Recurrent Cerebral Infarctions Research Articles

Roger’s syndrome presenting with stroke

Rogers syndrome (Thiamine responsive megaloblastic anaemia-TRMA) occurs due to defect in the SLC19A2 gene. SLC19A2 and SLC19A3 genes encode THTR1 and 2 respectively, which are thiamine transporter proteins. The SLC19A2 gene is expressed in the inner ear cells, β-islet cells, and hematopoietic stem cells; consequently, the typical clinical trial of TRMA is diabetes, TRMA, and sensorineural hearing loss. This syndrome, eponymously called Roger’s syndrome is rare. Mode of inheritance of TRMA is autosomal recessive. Clinical presentation as recurrent stroke is extremely rare. We present a case of a five-year-old boy who had recurrent large artery territory cerebral infarcts, with no other identifiable underlying cause of stroke. During current admission, no underlying etiology could be identified for cerebral infarct. On workup, a preliminary diagnosis of TRMA was made and thiamine supplementation was instituted. Gene analysis confirmed the diagnosis. The child was a product of non-consanguineous marriage. There was history of early childhood demise of two older siblings within five years of age due to diabetic ketoacidosis (DKA). TRMA is a rare autosomal recessive syndrome that manifests as a typical triad with diabetes, hearing loss and megaloblastic anaemia. The treatment is with high dose thiamine supplementation that can alleviate symptoms of diabetes and megaloblastic anaemia. Onset and progression of hearing loss may be delayed with treatment. Some cases may present with recurrent stroke as in the proband.

Mitral Valve Replacement for Enlarged Libman-Sacks Endocarditis in a Patient with Persistent Primary Antiphospholipid Syndrome and Recurrent Stroke: A Case Report.

BACKGROUND Anticardiolipin antibodies in patients with Libman-Sacks endocarditis (LS) are indicative of comorbid antiphospholipid syndrome (APS) and can result in cerebral infarctions. We describe a case of LS and primary APS with recurrent cerebral infarctions despite anticoagulation treatment. The patient underwent surgery for enlarged LS vegetation with high titers of antiphospholipid antibodies. CASE REPORT A 41-year-old Japanese man was admitted to hospital for small cerebral infarction recurrence in a left parietal lesion. At age 35, the patient had suffered multiple cerebral infarctions. He was found to have high serum titers of all 3 antiphospholipid antibodies. Transesophageal echocardiography (TEE) findings were normal. Differential diagnosis ruled out other autoimmune diseases and a clinical diagnosis of primary APS was made. Warfarin anticoagulation was started. When cerebral infarction recurred 6 years after the first episode, serum titers of antiphospholipid antibodies remained high, and TEE showed a 7×8 mm area of mitral vegetation. A TEE results from his first admission revealed a 5×6 mm area of mitral vegetation, which was believed to be related to the current vegetation. As anticoagulation produced no improvement, the mitral valve was replaced with a mechanical valve. Examination of the excised vegetation found it to be consistent with LS. The patient made good progress within 3 years after surgery. CONCLUSIONS LS size can increase despite anticoagulation in cases with high titers of all 3 antiphospholipid antibodies and cerebral infarction. Such patients require ongoing TEE follow-up and surgical treatment should be considered.

Cerebral Infarction Caused by Trousseau's Syndrome Associated With Lung Cancer.

Lung cancer is one of the common cancers that can cause Trousseau's syndrome. However, there are few reports of cerebral infarction due to Trousseau's syndrome associated with lung cancer. The aim of this study is to investigate the clinical features of lung cancer-related cerebral infarction and effective management practice. Japanese patients diagnosed with Trousseau's syndrome-related cerebral infarction associated with lung cancer between August 2012 and November 2021 in our hospital were retrospectively enrolled. Clinical data, treatment, and outcomes of the patients were collected. Ten patients were enrolled. The median age was 65 years (range: 43 - 84 years). All patients had advanced lung cancer. The histological types were adenocarcinoma (n = 8), pleomorphic carcinoma (n = 1), and small cell lung cancer (n = 1). Recurrent cerebral infarction occurred in six patients. Among four patients who had continued heparin since the initial infarction, recurrence occurred in one. D-dimer was high in all 10 patients at the initial cerebral infarction. D-dimer level at the time of recurrent cerebral infarctions was higher than that at the first cerebral infarctions. Since performance status declined in nine patients, one patient continued anticancer drugs after cerebral infarction. Four patients died within 100 days of the onset of cerebral infarction. Cerebral infarction of lung cancer-related Trousseau's syndrome has poor prognosis. Heparin may be effective in controlling the condition. In addition, D-dimer may serve as a marker of cancer-related thrombosis.

