Un caso probable de enfermedad de Creutzfeldt-Jacob de variante esporádica con larga supervivencia en Perú

Creutzfeldt-Jakob disease (CJD) is the commonest human prion disease with a reported annual incidence rate of one per million worldwide. CJD has a bad prognosis, and the mean length of survival is 4-6 months. Only 11 cases have been reported in the literature from Perú. Thus, we report a case of a 66-year-old male patient with the diagnosis of probable sporadic CJD in whom the survival duration was of 25 months. We identify that the time from disease onset to myoclonus and to reach the akinetic mutism state, the absence of early imaging findings, the delayed cortical and basal ganglia involvement and the supportive therapies implemented were factors that could contribute for the long survival in this patient. La enfermedad de Creutzfeldt-Jacob (ECJ) es la enfermedad por priones más común con una incidencia anual de 1 caso por un millón de habitantes. La ECJ tiene un mal pronóstico y el promedio de vida es de 4-6 meses. Solo se han reportado en la literatura 11 casos de ECJ en Perú. Presentamos el caso de un varón de 66 años con el diagnóstico probable de ECJ esporádico con sobrevida de 25 meses. Identificamos que el tiempo entre el inicio de la enfermedad hasta las mioclonías y hasta el mutismo acinético, la ausencia de hallazgos imagenológicos tempranos, el compromiso tardío de corteza y ganglios basales y la implementación de tratamientos de soporte son factores que podrían explicar a la sobrevida prolongada

Clinical practice guidelines for the diagnosis and management of Duchenne muscular dystrophy: a scoping review

IntroductionOur objective was to identify recent CPGs for the diagnosis and management of DMD and summarize their characteristics and reliability.MethodsWe conducted a scoping review of CPGs using MEDLINE, the Turning Research Into Practice (TRIP) database, Google Scholar, guidelines created by organizations, and other repositories to identify CPGs published in the last 5 years. Our protocol was drafted using the Preferred Reporting Items for Systematic Reviews and Meta-analyses for scoping reviews. To assess the reliability of the CPGs, we used all the domains included in the Appraisal of Guidelines Research and Evaluation II.ResultsWe selected three CPGs published or updated between 2015 and 2020. All the guidelines showed good or adequate methodological rigor but presented pitfalls in stakeholder involvement and applicability domains. Recommendations were coherent across CPGs on steroid treatment, except for minor differences in dosing regimens. However, the recommendations were different for new drugs.DiscussionThere is a need for current and reliable CPGs that develop broad topics on the management of DMD and consider the challenges of developing recommendations for RDs

Abstract 1122‐000046: A Latin American Model of the PHASES Score

Introduction: The PHASES score was developed to predict the 5‐year risk of rupture for intracranial aneurysms (IAs). However, only populations from North America, Europe, and Japan were included in the original study. As the population of origin is an item in the score, it has yet to be applied in a Latin American population. We aimed to determine the best approximation to employ this model in this previously unstudied population. Methods: We extracted the data of 848 Peruvian patients with ruptured (n = 486) and unruptured (n = 362) IAs from 2010 to 2020. According to the PHASES score, the North American and European (other than Finish), Japanese, and Finnish populations are rated with 0, 3 and 5 points, respectively. Therefore, we developed three PHASES‐derived models in which our Peruvian population is rated with 0 (Model A), 3 (Model B), and 5 (Model C) points. We compared the observed probability of each model to the expected probability reported by the original PHASES score using a scatter plot. We then compared the goodness‐of‐fit of each model using the Hosmer‐Lemeshow test in STATA version 14. Results: Nineteen percent of the patients were female. Hypertension was found in 34% of patients and 15% were >70 years. Fifty‐four percent of the aneurysms were smaller than 7mm, 25% ranged between 7 and 9.9mm, 18% were between 10 and 19.9mm, and 3% were larger than 20mm. Previous subarachnoid hemorrhage was found in 4%. The location of the aneurysms was the internal carotid artery in 4%, the middle cerebral artery in 4%, and arteries of the anterior and posterior circulation (including the anterior and posterior communicating artery) in 92%. When Model A was applied, 63% of the patients among the ruptured subgroup have an estimated 5‐year risk of rupture of 3% while in the unruptured subgroup an estimated risk of <3% was observed only in 4% of the patients. When comparing observed to expected frequencies, model B presented a better calibration to the values reported by the original PHASES score. Additionally, the Hosmer‐Lemeshow showed Model B to have improved goodness‐of‐fit, compared to other models, although all presented adequate fit. Conclusions: We found that rating the Peruvian population with 3 points was the best approximation to the estimated risk calculated by the PHASES score to predict the 5‐year risk of rupture for IAs

