Amyloid spell – A close mimic of transient ischemic attack!

Transient focal neurological episodes, also called amyloid spells occur as recurrent, transient episodes of spreading paresthesia seen in 14% of cerebral amyloid angiopathy (CAA) patients. An 81-year-old gentleman with coronary artery disease and a left ventricular clot was on anticoagulant treatment. He presented with three episodes of tingling in the left fingers spreading to the left arm and left leg, each lasting for 10 min. Magnetic resonance imaging of the brain with susceptibility imaging showed convexity hemorrhage, and curvilinear blooming in sulcal spaces of the right cerebral convexity and left precuneus. Warfarin was stopped. He was treated with clobazam, aspirin, and atorvastatin. He improved, so was discharged after 2 days. Amyloid spells can be confused with transient ischemic attack (TIA) or its mimics and the treatment given for TIA can lead to intracranial hemorrhage in CAA patients. Radiological features aid in the diagnosis of CAA and antiplatelets need to be administered cautiously in patients with suspected TIA.

Clinical profile, demographic distribution, and outcomes of ocular siderosis: Electronic medical record–driven big data analytics from an eye care network in India

Purpose: To describe the demographics, clinical profile, and outcomes of ocular siderosis in patients presenting to a multi?tier ophthalmology hospital network in India. Methods: This cross?sectional and hospital?based study included 3,082,727 new patients who presented between August 2010 and December 2021. Patients with a clinical diagnosis of ocular siderosis in at least one eye were included. Results: Overall, 58 eyes of 57 patients (0.002%) were diagnosed with ocular siderosis. The majority were men (96.49%) and had unilateral (98.25%) affliction. The most common age group at presentation was during the third decade of life with 24 patients (42.11%). A clear history of ocular trauma was documented in 47 patients (81.03%). Major clinical signs included corneal pigment deposition in nearly half of the eyes (27/58 eyes, 46.55%), corneal scar (20/58 eyes, 34.48%), cataract (22/58 eyes, 37.93%) and retinal detachment (11/58 eyes, 18.96%). The intraocular foreign body (IOFB) was anatomically localized in a majority of the eyes (i.e., 45/58 eyes, 77.59%). The most common location of the IOFB was in the posterior segment (22/58 eyes, 37.93%). The eyes that underwent a vitreoretinal surgery with removal of IOFB had a slightly better BCVA (1.0 ± 1.01) when compared to eyes with non?removal of IOFB (1.58 ± 1.00). Conclusion: Ocular siderosis is a rare sight?threatening entity, with half of the affected eyes exhibiting severe visual impairment. Majority of the eyes in ocular siderosis will have a detectable IOFB. Surgical removal of IOFB may lead to a better visual gain when compared to non?removal.

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.

The clinical features and etiology analysis of superficial siderosis of the central nervous system / 中华神经科杂志

Objective:To summarize the clinical manifestation and imaging of superficial siderosis of the central nervous system and explore the potential etiology.Methods:The clinical and imaging data of 7 patients diagnosed as superficial siderosis of the central nervous system in Peking Union Medical College Hospital from May 2013 to November 2019 were retrospectively reviewed. The etiology and follow-up prognosis through phone call were analyzed.Results:There were 7 patients included (3 male and 4 female) with an average age of 53 years (41-58 years). The cardinal manifestations were sensorineural deafness (all 7 cases), cerebellar ataxia (all 7 cases) and pyramidal signs (all 7 cases). Dizziness (6 cases), bladder disturbance (5 cases), headache (3 cases), double vision (2 cases) and congnitive impairment (1 case) could also happen. Magnetic resonance imaging showed symmetrical well-defined curvilinear homogeneous low signal on T 2 or blood-sensitive sequences (T 2* gradient echo or susceptibility-weighted imaging) over the superficial surface of cerebellar, brain stem, and spinal cord or cranio-cervical junction. All the 7 patients showed cerebellar atrophy especially the upper vermis. The potential causes included trauma history in 3 cases, intraspinal fluid-filled collection which indicated dural defect or duropathologies in 3 cases, intraspinal mass in 1 case and vertebral and disc degeneration in all 7 patients. The 5 patients who successsfully got follow-up showed exacerbation of variable degree. Conclusions:Classical superficial siderosis of the central nervous system is a rare disease with cardinal manifestation of progressive ataxia, sensorineural deafness and pyramidal signs. T 2WI of magnetic resonance imaging showing low signal over the superficial surface of cerebellar, brain stem, and spinal cord could indicate the diagnosis, and blood-sensitive sequences such as T 2* gradient echo or susceptibility-weighted imaging were more sensitive. Duropathologies or dural defect may be the most probable causes of the disease and should be examined and treated carefully.