Detection of atrial fibrillation in patients with cryptogenic stroke: The Nordic Atrial Fibrillation and Stroke (NOR-FIB) study - topline results

Abstract Funding Acknowledgements Type of funding sources: Other. Main funding source(s): NOR-FIB is an investigator driven academic study. 100 of 259 devices are supported by Medtronic. BRT and ATL are recipients of a PhD grants from the South-Eastern Norway Regional Health Authority. The study is supported by the research infrastructure of the European Cerebrovascular Research Infrastructure (ECRI). Background Cardioembolism due to occult atrial fibrillation (AF) is one of the common causes often identified by additional investigations in patients with cryptogenic stroke (CS). A large proportion of recurrent cerebral infarctions caused by AF can probably be prevented if more patients receive optimal cardiac monitoring after CS and TIA. Purpose The aim of the prospective observational multi-center NOR-FIB study was to detect and quantify AF in patients with cryptogenic stroke or TIA under continuous 12 months cardiac rhythm monitoring with an implantable cardiac monitor (ICM) and to possibly identify biomarkers predicting incident AF. Methods Patients with cryptogenic stroke and TIA diagnosed after state-of-the-art work-up had their ICM implanted by a stroke physician within 14 days after symptom onset. All patients were followed clinically and by rhythm monitoring for 12 months. AF was defined by detected atrial arrhythmia episodes ≥ 2 min, and these patients were considered for a change of their secondary prevention from antiplatelet drugs to oral anticoagulants (OAC). Results A total of 259 patients with cryptogenic stroke or TIA from 18 hospitals in Norway, Sweden and Denmark were included. After 12 months follow-up 74 (28.6 %) patients were diagnosed with paroxysmal AF, of which 91.9% were asymptomatic. Patients with AF had significantly higher mean age (72.6 vs 62.2, p<0.001), more severe stroke (median National Institute Stroke Scale Score on admission 2 vs 1, p 0.002) and higher pre-stroke median CHA2DS2-VASc score (3 vs 2, p<0.001) than patients without AF. Both hypertension and hyperlipidemia was more common in patients with AF. In 64 (86.5%) cases AF was detected early after index stroke, i.e., within the first two months of monitoring (mean 47.7 days + 52,1). Recurrent AF episodes were detected in 68 (91.9%) cases. Of the 74 AF patients, 72 (97.3%) were switched to OAC. Recurrent strokes during follow-up occurred in 2 AF patients (2.7 %) and in 9 non-AF patients (4.9 %). Conclusion AF was detected in 29% of all cryptogenic stroke/TIA patients. Most of the patients were asymptomatic for their arrhythmia, and would have gone undiagnosed without a continuous monitoring approach. Since most of the patients with detected AF were switched to OAC, the 12 months risk of recurrent stroke in this group was low. Prolonged cardiac rhythm monitoring with ICMs is an effective tool for diagnosing underlying asymptomatic AF in a patient population typically confined to a stroke unit.

A rare angiopathy with lesions of the skin and central nervous system: Sneddon syndrome and disease

Sneddon syndrome is a rare, progressive disease that affects relatively young people, mainly female, and manifests itself in two main symptoms: livedo reticularis and recurrent cerebral infarctions. First described in 1965 by the English dermatologist Ian Bruce Sneddon, the syndrome named in his honor unites a heterogeneous group of diseases with different pathogenesis and similar clinical manifestations. Skin manifestations of the disease in the form of reticular livedo are benign, cerebrovascular manifestations are reduced to repeated cerebral infarctions, progressive dementia and can lead to the death of patients. The literature review describes the basic concepts of the etiology, pathogenesis of primary and secondary forms of Sneddon syndrome, and proposes the concept of Sneddon’s disease to describe the primary forms of the syndrome. The article contains photographs of patients from the clinical practice of the authors of the article. The review also describes methods of diagnosis and differential diagnosis of the disease and recommended methods of treatment, which is based on the elimination of additional risk factors for vascular damage and thrombosis, blood pressure control, lipid and carbohydrate metabolism, as well as the appointment of anticoagulant and disaggregant therapy. The use of anti-inflammatory or immunosuppressive therapy remains controversial. The main goal of treatment is the prevention of organic brain damage and neuropsychiatric complications. Unfortunately, the neuropsychiatric prognosis of Sneddon syndrome is relatively poor with the development of impaired memory, concentration, and visual-spatial skills. In exceptional cases, the progression of the disease can lead to death.

