Cortical Superficial Siderosis and Risk of Bleeding after Thrombolysis for Ischemic Stroke.

BACKGROUND: Cortical superficial siderosis (CSS) is a neuroimaging marker of cerebral amyloid angiopathy and has been associated with a high risk for early subsequent major intracranial hemorrhage (ICH). Therefore, many experts recommend withholding of antithrombotic medication to patients with CSS. In this study, we sought to investigate the prevalence of CSS and the associated risk of ICH in the setting of intravenous thrombolysis (IVT) for ischemic stroke. METHODS: We retrospectively searched the medical documentation system of our primary and tertiary care university clinic for all patients with ischemic stroke that received IVT from 2009 to December 2014. All available imaging data were reviewed in a standardized manner and blinded to any clinical data for the presence of CSS and ICH. CSS was defined as linear signal loss along the cerebral cortex on gradient echo T2*-weighted sequences. A stroke neurologist, who was blinded to the neuroimaging data, extracted the corresponding clinical data including follow-up information. RESULTS: We identified 298 patients that received IVT and had undergone brain MRI (mean age 67.6 ± 12.6 years, 59.4% male). Cerebral MRI was performed in 116 patients (38.9%) before and in 182 patients (61.1%) after IVT (median time from stroke symptom onset to MRI: 1 day; range 0-7 days). Only 3 patients (2 females and 1 male aged 90, 76 and 73 years, respectively) had CSS (1%). All of them had a middle cerebral artery (MCA) stroke with a corresponding vessel occlusion. The 76-year-old female patient had extensive CSS and numerous cerebral microbleeds and received another IVT treatment for recurrent MCA stroke 8 months after the first event. After both IVTs, she had clinically asymptomatic small ICH outside the ischemic infarct and distant from CSS. The 2 other patients had only mild to moderate CSS and did not experience any ICH on postthrombolytic imaging. CONCLUSIONS: The prevalence of CSS in a clinical cohort of stroke patients that received IVT was low and thus does not appear to pose a substantial risk for symptomatic ICH although this may occur in individual patients. However, such analysis also needs to be extended to the very old stroke patients in whom IVT is increasingly used.

Deep Brain Stimulation in a Patient with Parkinson's Disease and Cortical Superficial Siderosis.

Cortical superficial siderosis (cSS) is a rare condition that is regarded as a potential magnetic resonance marker of cerebral amyloid angiopathy (CAA). We describe the case of a 68-year-old man with cSS and Parkinson's disease (PD) who subsequently exhibited incidental microhemorrhages, which were only detected on magnetic resonance imaging (MRI), at one week after deep brain stimulation (DBS) surgery. cSS is now considered to be a significant risk factor for CAA and future bleeding. Therefore, because DBS surgery is invasive and may increase the risk of intracerebral hemorrhage, the procedure should be performed carefully when managing patients with PD and CAA.

Superficial siderosis of the CNS.

OBJECTIVE: The objective of this article is to illustrate and describe the cause, physiopathology, natural history, and clinical and imaging presentations of superficial siderosis as a cause of progressive cerebellar ataxia with sensorineural hearing loss and to discuss the therapeutic rationale. CONCLUSION: Superficial siderosis resulting in subpial deposition of hemosiderin along the surface of the cerebellum, brain, spinal cord, and cranial and peripheral nerves is a known cause of progressive cerebellar ataxia and sensorineural hearing loss. MRI evaluation of the entire neuraxis plays a key role in establishing the diagnosis and identifying the source of chronic bleeding. Treatment of the source of bleeding can halt the otherwise deteriorating clinical course of disease.

[The role of iron in the interaction between host and pathogen]. / Papel del hierro en la interacción entre el huésped y el patógeno.