Abstract 1122‐000130: Prediction of Risk of Rupture of Intracranial Aneurysms in a Latin‐American Population: A Restrospective Study

Introduction: The Population, Hypertension, Age, Size, Earlier Subarachnoid Hemorrhage (SAH), Site (PHASES) score was developed in North America, Europe, and Japan and it is a widely used model in day‐to‐day clinical practice for intracranial aneurysm (IA) rupture risk stratification. Here, we aimed to determine the predictors of aneurysm rupture and assess the components of the PHASES score in a Latin American population. Methods: Four hundred eighty‐six Peruvian patients presented at our institution with ruptured IAs between 2010 and 2020. We retrospectively collected the following variables: age, sex, a hypertension or diabetes mellitus history, previous SAH, the aneurysm size in millimeters (4), presence of a pseudoaneurysm, and aneurysm location. We then performed two separate multivariate analysis. For the first one, we included variables using a stepwise approach with a cut‐off p‐value of 0.2 in univariate logistic regression. For the second one, we evaluated the PHASES score components. A p‐value of 0.05 was considered statistically significant. Results: The median age was 56 years old, and 114 females were included. One hundred seventy‐five patients had a hypertension history, 21 had a diabetes history, and 11 had a previous SAH. Seventy‐eight patients had an aneurysm with 20mm. There were 372 patients with a saccular aneurysm and an associated pseudoaneurysm was found in 197 patients. The most common location was posterior communicating artery (n = 219), followed by the anterior cerebral artery (n = 125), the middle cerebral artery (MCA) (n = 58), branches from the posterior circulation (n = 33), and finally by a paraclinoid aneurysm (n = 33). In our initial multivariate analysis, only the presence of an associated pseudoaneurysm was an independent predictor for aneurysm rupture (OR 7.93; 95% CI 3.45 – 18.25). An age >70 years (OR 1.12; 95% CI 0.3 – 4.12), the male sex (OR 1.39; 95% CI 0.54 – 3.62), a hypertension history (OR 1.14; 95% CI 0.53 – 2.44), a size of 10–20mm (OR 1.46; 95% CI 0.46‐ 4.64), and location in the MCA (OR 1.07; 95% CI 0.25 – 4.57) also predicted a higher rupture risk but without statistical significance. When we performed a multivariate logistic regression of the factors making up the PHASES score, we found that only the age (OR 1.79; 95% CI 1.11‐ 2.88) and a hypertension history (OR 1.61; 95% CI 1.14 – 2.27) were independent predictors of aneurysm rupture. Conclusions: Based on our findings and its limitations, we observed that the presence of an associated pseudoaneurysm was a predictor for aneurysm rupture. Moreover, we found that only two of the five components of the PHASES score were predictors of the event in our population: the age and a hypertension history. Therefore, new research should be carried out in the Latin American population to establish predictors for the development of clinical predictive models in this field

Abstract Number ‐ 132: Stent‐assisted repair of the intracranial aneurysms with loading dose of antiplanets and perioperative events.

