Hemophilia and non-accidental head trauma in two siblings: lessons and legal implications.

Non-accidental head trauma (NAHT) is a common cause of traumatic brain injury in childhood, origin of profound and disabling neurological sequalae, and in the most disgraceful cases, ultimately death.Subdural hematoma (SDH) is the most common intracranial finding in NAHT. On the other hand, congenital bleeding disorders are a minor but a significant cause of ICH in the neonate and toddler. Not uncommonly, intracranial bleeding is the first sign of a severe inherited coagulation disorder. In the presence of an unexpected intracranial bleeding after a minor trauma or without a clear history of the related events, physicians and caregivers may be confronted to the dilemma of a possible child abuse. It must be bear in mind that physical abuse and bleeding disorders can co-exist in the same child.We report here the case of two siblings in whom a diagnosis of hemophilia coexisted with the presumption of a non-accidental head trauma. Child abuses were inflicted in both children with a spare time of 2 years. A diagnosis of mild hemophilia was prompted in the first sibling after initial NAHT, while inflicted trauma was evident in the second sibling after neuroimaging findings and concomitant lesions. Lessons from this case in co-existing bleeding disorders and inflicted trauma and legal implications derived will be discussed thereafter.The possibility of a bleeding disorder should be considered in all children presenting with unexplained bleeding at a critical site in the setting of suspected physical maltreatment, particularly intracranial hemorrhage (ICH).

Megalencephaly-capillary malformation syndrome and associated hydrocephalus: treatment options and revision of the literature.

PURPOSE: We describe our series of 4 patients with megalencephaly-capillary malformation syndrome (MCAP) and review the literature in order to assess the optimal treatment for the associated hydrocephalus. METHODS: We review our institutional series of hydrocephalus associated with MCAP and review the literature, analyzing the causes that could originate the hydrocephalus and the different types of treatments proposed for them. RESULTS: Of our patients treated with ventriculoperitoneal (VP) shunt, one suffered a surgical revision of the shunt and died due to a cranial trauma unrelated to her syndrome or the previous shunt surgery, and the other did not undergo surgical revisions until the end of her follow-up. Our patients treated with endoscopic third ventriculostomy (ETV) have improved their symptomatology and have not suffered of any complications related to the hydrocephalus after the ETV surgery. CONCLUSIONS: We update the treatment of MCAP-associated hydrocephalus and propose ETV as a valid treatment, as it seems a safe procedure with a low rate of complications.

Gliomatosis cerebri and Rasmussen's encephalitis: Two different entities causing refractory epilepsy. Comparison through two clinical cases.

BACKGROUND AND IMPORTANCE: Rasmussen's Encephalitis (RE) is a chronic and progressive childhood disease caused by an inflammatory disorder that affects a cerebral hemisphere. On the other hand, Gliomatosis Cerebri (GC) is a rare primary neoplastic glial process with a diffuse and infiltrative growth. CLINICAL PRESENTATION: We present two clinical cases with a history of continuous focal epilepsy refractory to antiepileptic drugs. They share similar clinical and radiologic features, but a different histopathological diagnosis. A brain biopsy was needed to distinguish GC from a RE. CONCLUSION: The debut of a drug-resistant epilepsy with focal seizures and an ipsilateral progressive hemiparesis suggests the diagnosis of RE. However, there are other entities such as GC, which, despite its rarity, should be considered in the differential diagnosis. So, in some cases, histological diagnosis is needed.

Hypermanganesemia due to mutations in SLC39A14: further insights into Mn deposition in the central nervous system.

BACKGROUND: The SLC39A14, SLC30A10 and SLC39A8 are considered to be key genes involved in manganese (Mn) homeostasis in humans. Mn levels in plasma and urine are useful tools for early recognition of these disorders. We aimed to explore further biomarkers of Mn deposition in the central nervous system in two siblings presenting with acute dystonia and hypermanganesemia due to mutations in SLC39A14. These biomarkers may help clinicians to establish faster and accurate diagnosis and to monitor disease progression after chelation therapy is administered. RESULTS: A customized gene panel for movement disorders revealed a novel missense variant (c.311G > T; p.Ser104Ile) in SLC39A14 gene in two siblings presenting at the age of 10 months with acute dystonia and motor regression. Mn concentrations were analyzed using inductively coupled mass spectrometry in plasma and cerebrospinal fluid, disclosing elevated Mn levels in the index case compared to control patients. Surprisingly, Mn values were 3-fold higher in CSF than in plasma. We quantified the pallidal index, defined as the ratio between the signal intensity in the globus pallidus and the subcortical frontal white matter in axial T1-weighted MRI, and found significantly higher values in the SLC39A14 patient than in controls. These values increased over a period of 10 years, suggesting the relentless pallidal accumulation of Mn. Following genetic confirmation, a trial with the Mn chelator Na2CaEDTA led to a reduction in plasma Mn, zinc and selenium levels. However, parents reported worsening of cervical dystonia, irritability and sleep difficulties and chelation therapy was discontinued. CONCLUSIONS: Our study expands the very few descriptions of patients with SLC39A14 mutations. We report for the first time the elevation of Mn in CSF of SLC39A14 mutated patients, supporting the hypothesis that brain is an important organ of Mn deposition in SLC39A14-related disease. The pallidal index is an indirect and non-invasive method that can be used to rate disease progression on follow-up MRIs. Finally, we propose that patients with inherited defects of manganese transport should be initially treated with low doses of Na2CaEDTA followed by gradual dose escalation, together with a close monitoring of blood trace elements in order to avoid side effects.