Iron is essential for both pathogenic microbes and their host. Iron status may influence the occurrence and outcome of infections. For many microorganisms, iron is essential for growth, survival, and synthesis of virulence factors. However, because circulating iron is mostly transported bound to proteins and the level of free serum iron is therefore very low, some pathogens have developed complex systems to acquire iron efficiently. Understanding these systems is essential in the design of pharmaceutical agents and vaccines targeting pathogens with iron-linked virulence. In this review, we examine current data on the role of iron in the struggle between host and pathogen to regulate levels of this essential element. We hope that, in the near future, treatments aimed at reducing iron overload will improve the response to current therapies, and help control infection.

[Ocular siderosis]. / Siderosos bulbi--caz clinic.

We show a clinical case of siderosis bulbi with absolute secondary glaucoma. The initial diagnosis was primary open angle glaucoma in both eyes, and the patient was treated with eye drops for 4 years. After reassessment of anamnesis, clinical and paraclinical investigations we find that one eye is normal and the real diagnosis in the involved eye.

[Superficial Siderosis].

Superficial siderosis (SS) is a rare, neurodegenerative disease that results from toxic accumulation of hemosiderin on the surface of the brain and spinal cord. The Japanese guidelines for diagnosis and classification of SS have been classified into three categories, according to the distribution of iron deposition and the clinical symptoms, as follows: "classical" type, "localized" type and "atypical" type. In "classical" type SS, patients typically present with slowly progressive and irreversible cerebellar ataxia, sensorineural hearing loss, and/or myelopathy, due to involvement of the acoustic nerve, cerebellum, and spinal cord. T2-weighted imaging (WI) or T2* WI demonstrates characteristic linear low intensity signals along the surface of the brain and spinal cord. However, the natural history and clinical evolution of SS are poorly understood. Furthermore identification and resolution of the bleeding source do not elicit prompt clinical recovery or radiological reversal of SS in most cases. Pharmacological treatments for SS primarily include strategies that produce iron chelation. For example, deferiprone is the only treatment that crosses the blood-brain barrier and binds hemosiderin in the central nervous system.

Superficial siderosis: associations and therapeutic implications.

Superficial siderosis of the central nervous system results from hemosiderin deposition in the subpial layers of the brain and spinal cord. A clinical history of subarachnoid hemorrhage is often absent. Patients present with slowly progressive gait ataxia and sensorineural hearing impairment. A history of prior intradural surgery or trauma is common. With widespread use of magnetic resonance imaging, presymptomatic cases are being diagnosed and it is difficult to be certain about the true incidence of this disorder. Despite extensive investigations, the cause of bleeding is often not apparent. An intraspinal fluid-filled collection is a common accompaniment and may be the likely bleeding source. An early diagnosis and prompt intervention directed at removal of the bleeding source may prevent progression. This review discusses the role of multimodality imaging in evaluation of superficial siderosis and the therapeutic implications of identified associations.

Welder's lung associated with pneumothorax.

A 23-year-old male working in welding for 8 years was admitted to the hospital with chest pain. A large right pneumothorax with complete lung collapse was seen radiographically and a tube thoracostomy performed. At high-resolution computed tomography, multiple bilateral well-marginated nodules with surrounding ground-glass opacity were seen. Welder's lung was considered in this patient because of his work in welding for 8 years with iron dust exposure, multiple nodules with ground-glass opacity were seen on HRCT, and serum ferritin and ferritin levels in bronchoalveolar lavage fluid were elevated. The granules in alveolar macrophages obtained from parenchymal biopsy and bronchoalveolar lavage stained strongly with iron dyes. Following avoiding exposure to welding fumes, the lung lesions disappeared without additional therapeutic interventions after a 3-week period. Welder's lung with associated lung lesions has been described, although complicating pneumothorax is unique to the case presented herein.