Introduction Introduction: Thromboemboembolic event (TEE) remains the mail perioperative challenge in addition to the potential intracranial hemorrhage (IH) in stent‐assisted repair of intracranial aneurysms (IA), and there are no standard antiplatelet strategies. Bases on the data, antiplatelets effects are more pronounce in first 4 hours after administration of antiplatelets. Objectives: To evaluate the effect of acute loading doses of aspirin and clopidogrel (LDAC) in TEE and hemorrhagic events associate with stent‐assisted repair of intracranial aneurysm. Additionally, to review the outcome of those patient who underwent stent‐assisted coiling using LDAC. Methods Consecutive patients underwent stent‐assisted repair of aneurysm using loading doses of aspirin 324 mg (4 baby aspirin) and clopidogrel 300 mg 2 to 4 hours before the procedure were enrolled from 2011 to 2022. Patients demographics including intra‐operative and post operative events were recorded. Outcome was measured using modified Rankin Scale (mRS) score. Results 96 patients (6 had baseline mRS 2) with mean age of 53 ± 13 underwent 98 stent‐assisted procedures including two Y‐stent neck reconstructions to treat 98 (6 ruptured and 41 symptomatic aneurysms) IA. Aneurysms are Right internal carotid artery (ICA) 25, left ICA 30, middle cerebral artery (MCA) 22, basilar artery 17 and anterior communicating artery 2.Stent deployment was achieved in all cases. Coil prolapsed and stretching occurred in one right ICA case, required deployment of additional 3 intracranial stents and one carotid artery stent with no clinical events. There was no intra‐operative rupture or IH. A small perioperative left hemispheric subarachnoid hemorrhage was observed on a right MCA aneurysm on day 3 after discharged, which resolved spontaneously without stopping antiplatelet. Intra‐operative stent thrombosis developed in one who had two aneurysms in right ICA with significant cerebrovascular risk factors; resolved with intraarterial integrilin followed by intravenous for infusion with no clinical consequences immediately and 12 months follow‐up; was resistant to clopidrogel and placed on ticagrelor.Post‐operative TEE were observed in 2 cases (2%); first event was observed on day 2 in a 42 years old woman with a giant right ICA giant aneurysm with NIHSS 6 and who recovered completely (NIHSS 0, mRS 1) in 90 days. The second event was visual distortion and diplopia (NIHSS 0) developed on day 2 in a 66 years old woman with basilar artery aneurysm. Her symptoms resolved completely and return to nursing job. All ruptured and symptomatic aneurysms were secure immediately and there are no subarachnoid hemorrhages during follow‐up. TEE was associated withperiorerative smoking. Immediate complete and near complete obliteration of aneurysm was observed in 66% and subtotal in 34%. There was no mortality or permanent disability in our series. 90 days mRankins 0 and 1 was observed in 96% and mRS 2 in 7% (baseline mRS) Conclusions Our study revealed that LDAC in stent‐assisted repair of IA is associated with a low TEE without added risk of IH with clinical good outcome. Our antiplatelets loading regimen may be an option in stent‐assisted repair of IA, especially when patients are unreliable and aneurysms are symptomatic. Further studies are required

Abstract 1122‐000061: Initial Experience Using the Transvenous Embolization for Intracranial Arteriovenous Malformations in a Reference Endovascular Center

Introduction: Transvenous embolization (TVE) is used in cases of arteriovenous malformations (AVMs) with specific characteristics such as small size (2). Complete obliteration was defined as the total absence of the nidus and vein, subtotal obliteration was defined as the embolization of >95% of nidus and partial obliteration was defined as the embolization of <95% of nidus. Procedure‐related complications were defined as those that occurred during the procedure and were divided as intraoperative rupture and thrombosis. Results: Twenty‐one patients harboring 21 AVMs were evaluated. Fourteen patients (67%) were women. The mean age was 24.5 ± 14.1 years (7 – 48 years). A good preoperative clinical condition was present in 20 patients. Twenty AVMs were ruptured (95.2%). The most frequent locations were thalamus/basal ganglia in 6 patients (29%), followed by temporal/insular in 5 patients (24%). Spetzler‐Martin grades III, II and I were present in 11, 9 and 1 patients, respectively. The mean number of feeders was 2.1 per AVM. The feeders arised from the MCA in 9 cases, followed by PCA in 5 cases, ACA and AChoA in 3 cases, AICA in 2 cases, and ECA and PCom in 1 case, respectively. The mean number of veins was 1.3 per AVM. Deep venous drainage was present in 12 cases (57%). The mean size of the AVM nidus was 15.7 ± 7.8 mm (3.7 – 34 mm). Previous trans‐arterial embolization was done in 10 patients (47.6%). Pre‐embolization hematoma evacuation was done in 4 patients (19%). An immediate complete obliteration was achieved in 18 patients (85.7%), whereas a subtotal and partial obliteration were achieved in 2 and 1 patients, respectively. A poor post‐operative clinical condition occurred in 4 patients (20%). Procedure‐related complications occurred in 4 patients (20%): 3 cases with intra‐operative rupture of the AVM nidus and 1 case of a thrombus in the M1 treated with stent retriever. Mortality occurred in 3 patients (14.2%) of which two presented intra‐operative rupture with intracerebral hematomas that required decompressive craniectomy. One patient presented a post‐operative bleeding of the AVM nidus that required external ventricular drainage and decompressive craniectomy. Follow‐up angiography was done in 4 cases with total obliteration of all the cases (100%). Conclusions: The transvenous approach has emerged as an alternative to trans‐arterial approach with high grades of immediate total obliteration rates, but with potential procedure‐related complications. Thus, this technique should be used in selected cases in order to achieve complete cure rates

