The Art of Cerebral Revascularization: An Illustrative Case Series of Bypass Techniques for Complex Intracranial Aneurysms.

BACKGROUND: Complex intracranial aneurysms (CIAs) comprise a subset of lesions with defiant vascular architecture, difficult access, and prior treatment. Surgical management of CIAs is often challenging and demands an assessment on a case-by-case basis. The generational evolution of bypass surgery has offered a long-standing potential for effective cerebral revascularization. Herein, we aim to illustrate a single-center experience treating CIAs. METHODS: The authors conducted a retrospective analysis of clinical records of patients treated with cerebral revascularization techniques at Hospital Nacional Dos de Mayo, Lima, Peru, during 2018-2022. Relevant data were collected, including patient history, aneurysm features on imaging, preoperative complications, the intraoperative course, aneurysm occlusion rates, bypass patency, neurological function, and postoperative complications. RESULTS: Seventeen patients (70.59% female; median age: 53 years) with 17 CIAs (64.7% saccular; 76.5% ruptured) were included. The most common clinical presentation included loss of consciousness (70.6%) and headaches (58.8%). Microsurgical treatment included first-, second-, and third-generation bypass. In 47.1% of cases, an anastomosis between the superficial temporal artery and the M3 segment was predominantly used, followed by an A3-A3 bypass (29.4%), a superficial temporal artery-M2 bypass (17.6%), and an external carotid artery to M2 bypass (5.9%). The intraoperative aneurysm rupture rate was 11.8%. Postoperative complications included ischemia (40%), cerebrospinal fluid fistulas (26.7%), and pneumonia (20%). At hospital discharge, the median Glasgow Coma Scale score was 14 (range: 10-15). At the 6-month follow-up, 82.4% of patients had a modified Rankin Scale score ≤2, bypass patency was present in all cases, and the morbidity rate was 17.6%. CONCLUSIONS: CIAs represent a spectrum of defiant vascular lesions with a poor natural history. Bypass surgery offers the potential for definitive treatment. Our case series illustrated the predominant role of cerebral revascularization of CIAs with a critical case-by-case approach to provide optimal outcomes in a limited-resource setting.

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

Introduction: Our objective was to identify recent CPGs for the diagnosis and management of DMD and summarize their characteristics and reliability. Methods: We 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. Results: We 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. Discussion: There 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.

Diagnosis of Guillain-Barré syndrome and use of Brighton criteria in Peruvian hospitals.

BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disease of the peripheral nervous system that caused multiple epidemiological outbreaks in Peru during 2018 and 2019. It is usually diagnosed using the Brighton criteria (BC). OBJECTIVE: We aimed to determine the performance of Peruvian neurologists in diagnosing GBS based on the BC, along with its associated factors. METHODS: This was a retrospective multicenter cohort study. We included patients diagnosed with GBS between 2007 and 2018 in three public hospitals in Lima, Peru. We collected data regarding demographic, clinical and management characteristics. We evaluated the use of the BC for confirmatory diagnosis of GBS and developed a logistic regression model to identify factors associated with its use. RESULTS: Out of 328 cases, we reviewed 201 available charts. The median age was 48 years, with male predominance. Over half of the patients presented an inadequate motor examination according to their Medical Research Council (MRC) score. Additional testing included lumbar puncture and electrophysiological testing, in over 70% of the cases. The BC showed certainty level 1 in 13.4% and levels 2 and 3 in 18.3%. Neither the quality of the motor examination nor the type of institution showed any association with the BC. CONCLUSIONS: Level 1 diagnostic certainty of the BC was met in less than one quarter of the cases with a GBS diagnosis in three centers in Lima, Peru, between 2007 and 2018. This level was not significantly associated with being treated in a specialized institute, rather than in a general hospital.