Siderosis superficial del sistema nervioso central en una paciente con paraganglioma sacro asintomático como fuente de sangrado crónico / Superficial siderosis of the central nervous system in a patient with asymptomatic sacral paraganglioma as source of chronic bleeding

INTRODUCCIÓN: La siderosis superficial del sistema nervioso central es una patología poco frecuente secundaria al sangrado crónico en el líquido cefalorraquídeo. Los tumores medulares son causa poco habitual de siderosis superficial, y el ependimoma es la etiología más común. CASO CLÍNICO: Mujer con pérdida auditiva neurosensorial y ataxia cerebelosa, diagnosticada de siderosis superficial en la resonancia magnética cerebral. No tenía antecedentes de dolor raquídeo axial, dolor radicular ni incontinencia esfinteriana. En la resonancia magnética de la columna se encontró una lesión intradural en S1. No se observaron signos de hemorragia intratumoral en las secuencias de resonancia magnética en eco de gradiente. En la cirugía, se apreció una masa blanda intradural con signos de sangrado crónico que se resecó. Basado en el examen microscópico e inmunohistoquímico de la muestra, se alcanzó el diagnóstico de paraganglioma de grado I de la Organización Mundial de la Salud. CONCLUSIONES. Dado que el único tratamiento probado capaz de prevenir un mayor deterioro por la siderosis superficial es detener el sangrado crónico en el espacio subaracnoideo, es importante establecer un diagnóstico temprano de la fuente de sangrado. Los casos no justificados de siderosis superficial del sistema nervioso central deben incluir una resonancia magnética de la columna rutinaria para descartar el sangrado de un tumor medular, incluso en pacientes asintomáticos. Debido a la gravedad del deterioro potencial causado por la siderosis superficial, cualquier lesión tumoral observada en una resonancia magnética del raquis, incluso sin presentar signos de sangrado, debería ser objeto de indicación quirúrgica

INTRODUCTION: Superficial siderosis of the central nervous system is an infrequent pathology secondary to chronic bleeding into the cerebrospinal fluid. Spinal tumors are infrequent cause of superficial siderosis being ependymoma the most common etiology. CASE REPORT: We report the case of a woman with sensorineural hearing loss and cerebellar ataxia, diagnosed of superficial siderosis on brain MRI. She had no previous history of axial back pain or radicular leg pain or bowel or bladder incontinence. On spine MRI an intradural lesion was found at the S1 level. No signs of intratumoral hemorrhage were observed on MRI gradient-echo images. At surgery, an intradural soft mass with signs of chronic bleeding was completely resected. Based on microscopic examination and immunohistochemistry of the specimen, a diagnosis of paraganglioma World Health Organization grade I was made. CONCLUSIONS: Since the only proven treatment able to prevent further deterioration from superficial siderosis is to stop chronic bleeding into subarachnoid space, is of paramount importance to establish an early diagnosis of the source of bleeding. Cases of unexplained superficial siderosis of central nervous system should include routine spinal MRI to rule out bleeding of spinal tumor even in asymptomatic patients. Due to severity of potential deterioration caused by superficial siderosis, any tumoral lesion observed on spinal MRI even without documented sings of bleeding should be considered for resection

Diagnostic and therapeutic challenges in superficial CNS siderosis.

INTRODUCTION: Superficial CNS siderosis was previously almost unknown but is now diagnosed with increasing frequency owing to magnetic resonance imaging. Patients may present with sensory deafness, gait ataxia, various sensorimotor signs and, eventually, cognitive decline. They typically have a history of traumatic brain or spinal cord injury or previous neurosurgery, or may harbour congenital malformations. However, knowledge about treatment outcomes remains scarce. METHODS: We present a series of nine consecutive patients from a large tertiary neuroscience centre in order to highlight the challenges related to the diagnosis and treatment of superficial siderosis. RESULTS: A potential bleeding aetiology was identified in all patients, but removal of the offending bleeding source was achieved only in three (33%). Symptom progression was halted in just one patient (11%), which suggests that neurodegeneration due to haemosiderin-associated iron toxicity becomes irreversible with time. CONCLUSION: Surgical therapy in superficial CNS siderosis is rarely achieved. We suggest that prospective, large-scale multicentre studies are needed to search for non-surgical therapies that reverse (or prevent) ongoing neurotoxicity due to accumulating iron toxicity. FUNDING: not relevant. TRIAL REGISTRATION: not relevant.