Thoracic endovascular aortic repair for recurrent stroke due to atheromatic plaque in the proximal descending aorta: a case report

BackgroundDiastolic retrograde flow in the descending aorta (DAo) may occur in the presence of atherosclerosis and may be overlooked as a mechanism of retrograde embolization in patients with stroke. We performed thoracic endovascular aortic repair (TEVAR) in a patient with recurrent cerebral infarctions for treatment of aortic aneurysm with atheromatic plaque, which was considered as the source of embolism.Case presentationA 56-year-old man with a history of idiopathic thrombocytopenia and hypertension was referred to our hospital with paralysis of the right upper and lower limbs. Multiple cerebral infarctions were found and treated; however, 1 month later, another cerebral infarction developed. A small saccular aortic aneurysm with plaque was found beyond the left subclavian artery, and this site was deemed as the source of embolism. We performed TEVAR to prevent further recurrence of cerebral infarctions. No cerebral infarctions were observed 6 months post-operation.ConclusionsTEVAR is a useful treatment for not only aortic aneurysm and dissection, but also cerebral infarctions caused by an embolic source proximal to the DAo due to retrograde aortic blood flow.

Recurrent Cerebral Infarctions and Multi-Vessel Thrombosis in a Male Patient with Refractory Idiopathic Thrombocytopenic Purpura: a Case Report

Recurrent Cerebral Infarctions and Multi-Vessel Thrombosis in a Male Patient with Refractory Idiopathic Thrombocytopenic Purpura: a Case Report

O35 Efficacy & safety of tocilizumab in GCA: a multi-centre experience of NHS clinical practice

Abstract Background Giant cell arteritis (GCA) has a relapsing, remitting course with ischemic/vascular damage in a number of cases. Glucocorticoids (GC) remain mainstay of treatment with SAEs e.g. diabetes and fractures commonly seen. The GiACTA trial of tocilizumab (TCZ) in GCA led to NICE approval for 12 month’s therapy in relapsing/ refractory disease. We report real life experience in 51 cases. Methods Multicentre retrospective data collected through ENRAD and individual centres across England (Table 1). Outcomes were as assessed by supervising clinicians. Results Fifty-one patients were treated with TCZ (26 cranial, 13 LVV, 11 both). 11 (22.0%) had prior permanent sight loss due to AION, CRAO or both. One died prior to TCZ, from recurrent cerebral infarcts. Mean age was 71 years with 66.7% females. Ultrasound (US) was used for diagnosis in 28 (56.0%), exclusively or in combination with other tests such as biopsy, PET-CT or CTA. 70% had prior DMARD (LEF/MTX/AZT/MMF/CYC, mean duration 58 weeks). MTX was used in 50% alone or combination. Initial TCZ was given as subcutaneous or intravenous (85.4% and 14.6%). TCZ indication in 89.8% was relapsing, refractory or ischemic disease. Steroid AEs that prevented optimal GC dosing constituted 10% of TCZ indication. Mean follow up was 31 weeks, 30 (58.8%) continuing TCZ uninterrupted, 4 completed 12 months, 4 discontinued due to SAEs. Ten had brief interruptions due to minor AEs. One restarted after 12 months due to flare but died 12 weeks later from abdominal aortic aneurysm rupture. At follow up, 37(74%) were in remission with a mean GC dose of 6.97 mg (mean GC dose pre-TCZ 33.2 mg), Thirty-two (65.3%) ≤ 5mg. In 4 (8%) outcome could not be assessed as either they started TCZ recently or about to start. In 53% lipids were not checked after commencing TCZ. AEs seen were 37.0%. Conclusion In clinical practice, TCZ is efficacious and safe in relapsing/refractory/ischemic GCA. I.V. route is an option awaiting NHSE approval in refractory visual symptoms. Infections and other AEs do occur but overall safety profile is acceptable. US is excellent in identifying patients at need and is a useful disease activity monitoring tool. Disclosures A. Sebastian: None. A. Kayani: None. C. Ranasinghe: None. F. Hall: Consultancies; Sobi, S. Grants/research support; Actelion. C. Ransom: None. D. Jayne: None. V. Quick: Honoraria; Roche. M. Hughes: None. J. Stack: Member of speakers’ bureau; Roche. C. Mukhtyar: None. F. Coath: None. A. Bharadwaj: None. V. Hajela: None. S. Butler: Consultancies; Lily. M. Lwin: None. C. Edwards: Consultancies; Roche, Chugai. Grants/research support; Roche, Chugai. M. Whitlock: None. B. Dasgupta: Consultancies; Roche, Sanofi, GSK. Grants/research support; Roche.

Interventricular septal hematoma complicating placement of a ventricular assist device in an infant and support with bi-atrial cannulation.

Interventricular septal hematoma complicating placement of a ventricular assist device in an infant and support with bi-atrial cannulation.