Diagnosis of Guillain-Barré syndrome and use of Brighton criteria in Peruvian hospitals / Diagnóstico del Síndrome de Guillain-Barré y uso de los criterios de Brighton en hospitales Peruanos

ABSTRACT Background: Guillain-Barré syndrome (GBS) is an autoimmune disease of the peripheral nervous system that caused multiple epidemiological outbreaks in Peru during 2018 and 2019. It is usually diagnosed using the Brighton criteria (BC). Objective: We aimed to determine the performance of Peruvian neurologists in diagnosing GBS based on the BC, along with its associated factors. Methods: This was a retrospective multicenter cohort study. We included patients diagnosed with GBS between 2007 and 2018 in three public hospitals in Lima, Peru. We collected data regarding demographic, clinical and management characteristics. We evaluated the use of the BC for confirmatory diagnosis of GBS and developed a logistic regression model to identify factors associated with its use. Results: Out of 328 cases, we reviewed 201 available charts. The median age was 48 years, with male predominance. Over half of the patients presented an inadequate motor examination according to their Medical Research Council (MRC) score. Additional testing included lumbar puncture and electrophysiological testing, in over 70% of the cases. The BC showed certainty level 1 in 13.4% and levels 2 and 3 in 18.3%. Neither the quality of the motor examination nor the type of institution showed any association with the BC. Conclusions: Level 1 diagnostic certainty of the BC was met in less than one quarter of the cases with a GBS diagnosis in three centers in Lima, Peru, between 2007 and 2018. This level was not significantly associated with being treated in a specialized institute, rather than in a general hospital.

Resumen Antecedentes: El Síndrome de Guillain-Barré (SGB) es una enfermedad autoinmune del sistema nervioso periférico, causante de brotes epidemiológicos en Perú entre el 2018 y el 2019. El diagnóstico se realiza a través de los Criterios de Brighton (CB). Objetivo: Determinar el desempeño de neurólogos peruanos en diagnosticar SGB basándose en los CB, así como factores asociados. Métodos: Cohorte retrospectiva multicéntrica. Incluimos pacientes diagnosticados con SGB del 2007-2018 en 3 hospitales públicos en Lima, Perú. Recolectamos sus características demográficas, clínicas y de manejo. Evaluamos el uso de los CB para el diagnostico de SGB y empleamos un modelo de regresión logística para identificar los factores asociados con su uso. Resultados: De 328 casos, revisamos 201 historias disponibles. La edad mediana fue 48 años, con predominancia masculina. Mas del 50% de pacientes presento un examen motor inadecuado acorde con el puntaje MRC. Se realizaron exámenes auxiliares como punción lumbar y estudios electrofisiológicos en mas del 70% de pacientes. Se obtuvo un nivel de certeza 1 para los CB en un 13.4% de casos , y un nivel 2 o 3 en un 18.3%. El nivel no estuvo asociado con la calidad del examen motor ni el tipo de institución de atención. Conclusiones: Un diagnostico nivel 1 de certeza acorde con los BC se obtuvó en menos de un cuarto de casos diagnosticados como SGB. Este nivel no estuvo asociado con la atención en una institución especializada, comparado con un hospital general.

Agenesis of the internal carotid artery associated with intracranial aneurysms: Endovascular treatment and intraoperative complications management.

Agenesis of the internal carotid artery (ICA) is a rare condition, whereas the association of this condition and the presence of intracranial aneurysms is higher in comparison to patients with normal vasculature. Endovascular treatment of this particular subgroup of patients has been described, but complications associated during treatment have not. Herein, we describe the endovascular treatment of intracranial aneurysms in two cases complicated with coil protrusion and in-stent thrombosis in the setting of agenesis of the ICA which were treated successfully without clinical compromise. One-year follow-up digital subtraction angiography demonstrated the complete obliteration of both aneurysms. In most of the cases, coil protrusion does not require further intervention; nevertheless, in the setting of ICA agenesis, stent placement is a potential alternative. In-stent thrombosis should be treated promptly with the available tools, in our case intra-arterial Alteplase reperfused the parent vessel.