[Ocular siderosis: its management. Apropos of a case]. / La sidérose oculaire: attitude pratique. A propos d'un cas.

Siderosis bulbi, a rare ocular affection, occurs when an iron-containing foreign body is retained within the eye. The authors report the management of an unrecognized foreign body in one case.

[Treatment of iron overload with combination of recombinant human erythropoietin and phlebotomy].

A patient of Coombs negative autoimmune hemolytic anemia was massively transfused of 162 units concentrated red blood cells in 3 months and developed iron overload disease which was confirmed by liver biopsy. Hemolysis was successfully treated with high-dose methyl-prednisolone therapy and splenectomy. To treat iron overload, we administered recombinant human erythropoietin (Epo) in combination with phlebotomy. Total iron removed for 5 months was about 4 g. Thus, combination of Epo and phlebotomy was effective for the treatment of iron overload disease. Furthermore, we compared a degree of clinical effect of subcutaneous administration of Epo with that of intravenous administration in the clinical course and found the former more effective.

[Diagnosis and treatment of superficial siderosis].

Superficial siderosis (SS) is a rare condition in which hemosiderin is deposited on the pial surface of the brain and/or spinal cord. Hemosiderin deposition is the consequence of recurrent or persistent hemorrhage in the subarachnoid space. There are two types of SS. In "classical"-type SS, hypointense MRI signals are observed in the brainstem and cerebellum with diffuse and symmetrical margins. Causes of hemorrhage in the "classical" type include tumor, vascular abnormality, injury, and dural defect. The source of hemorrhage is not apparent in approximately 50% of patients despite extensive examination. In "localized"-type SS, hypointense MRI signals are localized in the cerebral cortex. The most common causes of hemorrhage in the "localized" type are cerebral amyloid angiopathy and/or Alzheimer's disease. Patients with SS usually present with slowly progressive and irreversible cerebellar ataxia, sensorineural hearing loss, and/or myelopathy due to involvement of the acoustic nerve, cerebellum, and spinal cord. T2-weighted imaging (WI) or T2* WI demonstrates characteristic linear low-intensity signals along the surface of the brain and spinal cord. Treatment of SS involves identification and surgical correction of the bleeding source. Deferiprone, which is a lipid-soluble iron chelator that can penetrate the blood-brain barrier, is reportedly effective at improving the clinical symptoms and deposition of hemosiderin. It is thus a hopeful treatment option for SS.

Meningosiderosis como forma de presentación de un melanoma en estadio IV / Superficial siderosis of the central nervous system as a presentation of stage iv melanoma

No disponible

CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: two case reports.

We report two cases of superficial siderosis (SS) of the central nervous system (CNS), which is caused by chronic haemorrhaging into the subarachnoid space with haemosiderin deposition in the superficial portion of the CNS. Patient 1 had fluid collection in the spinal canal, which was reported as the source of the chronic bleeding. Patient 2 was bleeding from thickened dura at the level of the sacral vertebrae. Both of the patients had xanthochromic cerebrospinal fluid. We surgically repaired the sources of bleeding. Subsequently the cerebrospinal fluid (CSF) cleared and their symptoms were not aggravated for about 1 year. We measured several CSF markers of SS before and after surgery. Total tau protein (CSF-t-tau), phosphorylated tau protein (CSF-p-tau), iron (CSF-iron) and ferritin (CSF-ferritin) in the CSF were highly elevated at diagnosis. After surgery, the levels of CSF-t-tau and CSF-p-tau were markedly reduced while CSF-iron and CSF-ferritin had not decreased. It is suggested that CSF-t-tau and CSF-p-tau reflected the neural damage in SS and were useful to evaluate the effectiveness of SS therapies.