Dabigatran Treatment of Acute Non-Cardioembolic Ischaemic Stroke: A Randomised Controlled Trial

Background: Transient ischaemic attack (TIA) and minor ischaemic stroke patients are at high risk for recurrent events. Anticoagulants are associated with reduced recurrent ischemic stroke rates, but also increased risk of haemorrhagic transformation (HT). Dabigatran, a novel oral anticoagulant associated with reduced risk of intracranial bleeding compared with warfarin in atrial fibrillation patients, has not been assessed in acute brain ischemia. We hypothesised that HT rates are not increased by dabigatran relative to aspirin after acute ischaemic stroke/TIA. Methods: DAbigatran Treatment of Acute Stroke II (DATAS II) was a prospective, randomised open label, blinded endpoint trial of dabigatran versus aspirin in acute stroke/TIA (clinicatrials.gov NCT02295826). Non-cardio-embolic stroke/TIA patients (NIHSS score ≤9, infarct volume < 25 ml) who did not receive thrombolytic therapy or thrombectomy were randomised 1:1 to dabigatran 150 mg BID (110 mg BID in patients ≥80 or if CrCl <50 ml/min) or aspirin (81 mg OD). Magnetic resonance imaging was performed prior to randomisation and was repeated at day 30, with clinical assessments at 30 and 90 days. Imaging endpoints were ascertained centrally by readers blinded to treatment. The primary endpoint was symptomatic HT within 37 days of randomisation. Secondary endpoints included asymptomatic HT and recurrent cerebral infarcts. Findings: A total of 305 patients, mean age 66±13.21 years, were randomised to dabigatran or aspirin a mean of 40.10±19.44 hours after symptom onset. The qualifying event was a TIA in 21%, and ischaemic stroke in79% of patients. Median NIHSS (IQR) was 1 (0-2), and mean infarct volume 3.2±6.5 ml. No symptomatic HT occurred. Asymptomatic petechial HT developed in 11/142 (7.8%) of dabigatran-assigned patients and 5/142 (3.5%) of aspirin-assigned patients (RR=2.32, 95% CI 0.78, 6.85). Baseline infarct volume predicted incident HT (OR 1.07; 95% CI 1.03-1.12, p=0.0026). Incident covert infarcts on day 30 imaging occurred in 9/142 (6.3%) of dabigatran and 14/142 (9.8%) of aspirin patients (RR=0.62, 95% CI 0.26, 1.48). There were no major bleeding events with either treatment. Interpretation: Dabigatran given to patients with acute minor non-cardioembolic ischaemic stroke/TIA was safe and was associated with a risk of HT similar to aspirin. Trial Registration Number: clinicatrials.gov NCT02295826 Funding Statement: DATAS II was funded by the Canadian Institutes for Health Research (G327075). Declaration of Interests: Dr. Butcher has served as an advisory board consultant and received speaker’s fees and investigatorinitiated grant support from Boehringer-Ingelheim, Bayer, BMS-Pfizer and Servier. Dr. Sharma has served as an advisory board consultant and or received speaker’s fees and investigator-initiated grant support from Boehringer-Ingelheim, Bayer, BMS-Pfizer, Daiichi Sankyo and Servier. Dr. Benavente has served as an advisory board consultant and or received speaker’s fees from Boehringer-Ingelheim, Bayer, BMS-Pfizer and Servier. Dr. Field has served as an advisory board consultant and received speaker’s fees from BoehringerIngelheim, Bayer, BMS-Pfizer and Servier and investigator-initiated grant support from BoehringerIngelheim and Bayer (study medication). Dr. Hill has served as a consultant for Boehringer-Ingelheim. He has received grants to the University of Calgary from Medtronic, Stryker, Boehringer-Ingelheim Canada, Bayer Canada. Dr. Gioia has received speaker honoraria from Bayer, and BMS Pfizer and has served as an advisory board consultant for Bayer, and Servier Inc. Dr. Coutts has served as an advisory board consultant for BMS-Pfizer. Boehringer-Ingelheim is providing study drug for an investigator-initiated study led by Dr. Coutts. Ethics Approval Statement: The trial was approved by research ethics review boards at each institution.

Multiple and recurrent cerebral infarctions in a patient with idiopathic thrombocytopenic purpura

• Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder which predispose to bleeding.