Predictores de mortalidad intrahospitalaria y de mal pronóstico funcional en pacientes sometidos a evacuación quirúrgica de hemorragia intracerebral / Predictors of in-hospital mortality and poor functional outcomes in patients who underwent surgical evacuation of intracerebral hemorrhage

RESUMEN Objetivo : Determinar predictores de mortalidad intrahospitalaria y mal pronóstico funcional en pacientes sometidos a cirugía por hemorragia intracerebral. Materiales y métodos : Se analizaron las historias clínicas, reportes operatorios y tomografías cerebrales de pacientes con hemorragia intracerebral desde marzo 2018 hasta marzo de 2020. Se realizó un análisis de regresión logística univariado y multivariado para determinar predictores independientes de mortalidad intrahospitalaria y mal pronóstico funcional al alta. Resultados : La mortalidad intrahospitalaria fue de 33,7 % (n = 31 pacientes). Predictores independientes de mortalidad fueron el sexo femenino (OR = 3.01; p = 0.031) y un Glasgow < 8 puntos al ingreso (OR = 3.19; p = 0.031). Un mal pronóstico funcional luego de la intervención se encontró en 77 pacientes (83,7 %). Predictores independientes de mal pronóstico funcional fueron una Escala de Rankin modificada > 3 (OR = 15.5; p = 0.01) y déficit motor pre-operatorio (OR = 8.95; p = 0.042). Conclusiones : En pacientes con diagnóstico de hemorragia intracerebral tratados con cirugía se encontró una alta mortalidad y morbilidad. El sexo femenino y factores clínicos como el estado de conciencia y el estado funcional al ingreso fueron predictores independientes de mortalidad intrahospitalaria y mal pronóstico funcional.

ABSTRACT Objective : To determine predictors of in-hospital mortality and poor functional prognosis in patients undergoing surgery for intracerebral hemorrhage. Materials and Methods : Clinical records, operative reports, and cerebral CT scans of patients with intracerebral hemorrhage from March 2018 to March 2020 were analyzed. Univariate and multivariate logistic regression analyses were performed for determining independent predictors of in-hospital mortality and poor functional prognosis at discharge. Results : In-hospital mortality was 33.7% (n= 31 patients). Independent predictors for mortality were female sex (OR= 3.01, p= 0.031) and Glasgow score <8 on admission (OR= 3.19, p= 0.031). A poor functional prognosis after the intervention was found in 77 patients (83.7%). Independent risk factors for a poor functional prognosis were score >3 in the modified Rankin scale (OR= 15.5; p= 0.01), and preoperative motor deficit (OR= 8.95; p= 0.042). Conclusions : In patients with intracerebral hemorrhage who were surgically treated, high morbidity and mortality rates were found. Female sex and clinical factors, such as consciousness condition and functional status on admission were independent predictors for in-hospital mortality and poor functional prognosis.

Validation of the ICH score and ICH-GS in a Peruvian surgical cohort: a retrospective study.

The intracerebral hemorrhage (ICH) score and the ICH-grading scale (ICH-GS) are mortality predictor tools developed predominantly in conservatively treated ICH cohorts. We aimed to compare and evaluate the external validity of both models in predicting mortality in patients with ICH undergoing surgical intervention. A retrospective review of all patients presenting with spontaneous ICH admitted to a Peruvian national hospital between January 2018 and March 2020 was conducted. We compared the area under the receiver operating characteristic curve (AUC) for the ICH score and ICH-GS for in-hospital, 30-day, and 6-month mortality prediction. The research protocol was approved by the Institutional Review Board. A total of 73 patients (median age 62 years, 56.2% males) were included in the study. The mean ICH and ICH-GS scores were 2.5 and 8.7, respectively. In-hospital, 30-day, and 6-month mortality were 37%, 27.4%, and 37%, respectively. The AUC for in-hospital, 30-day, and 6-month mortality was 0.69, 0.71, and 0.69, respectively, for the ICH score and 0.64, 0.65, and 0.68, respectively, for the ICH-GS score. In this study, the ICH score and ICH-GS had moderate discrimination capacities to predict in-hospital, 30-day, and 6-month mortality in surgically treated patients. Additional studies should assess whether surgical intervention affects the discrimination of these prognostic models in order to develop predictive scores based on specific populations.

Single-center experience with endovascular treatment of cerebral arteriovenous malformations with intent to cure in pediatric patients.

