of hemosiderin in subpial layers of the central nervous system following hemorrhage in subarachnoid spaces. Infratentorial superficial siderosis (ISS) presents with unique clinical features including progressive hearing loss, ataxia, and myelopathy, and the most common cause of idiopathic ISS is dural abnormality. Here we report a case of idiopathic ISS with radiological findings of spontaneous intracranial hypotension, whose clinical symptoms of ISS including cerebellar dysfunction improved after supine position was maintained for 2 months.

Superficial Siderosis with Peripheral Dizziness: Report of 2 Cases / 대한평형의학회지

Superficial siderosis (SS) of the central nervous system is a rare disease, which is caused by the accumulation of iron from the hemoglobin in the superficial layer of the brain, spinal cord, and central parts of cranial nerves. The etiology of SS is the accumulation of hemosiderin in the subarachnoid space due to chronic or repeated hemorrhage resulting in progressive and irreversible neurological dysfunction. The cause of the disease is aneurysm, trauma, tumor, and vascular malformation. In most cases, the cause of bleeding is unknown. Clinical features include sensorineural hearing loss, cerebellar ataxia, and myelopathy. Until now, magnetic resonance imaging (MRI) has only been diagnosed and there is no standardized treatment. We will investigate clinical features and MRI findings of SS disease in the central nervous system using 2 patient cases.

Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder / Siderose superficial do sistema nervoso central é uma doença rara e possivelmente subdiagnosticada

ABSTRACT Superficial siderosis (SS) of the central nervous system (CNS) is a rare and possibly underdiagnosed disorder resulting from chronic or intermittent bleeding into the subarachnoid space, leading to deposition of blood products in the subpial layers of the meninges. Magnetic resonance imaging (MRI) shows a characteristic curvilinear pattern of hypointensity on its blood-sensitive sequences. Methods Series of cases collected from Brazilian centers. Results We studied 13 cases of patients presenting with progressive histories of neurological dysfunction caused by SS-CNS. The most frequent clinical findings in these patients were progressive gait ataxia, hearing loss, hyperreflexia and cognitive dysfunction. The diagnoses of SS-CNS were made seven months to 30 years after the disease onset. Conclusion SS-CNS is a rare disease that may remain undiagnosed for long periods. Awareness of this condition is essential for the clinician.

RESUMO Siderose superficial (SS) do sistema nervoso central (SNC) é uma doença rara e provavelmente subdiagnosticada, resultante de sangramento crônico no espaço subaracnóide, levando ao depósito de produtos sanguíneos nas camadas meníngeas subpiais. Ressonância magnética (RM) mostra um padrão curvilíneo característico com hipointensidade nas suas sequências sensíveis a sangue. Métodos Série de casos coletados de centros brasileiros. Resultados Apresentamos 13 casos de pacientes com história progressiva de disfunção neurológica causada por SS-SNC. Os achados clínicos mais frequentes destes pacientes foram ataxia progressiva da marcha, perda auditiva, hiperreflexia e disfunção cognitiva. O diagnóstico de SS-SNC foi firmado de sete meses a 30 anos após o início da doença. Conclusão SS-SNC é uma condição rara que pode permanecer sem diagnóstico por longos períodos. O conhecimento desta entidade é essencial ao clínico.

Superficial Siderosis of the Central Nervous System Originating from the Thoracic Spine: A Case Report

Superficial siderosis of the central nervous system(SSCNS) is a rare disease characterized by hemosiderin deposition on the surface of the central nervous system. We report a case of SSCNS originating from the thoracic spine, presenting with neurological deficits including, sensorineuronal hearing loss, ataxia, and corticospinal and dorsal column tract signs. The patient underwent dural repair with an artificial dural patch. Clinical findings were elicited by neurological examination, imaging studies, and intraoperative findings, and these were addressed through literature review.

Comparison of welder's pneumoconiosis with silicosis and follow-up study of welder's pneumoconiosis / 中华劳动卫生职业病杂志