Cost-Effectiveness of Blood Transfusions Versus Observation for Silent Cerebral Infarcts from the Silent Cerebral Infarct Trial

▪ BACKGROUND AND OBJECTIVEIn the Silent Cerebral Infarct Trial (SIT), regular blood transfusion therapy significantly reduced the incidence of recurrent cerebral infarctions in children with sickle cell anemia (SCA). As a follow-up analysis of the SIT Trial, we compared healthcare utilization, as measured by adverse events, hospitalizations and costs in regularly transfused children (transfusion group) to those who were not transfused (observation group). <>METHODS: In this multi-center trial, we randomly allocated 196 children aged 5-15 years with SCA and prior history of silent cerebral infarcts (SCI) to receive monthly blood transfusion or observation for at least 36 months or until a study endpoint was reached. The number of and reasons for hospitalizations were recorded at each site. The transfusion group was determined by a protocol approach, with all patients receiving regular transfusions over a period of at least 6 months included, irrespective of the original group assignment in the SIT study. Estimated costs per day of hospitalization were determined using data obtained from the 14 SIT institutions which contributed administrative data to the Pediatric Health Information System (PHIS) database maintained by the Children’s Hospital Association. Inpatient costs were based on length of hospital stay, modified by the occurrence of categories of adverse events in the following non-overlapping hierarchy: acute chest syndrome, vaso-occlusive pain crisis, fever/infection, exchange transfusion, surgery and asthma. Outpatient expenses not related to transfusion or iron chelation were considered equivalent for transfused patients and controls for the purposes of this study and were not included in the costing model. Chelation and blood transfusion costs were based on a child that weighed 30 kg and received 20 mg/kg/day of deferoxamine or deferasirox. Follow-up occurred from time of random allocation to primary endpoint (overt stroke or new or progression of SCI) or exit MRI, whichever came first. The SIT Trial is registered at www.clinicaltrials.gov (NCT00072761). <> <>RESULTS: A total of 90 and 106 patients comprised the final transfusion and observation groups, respectively. Fifteen of the patients originally randomly allocated to the transfusion group crossed over to the observation group by either never receiving blood transfusion (N=9) or receiving less than 6 months of regular blood transfusion (N=6) and were counted as not being effectively transfused (i.e., part of the observation group). The mean follow up for individuals who did or did not receive blood transfusion therapy was 3.04 and 3.01 years, respectively. The average age of all participants at randomization was 10.0 years, with 43.4% males. There were 144 hospitalizations in the transfusion group and 269 in the observation group; average length of hospital stay was 2.5±1.8 days vs. 3.4±2.2 days for transfused and observation groups, respectively (p<0.001). An average of 1.6 and 2.5 hospitalizations occurred per patient with a total of 358 and 912 patient days for patients in the transfusion and observation groups, respectively. The most common reason for hospitalization was an acute pain episode (49.6%), followed by acute chest syndrome (9.4%). For every 100 children with history of SCI treated with regular blood transfusions for one year, there were 71 fewer hospital days for all SCA-related conditions per SCI prevented (157 fewer days/2.19 fewer SCIs) when compared to 100 children with SCA and history of SCIs who are not treated with transfusion therapy. Hospitalization costs were reduced 54% per year ($4,302 vs. $9,407) for children receiving blood transfusion therapy compared to those observed. Total yearly costs not related to hospitalization for patients in the transfusion group ranged from $18,149 to $67,361/year, depending on the estimated costs for the type of chelation used and type of red blood cell transfusion (manual partial exchange or apheresis). (Table) CONCLUSIONSChildren with SCA and silent cerebral infarcts receiving regular blood transfusions have a 54% relative reduction in hospitalization cost when compared to children with SCA; however their outpatient costs of monthly prophylactic blood transfusions are high and heavily dependent upon the type of blood transfusion therapy and choice of chelation therapy. [Display omitted] DisclosuresMcCavit:Novartis: Speakers Bureau; Pfizer: Consultancy, Research Funding. King:HRSA: Research Funding; NIH - NHLBI: Research Funding; NIH - BMTCTN: Research Funding. Strouse:NHLBI: Research Funding; HRSA: Consultancy; Maryland Dept of Health and Mental Hygiene: Research Funding; HRSA: Research Funding. Casella:Johns Hopkins: Patents & Royalties; Mast Therapeutics: Research Funding; ImmunArray: Patents & Royalties.

Progressive Loss of Brain Volume in Children with Sickle Cell Anemia: A Report from the Silent Cerebral Infarct Transfusion Trial Cohort