PURPOSE: This study aimed to report the incidence of technical complications and immediate complete angiographic occlusion, identify associated factors with failure of complete occlusion and identify predictors of technical complications in a single-center experience of pediatric arteriovenous malformations (AVM) treated with endovascular treatment with intent to cure. METHODS: Patients between 1 and 18 years of age undergoing endovascular embolization between 2011 and 2020 were included. RESULTS: A total of 120 embolizations were performed in 69 patients. The most frequent clinical presentation was intracerebral hemorrhage (76.8%). Immediate obliteration of the malformations was achieved in 40 (58%) cases. The technical complication rate was 15%. AVM nidus size between 3 and 6 cm (OR: 3.91; 95% CI 1.1-13.85; p = 0.035) and the presence of multiple feeders (OR: 5.08; 95% CI 1.41-18.28; p = 0.074) were predictive of failure of immediate complete occlusion. The location of the temporal lobe (OR: 7.83; p = 0.048), deep venous drainage (OR: 4.67; p = 0.112), and the presence of an intranidal aneurysm (OR: 3.58; p = 0.134) were predictors of technical complications. CONCLUSIONS: Embolization of pediatric AVMs with intent to cure shows a high rate of technical complications and acceptable immediate occlusion rates. Nidus size and the presence of multiple feeders were predictive of failure of complete occlusion, while temporal lobe location, deep venous drainage, and the presence of an intranidal aneurysm were predictors of technical complications. Further studies are needed to determine the best therapeutic approach in the pediatric population.

Diagnostic accuracy of brief cognitive screening tools to diagnose vascular cognitive impairment in Peru.

INTRODUCTION: To evaluate the diagnostic accuracy of three brief cognitive screening (BCS) tools, Peruvian version of Addenbrooke's Cognitive Examination (ACE-Pe), of INECO Frontal Screening (IFS-Pe) and of the Mini-Mental State Examination (MMSE-Pe), for the diagnosis of vascular cognitive impairment (VCI) and its non-dementia stages (VCI-ND) and vascular dementia (VD) in patients with cerebral stroke in Lima-Peru. MATERIALS AND METHODS: A cohort analysis to evaluate the diagnostic accuracy of three BCS for VCI. RESULTS: Two hundred and four patients were evaluated: 61% Non-VCI, 30% VCI-ND and 9% VD. To discriminate patients with VCI from controls, the area under the curve (AUC) of ACE-Pe, IFS-Pe and MMs-Pe were 0.99 (95% confidence interval [CI] 0.98-0.99), 0.99 (95%CI 0.98-0.99) and 0.87 (95%CI 0.82-0.92), respectively. Of the three BCS, the IFS-Pe presented a larger AUC to discriminate VCI-ND from VD (AUC = 0.98 [95%CI 0.95-1]) compared to ACE-Pe (AUC = 0.84 [95%CI 0.74-0.95]) and MMSE-Pe (0.92 [95%CI 0.86-0.99]). The IFS-Pe presented a higher sensitivity (S), specificity (Sp), and positive (+LR) and negative likelihood ratios (-LR) (S = 96.72%, Sp = 89.47%, +LR = 9.1 and -LR = 0.03) than ACE-Pe (S = 96.72%, Sp = 63.16%, +LR = 2.62 and -LR = 0.05) and MMSE-Pe (S = 90.16%, Sp = 78.95%, +LR = 4.28 and -LR = 0.12). In the multiple regression analysis, the IFS-Pe was not affected by age, sex or years of schooling. CONCLUSION: The IFS-Pe has the best diagnostic accuracy for detecting VCI and discriminating between pre-dementia (VCI-ND) and dementia (VD) stages.

Costs Related to Frontotemporal Dementia in Latin America: A Scoping Review of Economic Health Studies.

Introduction: Frontotemporal dementia (FTD) is a complex syndrome characterized by changes in behavior, language, executive control, and motor symptoms. Its annual economic burden per patient in developed countries has been classified as considerable, amounting to US$119,654 per patient, almost double the patient costs reported for Alzheimer's disease. However, there is little information regarding cost-of-illness (COI) for FTD in Latin-America (LA). Aim: To describe the costs related to FTD in LA. Methods: We included COI studies on FTD conducted in LA published in English, Spanish, or Portuguese from inception to September 2020. We carried out a systematic search in Pubmed/Medline, Scopus, Web of Science, Scielo, Cochrane, and gray literature. For quality assessment, we used a COI assessment tool available in the literature. All costs were reported in USD for 1 year and adjusted for inflation. Results: We included three studies from Argentina, Brazil, and Peru. Direct costs (DCs) included medication (from US$959.20 to US$ 4,279.20), health care costs (from US$ 2,275.80 to US$7,856.16), and caregiver costs (from US$9,634.00 to US$28,730.28). Indirect costs (ICs) amounted to US$43,076.88. Conclusions: In LA countries, the reporting of costs related to FTD continues to be oriented toward DCs. They remain lower than in developed countries, possibly due to the limited health budget allocated. Only one Brazilian report analyzed ICs, representing the highest percentage of the total costs. Therefore, studies on the COI of this disease in LA are essential, focusing on both out-of-pocket spending and the potential economic loss to patients' homes and families.

