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.
Aged ; Biomarkers ; Cerebral Small Vessel Diseases ; Epidemiology ; Humans ; Leukoaraiosis ; Mass Screening ; Mortality ; Neuroimaging ; Risk Factors ; Siderosis ; Stroke, Lacunar ; White Matter

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.
Adult ; Central Nervous System Diseases ; China ; Ependymoma ; Humans ; Magnetic Resonance Imaging ; Male ; Siderosis ; Spinal Cord

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.
Alzheimer Disease* ; Amyloid ; Apolipoproteins ; Apolipoproteins E ; Atrophy ; Autopsy ; Brain ; Cerebral Amyloid Angiopathy* ; Genotype ; Homozygote ; Humans ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Pathology ; Plaque, Amyloid ; Prevalence ; Siderosis

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.
Aneurysm ; Brain ; Central Nervous System ; Cerebellar Ataxia ; Cranial Nerves ; Dizziness ; Hearing Loss, Sensorineural ; Hemorrhage ; Hemosiderin ; Humans ; Iron ; Magnetic Resonance Imaging ; Rare Diseases ; Siderosis ; Spinal Cord ; Spinal Cord Diseases ; Subarachnoid Space ; Vascular Malformations ; Vertigo

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.
Ataxia ; Central Nervous System ; Cerebellar Diseases ; Hearing Loss ; Hemorrhage ; Hemosiderin ; Intracranial Hypotension ; Siderosis ; Spinal Cord Diseases ; Subarachnoid Space ; Subdural Effusion ; Supine Position

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.
Central Nervous System ; Cerebellar Ataxia ; Craniopharyngioma ; Hearing Loss, Sensorineural ; Hemorrhage ; Humans ; Magnetic Resonance Imaging ; Pyramidal Tracts ; Siderosis ; Subarachnoid Space ; Third Ventricle

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.
Ataxia ; Central Nervous System* ; Hearing Loss ; Hemosiderin ; Humans ; Neurologic Examination ; Rare Diseases ; Siderosis* ; Spine*

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.
Cataract* ; Foreign Bodies ; Glaucoma* ; Glaucoma, Open-Angle ; Humans ; Iron ; Male ; Middle Aged ; Siderosis* ; Trabeculectomy

OBJECTIVETo study the character of welder's pneumoconiosis on CT, pathology, and lung function.

METHODSTo 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.

RESULTSAverage 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.

CONCLUSIONChanges in welder's lung caused by welding fume were not only siderosis, but also interstitial fibrosis.

Adult ; Dust ; Fibrosis ; Follow-Up Studies ; Glass ; Humans ; Lung ; pathology ; Middle Aged ; Pneumoconiosis ; physiopathology ; Radiography, Thoracic ; Siderosis ; physiopathology ; Silicosis ; diagnosis ; physiopathology ; Welding

Ataxia ; Carbamazepine ; analogs & derivatives ; therapeutic use ; Carcinoma ; Central Nervous System ; pathology ; Diagnosis, Differential ; Female ; Gait ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Nasopharyngeal Neoplasms ; complications ; radiotherapy ; Nervous System Diseases ; complications ; diagnosis ; Radiotherapy ; adverse effects ; Seizures ; complications ; diagnosis ; Siderosis