Introduction: Neurological injury is a common complication of sickle cell anemia (SCA). SCA is the most common cause of stroke in children between 1-14 years of age, while silent cerebral infarct is the most common neurologic injury in this population. Young children are particularly vulnerable to the adverse impact of strokes, and are at risk of cognitive and developmental deficits; thus, we sought to examine brain volume changes in children with SCA.The Silent Cerebral Infarct Transfusion (SIT) Trial was a multicenter trial designed to determine the efficacy of blood transfusion therapy for prevention of recurrent silent cerebral infarcts in participants with SCA. This intervention trial followed 196 children with SCA aged 5-14 years randomized to receive blood transfusion therapy or observation for 36 months (DeBaun et al. N Engl J Med. 2014;371(8):699-710). As a planned secondary analysis, we used the SIT Trial brain imaging archive to determine longitudinal brain volume change in children with SCA. We performed a multivariable analysis using demographic and clinical variables to identify predictors of brain volume change.MethodsSIT participants: The current study used a subset of the registered participants (n=169) in the SIT Trial with brain MRIs at study entry and exit, demographic and clinical information.Estimation of brain volume and percent brain volume change: Baseline brain volume and longitudinal percent brain volume change (PBVC) were derived from T1-weighted structural MRIs using the automated image analysis programs SIENAX and SIENA, respectively, from the FMRIB Software Library (FSL) (www.fmrib.ox.ac.uk/fsl). For each participant, PBVC was calculated between entry and exit brain MR scans, a mean time of 41 ± 6 months. A negative PBVC indicates a decrease in brain volume and a positive PBVC indicates an increase in brain volume. All analyses were performed blinded to the participant's identity.Statistical Analysis: A multivariable linear regression analysis was used to determine predictors of PBVC. The model included the following potential covariates : demographic variables (age, gender), laboratory measures (baseline oxygen saturation, steady state hemoglobin, steady state WBC, systolic blood pressure, % Hemoglobin F, steady state reticulocytes), clinical measures (acute chest syndrome and painful crisis rates), and regular transfusions provided as part of the trial.Results: Of the 169 SIT participants, 96 were male and 73 were female, including 80 participants in the observation group and 89 in the transfusion group. Median normalized brain volume was 1,237.6 cm3 at the study entry and 1,194.7 cm3 at exit. Baseline brain volume in males was different from females (1,290.6 cm3 vs. 1,166.6 cm3; p<0.001). Comparing total brain volumes from entry to exit, the mean percent brain volume change was -2.7% (median, -2.4%; SD, 2.75). Percent brain volume change in males was not different from females (-2.6% vs. -2.8%; p=0.75). In the multivariable analysis of demographic, clinical, and pain measures, age was the only significant predictor of PBVC (p<0.001) (Figure 1).Conclusion: Using longitudinal MRI images from the SIT Trial, we demonstrate progressive brain volume loss in children, with SCA with a mean percent brain volume change of -2.7% in the SIT Trial cohort. In comparison, brain volumes of children without SCA have been reported to remain stable in this age group (Cereb Cortex. 2012; 22 (1):1-12). We conclude that loss of brain volume begins early in children with SCA. Further investigations into the etiology of brain volume loss in children with SCA, and measures to better understand and reduce or prevent brain volume loss in SCA are needed.

Carotid Stump as a Cause of Recurrent Embolism-Endovascular Treatment in a Case of Congenital Carotid Stump Syndrome.