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.

MRI findings of superficial siderosis of the central nervous system / 实用放射学杂志

Objective To study the MRI findings of superficial siderosis of the central nervous system(SSCNS)and to improve the understanding of SSCNS imaging features.Methods MRI images of 21 cases were retrospectively reviewed.MRI plain scan (T1WI,T2WI)and diffusion weighted imaging(DWI)were performed in all patients;T2* weighted angiography(SWAN)scan was performed in 8 patients.Results On T1WI of 21 cases,only 4 cases showed infratentorial superficial siderosis which appeared as linear hypointense signal in sulci cerebelli and around brainstem.On T2WI of 21 cases,18 cases presented with linear hypointense signal on cerebral surface.On DWI of 21 cases,20 cases showed linear hypointense signal on cerebral surface,and the other one case showing negative signal on DWI presented hypointense signal on SWAN.On SWAN of 8 cases,more lesions were found in more regions.Conclusion SSCNS has characteristic findings on MRI.SWAN is sensitive to superficial siderosis.MRI is a reliable method for the assessment of SSCNS.

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.

MRI evaluation of superficial siderosis of the central nervous system after traumatic subarachnoid hemorrhage / 实用放射学杂志

Objective To assess the diagnostic value of 3D-enhanced T2 ? weighted angiography (ESWAN )in evaluating superficial siderosis of the central nervous system (SS-CNS)after traumatic subarachnoid hemorrhage (tSAH).Methods ESWAN and GRE T2 ? WI sequence were performed on 30 patients with tSAH,detection rate and the number of distribution areas of SS-CNS were compared between the two sequences.A McNemar’s test and Wilcoxon signed-rank test were used to analyze the differences between T2 ? WI and ESWAN images sequences. Results In the tSAH group,29 of 30 patients (96.7%)showed SS-CNS on ESWAN images,the total number of SS-CNS regions was 134.Identified SS-CNS positive rate respectively was 95.8% (23/24)on ESWAN images and 66.7% (1 6/24)on T2 ? WI in 24 patients simultaneously perform ESWAN and T2 ? WI sequences.A McNemar ’s test showed that there was significant difference between the positive rates of two sequences in detecting the SS-CNS (χ2 =7.0,P <0.05).The number of SS-CNS regions on ESWAN images and T2 ? WI was 106 and 34 respectively.The Wilcoxon signed rank test showed that the number of SS-CNS regions difference between two sequences was significant (Z =-4.225,P <0.01).Conclusion Various degress of SS-CNS are detected in a majority of tSAH atients.ESWAN sequence is a reliable and efficient method for assessment of SS-CNS.

Symptomatic Pulmonary Siderosis A case report

Pulmonary siderosis or Welder’s lung is a rare occupational lung disease which occurs due to long term inhalation of iron compounds. It is most commonly seen in arc-welders. Most of the patients are asymptomatic with mild or no functional impairment. We report a case of pulmonary siderosis with symptomatic respiratory disease and restrictive pattern in pulmonary function test.

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.

Interstitial Lung Disease due to Siderosis in a Lathe Machine Worker.