Endovascular Treatment of an Arteriovenous Malformation Associated with a Double Origin of the Posterior Inferior Cerebellar Artery.

INTRODUCTION: A double origin of the posterior inferior cerebellar artery (DOPICA) is a rare anatomical variant. Posterior fossa arteriovenous malformations (AVMs), especially cerebellar AVMs, are also not common. Consequently, the association of a DOPICA with a cerebellar AVM is even rare. CASE PRESENTATION: We present a rare case of a pediatric cerebellar AVM supplied by a branch of a DOPICA which was treated endovascularly with NBCA. Total obliteration was achieved in the immediate controls and at 1-year follow-up. CONCLUSION: Navigation through tortuous and long branches from a DOPICA is technically feasible. Although NBCA cure rates are relatively low, when the microcatheter can no longer navigate through the feeding artery, a correct dilution of NBCA with lipiodol can provide adequate penetration of this embolic agent, to obliterate the AVM nidus completely.

Contralateral catheterization of the ophthalmic artery to deliver intra-arterial chemotherapy in retinoblastoma.

Retinoblastoma is the most frequent ocular malignancy in the pediatric population and intra-arterial chemotherapy has emerged as the first-line treatment of this entity with cure rates ranging from 33-100%, depending on the severity of the disease. We present the case of an advanced retinoblastoma in a pediatric patient who underwent intra-arterial chemotherapy through a contralateral route due to unsuccessful catheterization of the ophthalmic artery. The patient was diagnosed with a class D retinoblastoma which underwent the catheterization of the ophthalmic artery through the contralateral internal carotid and through the anterior communicating artery. In this case, intra-arterial chemotherapy administration was successfully delivered without complications. Contralateral routes for intra-arterial chemotherapy are safe and allow adequate penetration of the chemotherapeutic drugs in cases where a well-developed anterior communicating artery is present.

Predicting Mechanical Ventilation Using the EGRIS in Guillain-Barré Syndrome in a Latin American Country.

BACKGROUND: Up to one fifth of patients with Guillain-Barré syndrome (GBS) require mechanical ventilation (MV). The Erasmus GBS Respiratory Insufficiency Score (EGRIS) is a clinical predictive model developed in Europe to predict MV requirements among patients with GBS. However, there are significant differences between the Latin American and European population, especially in the distribution of GBS subtypes. Therefore, determining if the EGRIS is able to predict MV in a Latin American population is of clinical significance. METHODS: We retrospectively analyzed clinical and laboratory data of 177 patients with GBS in three Peruvian hospitals. We performed a multivariate logistic regression of the factors making up the EGRIS. Finally, we evaluated the EGRIS discrimination through a receiver operating characteristic curve and determined its calibration through a calibration curve and a Hosmer-Lemeshow test, a test used to determine the goodness of fit. RESULTS: We found that 14.1% of our patients required MV. One predictive factor of a patient's need for early MV was the number of days between the onset of motor symptoms and hospitalization. The Medical Research Council sum score did not alter the likelihood of early MV. Bulbar weakness increased the likelihood without showing statistical significance. In contrast, facial weakness was a protective factor of it. The EGRIS was significantly higher in patients who required early MV than in those who did not (P = 0.018). It showed an area under the curve (AUC) of 0.63, with an insignificant Hosmer-Lemeshow test result. CONCLUSIONS: Although the EGRIS was higher in patients who required early MV than in those who did not, it only showed a moderate discrimination capacity (AUC = 0.63). Facial weakness, an item of the EGRIS, was not found to be a predictive factor in our population. We suggest assessing whether these findings are due to subtype predominance and whether a modified version of the EGRIS could improve performance.

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

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.

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 Peru. 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.