Dear Sir: Cryptogenic stroke, a stroke for which there is no specific attributable cause after a comprehensive evaluation for the most common causes, accounts for about one-fourth of stroke patients. Most cryptogenic strokes are thromboembolic and occur more frequently in younger patients. There have been efforts to detect paradoxical embolism in patients with cryptogenic embolic stroke because chronic anticoagulation can effectively prevent recurrent stroke in these patients. However, other causes may also cause cryptogenic embolic stroke, and the various causes of cryptogenic strokes have been increasingly recognized recently [1]. Herein, we present a case of recurrent strokes possibly associated with a carotid stump related to congenital non-bifurcating common carotid artery (CCA) and its successful treatment using stenting. A 39-year-old man was admitted to our hospital due to recurrent ischemic strokes. He had no risk factors for stroke except for smoking. He had a history of recurrent stroke despite the use of antithrombotics. Three years ago, he had a cortical infarction in the left anterior cerebral artery area with symptoms of transient right-sided weakness and was treated with cilostazol owing to an aspirin allergy (Figure 1). One year ago, he was admitted for transient right-sided weakness and motor aphasia that lasted 5 minutes. Magnetic resonance imaging (MRI) revealed an acute cortical infarction in the left middle cerebral artery (MCA) area (Figure 1). He was treated with warfarin temporarily, and was subsequently treated with clopidogrel. Figure 1. A total of three recurrent left hemispheric strokes occurred in 3 years. (A) Diffusion-weighted imaging findings of recurrent left middle or anterior cerebral artery area cortical infarcts. The patient first had a left anterior cerebral artery cortical ... While taking clopidogrel, the patient experienced a third ischemic stroke. He presented with sensory change in the right hand. The neurologic exam result was normal, except for numbness in his right hand area. Diffusion-weighted imaging revealed an acute cortical infarction in the left MCA area and a chronic infarction in the left MCA and the anterior cerebral artery areas (Figure 1). Magnetic resonance angiography showed congenital agenesis of the left internal carotid artery (ICA) and an ICA stump. The left carotid area was supplied by a connection between the distal ICA and the external carotid artery (ECA) (Figure 1B). There was no stenotic lesion in the intracranial vessels and in the distal left common carotid artery of ECA origin. Carotid CT angiography did not show any atherosclerotic plaque in the carotid stump area. A selective left CCA injection revealed a bulbous carotid stump and turbulent flow within the ICA stump (Figure 2A). Comprehensive diagnostic work ups, including laboratory tests for coagulopathy, echocardiography with the bubble test to detect paradoxical embolism, 24-hour Holter monitoring, and coronary CT angiography for aortic pathology yielded negative results. Laboratory test for clopidogrel resistance was negative. Figure 2. Conventional angiographic findings. (A) Cerebral angiography revealed turbulent flow in the left carotid stump. (B) Stenting and stent-assisted coiling were performed across the origin of the left internal carotid artery. (C) No residual stump on postprocedure ... The decision was made to insert a stent and coil to block the stump because the patient suffered a recurrent left hemispheric stroke despite receiving antithrombotics, and only an ICA stump could be a possible source of the embolism. Stent insertion and stent-assisted coiling were successfully performed without complications (Figure 2B and ​andC).C). A closed cell-type self-expanding stent (Wall stent, Boston Scientific, Ireland) was used. The patient was discharged with triflusal and clopidogrel treatment, and remained asymptomatic at 5 months of follow-up. Carotid stump syndrome remains a rare entity, but it is a treatable cause of recurrent retinal and hemispheric symptoms after ICA occlusion. Rarely, after complete occlusion of the ICA, a short patent portion of the proximal ICA can be shown by angiography as well as by other imaging modalities, and is referred to as the “carotid stump.” In this case, congenital non-bifurcating CCA with an anomalous ECA-ICA connection, rather than acquired ICA occlusion could be the cause of the carotid stump [2]. Both surgical exclusion of the carotid stump with endarterectomy of the ECA, and carotid stenting of the distal common and external carotid arteries to exclude the ICA stump, can be used. Lakshminarayan and colleagues reported the results of a small case series that used endovascular treatment suggesting that long-term outcomes are equivalent to those achieved by surgery [3]. Our and other cases suggest that in the case of recurrent retinal or hemispheric symptoms with an ipsilateral carotid stump but no other demonstrable cause, carotid stenting with/without coiling should be considered to normalize the regional hemodynamics associated with the carotid stump.

Recurrent Ischemic Strokes and Headaches Originating from Lambl's Excrescences: A Case-Report

Determining recurrent stroke etiology and subsequent therapeutic approaches is an important, but not always straightforward task. Lambl’s Excrescences (LE) are cardiac valve strands that can be a source of recurrent cerebral ischemic events. We report the case of a 60 year old female that was seen in neurologic consultation for recurrent ischemic strokes. The patient developed migraine headache with complex auras at the age of 59. She reported three admissions for ischemic strokes in the past year. Stroke work-ups were unrevealing. The patient remained on daily Aspirin. Upon current admission, a brain MRI showed a combination of sub acute and chronic infarcts in diverse brain areas bilaterally. Further diagnostic evaluation revealed a relatively unremarkable CT head and neck angiogram. Transcranial Doppler (TCD) showed increased pulsatility index throughout the insonated vessels. Transesophageal echocardiogram (TEE) showed a trileaflet aortic valve with a 10 mm long thin fibrin strand prolapsing into the left ventricle outflow tract in diastole, meeting echocardiographic criteria for an LE. The patient refused anticoagulation and surgical intervention. She opted instead for a combination of two antiplatelet agents. Recurrent cerebral infarcts presenting concurrently with late-age onset migraine with aura should raise a clinical suspicion of cardiogenic valvular etiology. The use of TEE in evaluating patients with unexplained recurrent stroke is imperative so that LE is not missed. Anti-platelet agents, anti-coagulants, and cardiothoracic surgical intervention are proposed in the literature for the management of long-term complications associated with LE. Asymptomatic patients should be monitored, counseled, and made aware of LE complications, neurological symptoms, and alarm signals. Migraine headaches associated with LE should raise a red flag and prompt treatment with anti-platelet agents.