Abstract 1122‐000080: Remote Learning for Neuroendovascular Procedures During the COVID‐19 Pandemic

Introduction: Telemedicine coupled with teleproctoring have been a novel practice in the last months given the restrictive mobilization orders worldwide due to the COVID‐19 pandemic, generating the impossibility to travel and learn new techniques or bring a proctor to perform procedures on‐site. Previous papers have reported the benefits of remote proctoring for endovascular procedures using online platforms, whereas others proposed the use of more simple platforms and applications for telemedicine such as ZoomÒ, WhatsAppÒ or Google GlassÒ. Our study aimed to describe our experience in the implementation of remote learning for endovascular treatment of vascular lesions using a multicamera system streamed by a web‐based platform. Methods: Endovascular treatment of aneurysms, arteriovenous malformations, and chronic subdural hematomas were streamed through a multicamera system installed in the angiosuite and shared via Zoom® platform. Four main cameras projected the angiography monitors, the operator’s hands and the overview of the room. Results: Eleven cases were performed. Aneurysms, arteriovenous malformations and chronic subdural hematomas were treated by endovascular means. Preoperative angiographic setup, intraoperative endovascular technique and postoperative management were discussed during the live streaming. No technical problems were reported. Conclusions: Remote learning with online platforms is nowadays an important tool but not a substitute to hands‐on learning for endovascular procedures. We recommend its implementation during the COVID‐19 pandemic as a temporary substitute especially for trainees who do not have access to advanced endovascular interventions

Abstract 1122‐000060: Endovascular Treatment of Large Intracranial Aneurysms Using Large Volume Coils: Safety and Efficacy

Introduction: Large volume coils in the treatment of intracranial aneurysms have demonstrated better packing density, shorter operative times, less number of coils per aneurysm and better cost‐effectiveness. However, most of the studies evaluated these coils in small or medium sized aneurysms. Therefore, our study aimed to determine our experience using large volume coils in the treatment of large intracranial aneurysms and determine its safety and efficacy. Methods: We retrospectively reviewed consecutive cases of intracranial aneurysms treated with Penumbra Coils 400 (PC400) at our institution between May 2016 and September 2019. Aneurysms > 12 mm in maximal diameter were selected according to the ISUIA trial. Clinical and radiological variables were collected. The modified Rankin Scale (mRS) was used to determine the clinical outcome and was dichotomized (good clinical outcome: mRS £2; poor clinical outcome: mRS >2). The Raymond Roy occlusion classification (RROC) was used to determine obliterations rates. An adequate obliteration was defined as RROC 1 or 2. Categorical variables were expressed as percentages and continuous variables as mean ± standard deviation. Stata v14 software was used for the analysis. Results: Eighteen patients harboring 18 intracranial aneurysms were treated. The mean age was 55 ± 12 years and 14 patients (78%) were women. A good preoperative clinical condition was found in 13 patients (72%). Ten aneurysms were unruptured (56%) and eight were dysplastic (44%). Paraclinoid aneurysms were the most frequently treated (61%). The mean number of coils were 6.2/aneurysm. The mean maximal diameter and neck were 18.9 ± 4.3 mm, and 5.7 ± 2.6 mm, respectively. The mean aspect ratio (AR) was 4 ± 1.9. Coiling was used in 10 cases (56%) followed by stent‐assisted coiling in 7 cases (39%) and balloon‐assisted coiling in 1 case. An immediate adequate obliteration rate was found in 8 cases (44%). Intraoperative complications occurred in two patients in which a coil loop migrated to the parent artery and a stent was placed without clinical consequences. In twelve patients (67%), angiographic follow‐up was performed. The mean follow‐up duration was 9.7 months. Nine patients (75%) showed a complete obliteration (RROC 1), whereas in three patients a residual aneurysm was still present. A good postoperative clinical outcome at discharge was found in 14 patients (78%). Procedure‐related morbidity and mortality were not reported. Conclusions: Embolization with large volume coils is a safe and effective alternative to conventional coils, with high obliteration rates at mid‐term follow‐up. Longer duration of angiographic follow‐up are needed in order to confirm the results presented here