<p><b>OBJECTIVE</b>To study the character of welder's pneumoconiosis on CT, pathology, and lung function.</p><p><b>METHODS</b>To contrast 185 welder's pneumoconiosis and 115 silicosis on CT, pathology, and clinical characters which were diagnosed between Jan 2008 and Dec 2013. Chest X-ray and lung function of 39 welder's pneumoconiosis patients were followed up after diagnosed 4~6 years later.</p><p><b>RESULTS</b>Average age and working years of welder's pneumoconiosis were 36.7 and 11.5, less than silicosis patients 58.8 and 22.1, respectively (P<0.05). Of all 185 welder's pneumoconiosis 98.4% were of stage I and no stage III, while in silicosis group stage I, stage II and stage III were 56.5%, 22.6% and 20.9%, respectively. The ratio differences between the two groups were statistically significant,P<0.05. 82.7% of welder's pneumoconiosis patients were observed pathologically moderate or above dust deposition in lung tissue while interstitial fibrosis level was just mild (97.6% patients) or no fibrosis (2.4% patients). By contrast, 60.0% silicosis patients pathologically showed moderate or above dust deposition while 77.8% were of moderate or above fibrosis. CT findings in welder' s pneumoconiosis were diffuse branching linear structure (38.9%), low density consistent size centrilobular micronodules (19.5%), or both (30.8%). Poorly-defined ground-glass attenuation centrilobular micronodules or widely ground glass shadow were observed in 6.4% welder's pneumoconiosis patents. 30.8% patients failed to reach the original stage when 39 welder's pneumoconiosis followed up chest radiograph.</p><p><b>CONCLUSION</b>Changes in welder's lung caused by welding fume were not only siderosis, but also interstitial fibrosis.</p>

Atypical Presentation of Cerebral Superficial Siderosis Mimicking Recurrent Sciatic Neuralgia

No abstract available.

Cerebral Amyloid Angiopathy: Emerging Concepts / 대한뇌졸중학회지

Cerebral amyloid angiopathy (CAA) involves cerebrovascular amyloid deposition and is classified into several types according to the amyloid protein involved. Of these, sporadic amyloid beta-protein (Abeta)-type CAA is most commonly found in older individuals and in patients with Alzheimer's disease (AD). Cerebrovascular Abeta deposits accompany functional and pathological changes in cerebral blood vessels (CAA-associated vasculopathies). CAA-associated vasculopathies lead to development of hemorrhagic lesions [lobar intracerebral macrohemorrhage, cortical microhemorrhage, and cortical superficial siderosis (cSS)/focal convexity subarachnoid hemorrhage (SAH)], ischemic lesions (cortical infarction and ischemic changes of the white matter), and encephalopathies that include subacute leukoencephalopathy caused by CAA-associated inflammation/angiitis. Thus, CAA is related to dementia, stroke, and encephalopathies. Recent advances in diagnostic procedures, particularly neuroimaging, have enabled us to establish a clinical diagnosis of CAA without brain biopsies. Sensitive magnetic resonance imaging (MRI) methods, such as gradient-echo T2* imaging and susceptibility-weighted imaging, are useful for detecting cortical microhemorrhages and cSS. Amyloid imaging with amyloid-binding positron emission tomography (PET) ligands, such as Pittsburgh Compound B, can detect CAA, although they cannot discriminate vascular from parenchymal amyloid deposits. In addition, cerebrospinal fluid markers may be useful, including levels of Abeta40 for CAA and anti-Abeta antibody for CAA-related inflammation. Moreover, cSS is closely associated with transient focal neurological episodes (TFNE). CAA-related inflammation/angiitis shares pathophysiology with amyloid-related imaging abnormalities (ARIA) induced by Abeta immunotherapies in AD patients. This article reviews CAA and CAA-related disorders with respect to their epidemiology, pathology, pathophysiology, clinical features, biomarkers, diagnosis, treatment, risk factors, and future perspectives.

A Case of Ocular Siderosis with Cataract and Delayed-Onset Secondary Glaucoma

PURPOSE: We report a rare case of ocular siderosis with delayed-onset secondary glaucoma occurring 10 years after cataract and iron foreign body removal. CASE SUMMARY: A 47-year-old male who suffered an ocular injury with an iron material to his left eye 3 years prior to his initial visit was treated in our clinic for a cataract with siderosis lentis. Ten years after he underwent cataract surgery he developed secondary open-angle glaucoma. Trabecular block taken during trabeculectomy showed no iron material, however, a fibrosclerotic change was observed. CONCLUSIONS: In patients with a history of traumatic siderosis lentis, a potential risk of siderosis remains for a long period.

Welders' siderosis: a retrospective cohort study on welder's pneumoconiosis patients with small round opacities on chest radiograph / 中华劳动卫生职业病杂志