Since its first description in 1936, siderosis of lung has been considered a benign pneumoconiosis due to absence of significant clinical symptoms or respiratory impairment. Subsequently, authors have questioned the nonfibrogenic property of iron. However, siderosis causing interstitial lung disease with usual interstitial pneumonia (UIP) pattern has not been described in the past. We report a case of UIP on high resolution computed tomography, proven to be siderosis on transbronchial lung biopsy in a lathe machine worker.

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.

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.

Superficial Siderosis in Central Nervous System: A Case Report and Literature Review / 대한신경손상학회지

Superficial siderosis (SS) in central nervous system is a rare, slowly progressive disease and usually misdiagnosed or diagnosed too late when the patient is chronically devastated. A 55-year-old man with deafness and gait disturbance for ten years was referred from otorhinologist for evaluation of brain. Magnetic resonance image (MRI) showed symmetric hypointense rim partially delineated the bilateral hemisphere on gradient-recalled-echo T2-weighted image, and it was diagnosed as hemosiderin deposition in subarachnoid and subpial meningeal layer. The correct diagnosis of cerebral superficial siderosis can be achieved by careful neurological examination and MRI because computed tomography findings and symptoms are ambiguous. Serial follow-up of imaging study and education for patient are necessary to prevent progression of SS.

Superficial Siderosis in Central Nervous System: A Case Report and Literature Review / 대한신경손상학회지

Superficial siderosis (SS) in central nervous system is a rare, slowly progressive disease and usually misdiagnosed or diagnosed too late when the patient is chronically devastated. A 55-year-old man with deafness and gait disturbance for ten years was referred from otorhinologist for evaluation of brain. Magnetic resonance image (MRI) showed symmetric hypointense rim partially delineated the bilateral hemisphere on gradient-recalled-echo T2-weighted image, and it was diagnosed as hemosiderin deposition in subarachnoid and subpial meningeal layer. The correct diagnosis of cerebral superficial siderosis can be achieved by careful neurological examination and MRI because computed tomography findings and symptoms are ambiguous. Serial follow-up of imaging study and education for patient are necessary to prevent progression of SS.

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.

Evaluation of Cardiac Iron Load by Cardiac Magnetic Resonance in Thalassemia.

Objective: To quantify myocardial iron stores by Cardiac Magnetic Resonance (CMR) . Design: Prospective cohort study. Setting: Thalassemia center in a teaching hospital. Participants: 60 transfusion dependant thalassemia major patients and 10 controls during 2008-2009. Methods: MRI T2* for cardiac iron load and cardiac functions was performed on a 1.5 Tesla Siemens Sonata machine using the thalassemia tools software. Ejection fraction (EF) was measured using standard CMR sequence and EF <56% considered as cardiac dysfunction. Quantification of iron deposition was categorized as T2* <10 milliseconds (ms) as high risk, 10-20 ms as intermediate risk and >20 ms as low risk. Simultaneous liver iron T2* values were categorized into normal i.e. >6.3 ms, mild iron overload 6.3 - 2.7 ms , moderate iron overload 2.7- 1.4 ms and severe iron overload <1.4 ms. Pretransfusion serum ferritin levels were simultaneously determined. Data was analyzed by paired and unpaired t test of mean. Results: Of 60 patients, 50% had no cardiac siderosis; 33.3% had mild to moderate and while 16.7% had severe cardiac siderosis . In contrast, only 8.3% had normal liver iron values, 55.7% had mild to moderate and 36% had severe iron stores. The mean serum ferritin of all 60 cases was 3528.6 ± 1958.6 ng/mL. There was a statistically significant difference in the mean cardiac T2* of patients (23.45 ± 13.4 ms) as compared to controls (32.67 ± 2.68 ms) (P<0.01). Conclusions: Thalassemia patients had significantly higher cardiac iron stores as compared to controls. Serum ferritin and liver iron values did not correlate with cardiac iron values. Three of 10 patients <10 years showed evidence of myocardial siderosis.