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.

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.

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.

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.

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.

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.

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.

Superficial Siderosis Combined With Spinal Cerebrospinal Fluid Collection

No abstract available.

A Case of Spinal Cord Ependymoma resulting in Superficial Siderosis

A 63-year-old woman developed left leg weakness, bilateral sensorineural hearing loss and headache. Brain MRI showed a hypointense rim on the surfaces of the cerebellum, brainstem, and cerebral cortex indicating superficial siderosis. Further examination showed an intramedullary ependymoma at the level of T12-L2. We report a patient with a spinal cord ependymoma resulting in superficial siderosis of the central nervous system.