<p><b>OBJECTIVE</b>To study the radiographycal changes and prognosis of welders' pneumoconiosis patients diagnosed within the last few years.</p><p><b>METHODS</b>Occupational hygienics data (including years on welding work, welding materials exposed to and work environment), symptoms, lung function test results and follow-up data of 136 welders' pneumoconiosis patients were collected retrospectively. The follow-up lasted up till June 30, 2014, with follow-up interval being one year. Chest radiographs were read/reviewed together by 3 senior experts experienced in pneumoconiosis diagnosing and shape and size of small opacity, overall profusion, affected lung zones and pneumoconiosis stages were all logged.</p><p><b>RESULTS</b>Of all 136 pneumoconiosis cases, five were of stage II and 131 were stage I, and they had been engaged in welding work for 9.2±6.34 years. All patients were present with slight symptoms including coughing, chest distress and dyspnea, while their lung function remained normal. Small round opacities were found on chest radiographs of 88.9% of the 136 cases. Years of follow-up on 131 cases of stage I Welder's pneumoconiosis showed that irregular small opacities remained fundamentally unchanged, while small round opacities tended to gradually lessen instead of coalescence. Two years after primary diagnosing, the total affected lung zones of follow-up objects were noticed to start lessening, and reduced by 0.02 per patient among the 48 follow-up subjects. Of the 48 cases followed up up to six years after primary diagnosing, the affected lung zone number was reduced by 0.54 per patient while 4 cases (8%) went down to below stage I. As of the 36 patients followed up 10 years after after primary diagnosis, the number of affected lung areas decreased by 1.14, and 22% (8 cases) appeared less than stage I on chest radiograph.</p><p><b>CONCLUSION</b>It seemed that the so-called welders' pneumoconiosis featuring small round opacities tended to get improved over time, which suggested the diagnosis of siderosis.</p>

Optimal method for early detection of cardiac disorders in thalassemia major patients: magnetic resonance imaging or echocardiography?

BACKGROUND: Heart failure resulting from myocardial iron deposition is the most important cause of death in beta-thalassemia major (TM) patients. Cardiac T2*magnetic resonance imaging (MRI), echocardiography, and serum ferritin level serve as diagnostic methods for detecting myocardial iron overload. In this study, we aimed to evaluate the relationship between the above-mentioned methods. METHODS: T2*MRI and echocardiographic measurement of left ventricular (LV) systolic and diastolic function were performed in 63 patients. Serum ferritin level was measured. The relationships between all assessments were evaluated. RESULTS: There were 40 women and 23 men with a mean age of 23.7+/-5.1 years (range, 15-35 years). There was no statistically significant correlation between serum ferritin level and LV systolic and diastolic function (P=0.994 and P=0.475, respectively). T2*MRI results had a significant correlation with ferritin level; 63.6% of patients with serum ferritin level >2,000 ng/mL had abnormal cardiac MRI, while none of the patients with ferritin level <1,000 ng/mL had abnormal cardiac MRI (P=0.001). There was no significant correlation between MRI findings and LV systolic function (P=1.00). However, we detected a significant difference between LV diastolic function and cardiac siderosis (P=0.03) CONCLUSION: MRI findings are a good predictor of future cardiac dysfunction, even in asymptomatic TM patients; however, diastolic dysfunction may happen prior to cardiac siderosis in some patients, and echocardiography is able to diagnose this diastolic dysfunction while T2*MRI shows normal findings.

Pneumoconioses.

Occupational lung diseases are caused or made worse by exposure to harmful substances in the work-place. “Pneumoconiosis” is the term used for the diseases associated with inhalation of mineral dusts. While many of these broadspectrum substances may be encountered in the general environment, many occur in the work-place for greater amounts as a result of industrial processes; therefore, a range of lung reactions may occur as a result of work-place exposure. Physicians in metropolitan cities are likely to encounter pneumoconiosis for two reasons: (i) patients coming to seek medical help from geographic areas where pneumoconiosis is common, and (ii) pneumoconiosis caused by unregulated small-scale industries that are housed in poorly ventilated sheds within the city. A sound knowledge about the various pneumoconioses and a high index of suspicion are necessary in order to make a diagnosis. Identifying the disease is important not only for treatment of the individual case but also to recognise and prevent similar disease in co-workers.

Histopathologically proven siderotic cataract with disintegrated intralenticular foreign body.

Cataract formation may be an indicator of early siderosis and has been associated with intralenticular foreign bodies. We report a unique case of histopathologically proven lens siderosis in a young man with a preceding history of trauma but no signs of retained intraocular foreign body. He presented with a total white cataract with brownish deposits on anterior capsule and underwent cataract surgery for same followed by histopathological staining of anterior capsule for iron deposits. This case illustrates the importance of close monitoring of patients with history of trauma or previous penetrating injury to the eye, albeit no intraocular foreign body, as they might develop ocular siderosis at a later stage.

Lobar Intracerebral Hemorrhage Associated With Cortical Superficial Siderosis

Superficial siderosis (SS) is a rare disorder characterized by deposition of hemosiderin in the leptomeninges and subpial layer of the central nervous system. Recently SS suggested a subtype of cerebral amyloid angiopathy which is an important cause of lobar intracerebral hemorrhage (ICH). We present a patient with cortical SS had seizure and cognitive dysfunction as symptom and lobar ICH in the existed area of cortical SS 4 years later. This case suggested cortical SS could be a warning sign of lobar ICH.