Abstract 1122‐000081: Initial Experience Implementing the Transradial Approach for Diagnostic Cerebral Angiography

Introduction: The transradial approach (TRA) has gained acceptance among interventionists due to the lower operative complication rates, less operative time and better patient comfort. Our study aimed to analyze our experience in the implementation of the TRA for diagnostic cerebral angiographies. Methods: Between March 2020 and July 2021, consecutive patients who underwent TRA in two institutions were selected and data was retrospectively collected. Demographics, technical details of the procedure, duration of the procedure, fluoroscopy time and radiation exposure were analyzed. Results: A total of 76 angiographies using the TRA were done. The mean age was 47.5 ± 16.8 years (8 – 82 years). Women represented 57% of cases. Successful radial artery (RA) catheterization was done in 94% (85 patients/80 successful). A preoperative cocktail was used in all the cases. Subcutaneous lidocaine and a 5F sheath were used in 46% and 57% of cases, respectively. The Simmons 2 catheter was used in all the cases. Glidecath, followed by Merit were used in 40% and 32% of the cases, respectively. Right internal carotid artery (ICA), left ICA, right vertebral artery (VA), left VA, right external carotid artery (ECA) and left ECA were studied in 95%, 91%, 76%, 20%, 20% and 15% of the cases, respectively. Post‐operative vasospasm occurred in 29% of the cases, which resolved with intra‐arterial verapamil. Vasospasm was not associated with sheath diameter (p = 0.129) or local anesthesia (p = 0.065). The mean fluoroscopy time was 16 minutes. Conversion to TFA was done in 9 patients (10.6%), of which the RA was successfully catheterized in 4 patients: 1 patient had an atheroma in the brachial artery, 1 patient had a thrombus in the subclavian artery and 2 patients presented severe pain in the forearm. In the remaining 5 patients, there were 2 radial dissections and in 3 the RA could not be approached. Conclusions: The TRA is a safe and effective alternative to perform diagnostic cerebral angiographies with conversion rates according to the literature. The use of appropriate catheters is necessary in order to lower fluoroscopy times when this technique is chosen

Abstract Number ‐ 142: Flow Diversion for Posterior Communicating Artery Aneurysms: Systematic Review and Meta‐Analysis

Introduction Posterior communicating artery (PComA) aneurysms are common and have a high risk of rupture. Flow diverters (FD) have demonstrated a safe and effective profile. However, the use of FD in PComA aneurysms has shown controversial results with high rates of recurrence and a high risk of potential ischemic complications. There, we aimed to evaluate the safety and efficacy of flow diversion for the treatment of PComA aneurysms with a meta‐analysis of the literature. Methods We performed a systematic search in Scopus, Embase, Medline, and Web of Science from inception until May 2022 for all the studies that reported the safety and effectiveness of FD for the treatment of intracranial aneurysms located in the posterior communicating artery. The primary effectiveness endpoint was a complete aneurysm occlusion rate at final follow‐up. The primary safety endpoint was a composite measure of cumulative events that occurred during and after the procedure. Events included death and ischemic/hemorrhagic complications. Random‐effects meta‐analysis was used to calculate proportions. Statistical heterogeneity across studies was assessed with I2 statistics. Results A total of 13 studies with 397 patients harboring 403 aneurysms were included in our analysis. Mean age was 48 years and mean aneurysm size was 5.3 mm. Complete aneurysm occlusion at final follow‐up was 74% (95% CI 66–81%; I2 = 54%). The primary safety composite outcome was 5% (95% CI 3–9%; I2 = 0%). The mortality rate was 1% (95% CI 0–2%; I2 = 0%). Subgroup analysis showed that patients with a non‐fetal PComA had a higher rate (76%; 95% CI 62%‐86%; I2 = 53%) of complete aneurysm occlusion compared to those with a fetal PComA (36%; 95% CI 21%‐54%; I2 = 0%). Conclusions Our findings show that flow diversion for the treatment of aneurysms located in the PComA is effective and safe. However, the same treatment for aneurysms located in a fetal‐type PComA did not show the same efficacy profile suggesting that these cases might require an alternative treatment to achieve permanent occlusion rates