Regular Transfusion Can Significantly Reduce the Chance of Recurrent Cerebral Infarcts in Young Sickle Cell Patients

Regular Transfusion Can Significantly Reduce the Chance of Recurrent Cerebral Infarcts in Young Sickle Cell Patients

Use of Flow-Diverting Devices in Fusiform Vertebrobasilar Giant Aneurysms: A Report on Periprocedural Course and Long-Term Follow-Up

Fusiform vertebrobasilar giant aneurysms are a rare (<1% of all intracranial aneurysms) but challenging aneurysm subtype. Little data are available on the natural history of this aneurysm subtype and the impact of the use of flow-diverting stents on the long-term clinical and imaging follow-up. In this article, we present our experience with the treatment of fusiform vertebrobasilar giant aneurysms by flow diverting stents. We aim to stimulate a discussion of the best management paradigm for this challenging aneurysm subtype. We retrospectively identified 6 patients with fusiform vertebrobasilar giant aneurysms who had been treated with flow-diverting stents between October 2009 and March 2012 in our center. The available data were re-evaluated. The modified Rankin Scale score was assessed before intervention, during the stay in hospital, and at discharge. Six patients were identified (all male; age range, 49-71 years; median age, 60 years). Handling of material was successful in all cases. No primary periprocedural complications occurred. The mean follow-up was 13 months (15 days to 29 months). During follow-up, 3 of 6 patients had recurrent cerebral infarctions, but no patient experienced SAH. Two patients presented with acute thrombotic stent occlusion. The modified Rankin Scale score was not higher than 3 in any of the cases before intervention, whereas the best mRS score at the last follow-up was 5. Four of 6 patients died during follow-up. Endovascular treatment of fusiform vertebrobasilar giant aneurysms with flow-diverting devices is feasible from a technical point of view; however, changes in hemodynamics with secondary thrombosis are not predictable. We currently do not intend to treat fusiform vertebrobasilar giant aneurysms with flow-diverting devices until we have further understanding of the pathophysiology, natural history, and hemodynamic effects of flow diversion.

Recurrent Cerebral Infarctions in a Patient with Ovarian Cancer: A Fatal Case of Trousseau’s Syndrome

Cancer patients are at increased risk of thromboembolic events, and the combination of these two pathological conditions is known as Trousseau’s syndrome. Venous thromboembolisms (VTE) and pulmonary embolisms (PE) are the most common clinical features of Trousseau’s syndrome. However, arterial thromboembolisms can also occur. We report the case of a 55-year-old ovarian cancer patient, who developed repeated cerebral infarctions with catastrophic consequences. The initial ischemic stroke developed in the right middle cerebral artery territory 4 days before she was scheduled to undergo surgery. Anticoagulation therapy involving unfractionated heparin was administered, and the patient underwent emergency surgery, during which the right ovarian tumor was completely removed. Under a diagnosis of stage Ic ovarian cancer, postoperative adjuvant chemotherapy consisting of carboplatin plus paclitaxel was initiated. During the course of the adjuvant chemotherapy, the patient developed recurrent ovarian cancer, and second-line chemotherapy involving single agent gemcitabine was scheduled. After two months of second-line chemotherapy, which was not effective, the patient developed a second cerebral infarction in her left middle cerebral artery despite continuing to receive anticoagulation therapy. She died 5 days after the second stroke. Given the fact that recurrent thromboembolic events can occur even during ongoing anticoagulation therapy, intensive cancer treatment, thromboprophylaxis, and careful follow-up for the early detection of recurrent stroke events are recommended.

Role of high-resolution magnetic resonance imaging in the diagnosis of primary angiitis of the central nervous system.

BackgroundPrimary angiitis of the central nervous system (PACNS) is a rare disorder and is often difficult to diagnose due to the lack of a confirmatory test. PACNS can generally be diagnosed based on typical angiographic findings. We describe herein a patient diagnosed with PACNS despite the presence of normal findings on conventional angiography.Case ReportA 44-year-old man with a recent history of ischemic stroke in the right posterior cerebral artery territory developed acute-onset vertigo. Diffusion-weighted imaging revealed an acute infarction within the left posterior inferior cerebellar artery. His medical history was unremarkable except for hyperlipidemia; the initial examination revealed mild gait imbalance. During the 10 days of hospital admission, the patient experienced four recurrent ischemic strokes within the posterior circulation territory (occipital lobe, pons, and cerebellum). He was diagnosed with recurrent cerebral infarctions due to PACNS. The basilar artery exhibited no demonstrable luminal stenosis, but there were direct imaging signs of central nervous system angiitis including wall thickening and contrast enhancement. High-dose intravenous steroid therapy followed by oral prednisolone was administered. There was no further stroke recurrence and follow-up imaging of the arterial walls showed normalization of their characteristics.ConclusionsThe present case emphasizes the importance of wall imaging in the diagnosis and treatment of PACNS.

Rabson–Mendenhall syndrome with recurrent cerebral infarcts caused by a novel INSR mutation

Rabson-Mendenhall syndrome (RMS) is an autosomal recessive disorder characterized by extreme insulin resistance and certain characteristic phenotypic features. The primary defect lies in the insulin receptor and involves biallelic mutations that lead to a loss of function through various postulated defects. We present a 9-year-old female patient with RMS complicated by multiple cerebral infarcts, in whom genetic analysis revealed a novel insulin receptor mutation.