Luxación de caderas y dolor en la atrofia muscular espinal tipo III. Casos clínicos / Hip dislocation and pain in spinal muscular atrophy type III. A report of two cases

La atrofia muscular espinal, es la segunda enfermedad neuromuscular más frecuente en la infancia. Se transmite con carácter autosómico recesivo. Se conocen tres formas clínicas, según la edad de inicio de los síntomas y la capacidad de marcha, con una gran variabilidad en el pronóstico vital y funcional. Durante su evolución aparecen distintas complicaciones del aparato locomotor, entre las que destaca la luxación de caderas, en la cual, según la mayoría de los autores no se recomienda actualmente la cirugía, debido a la alta frecuencia de recidivas. Por otro lado, se cree que la existencia de dolor asociada a la misma es poco frecuente, y tanto su aparición como el manejo del mismo, están muy poco reportados. Presentamos dos pacientes con atrofia muscular espinal III, con luxación de caderas asociada a dolor, a las que se practicó una artrocentesis más infiltración con anestésico y corticoides, consiguiéndose de esta forma el control del dolor. Creemos que es una opción terapéutica a tener en cuenta, cuando la luxación de cadera se acompaña de dolor, se objetiva derrame intraarticular y el cuadro no remite con el tratamiento farmacológico habitual (AU)

Spinal muscular atrophy is the second most frequent hereditary neuromuscular disease during childhood. Transmission is autosomal recessive. There are three clinical forms, classified according to age of symptom onset and ability to walk, with a wide range of severity. As the disorder progresses, several complications related to the locomotor apparatus may appear. Hip dislocation is a frequent manifestation associated with spinal muscular atrophy. Currently, most authors believe that surgery of the hip joint in these patients is not justified because of the high incidence of redislocations. Moreover, it is believed that hip dislocation is rarely associated with pain, with very few reports of its presence or the need for pain management. We report two patients with spinal muscular atrophy type III and hip dislocation with associated pain. The patients underwent arthrocentesis and infiltration with anaesthetic and corticoids, which achieved pain control. We believe that this is a good therapeutic option to consider when hip dislocation is associated with pain that cannot be controlled with conventional drug treatment (AU)

[Hydrocephalus due to hyperplasia of the choroid plexuses in a patient with trisomy 9 mosaicism. A real diagnostic and therapeutic challenge]. / Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomia 9. Un verdadero reto diagnostico y terapeutico.

INTRODUCTION: Trisomy 9 is an unusual chromosome abnormality in live-born patients, which is frequently accompanied by functional and structural anomalies of the central nervous system. Among many other alterations, several studies have been published in the English-speaking literature that show an association between chromosome 9 abnormality and pathologies affecting the choroid plexuses. CASE REPORT: We report the case of a 4-month-old male with trisomy 9 mosaicism associated to hydrocephalus secondary to choroid plexus hyperplasia, who was referred due to a clinical picture of intracranial hypertension. The cerebrospinal fluid (CSF) drainage procedure that was initially chosen caused massive ascites due to an excessive production of CSF, and led to a cascade of multiple surgical interventions, which included endoscopic and drainage procedures. CONCLUSIONS: This is another example of an association between choroid plexus pathologies and chromosome 9 abnormality. Due to its scarce incidence, diagnosis of hydrocephalus secondary to plexus hyperplasia is difficult, as is selecting its most suitable treatment. In this type of hydrocephalus there is a double pathophysiological mechanism, which involves an increase in CSF production and a decrease in its reabsorption. Despite taking these considerations into account, the treatment of hydrocephalus secondary to plexus hyperplasia is a real challenge that usually leads to multiple surgical interventions ranging from plexectomy or coagulation of the choroid plexuses to the implantation of CSF drainage devices.

TITLE: Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomia 9. Un verdadero reto diagnostico y terapeutico.Introduccion. La trisomia 9 es una cromosomopatia inusual en pacientes nacidos vivos, que frecuentemente se acompaña de anomalias funcionales y estructurales del sistema nervioso central. Entre otras muchas alteraciones, varios trabajos en la bibliografia anglosajona demuestran una asociacion entre cromosomopatia 9 y patologia de los plexos coroideos. Caso clinico. Varon de 4 meses de vida con mosaicismo de trisomia 9 asociado a hidrocefalia secundaria a hiperplasia de los plexos coroideos, que fue remitido por clinica de hipertension intracraneal. El procedimiento derivativo de liquido cefalorraquideo por el que optamos inicialmente provoco una ascitis masiva debida a la produccion desmesurada de liquido cefalorraquideo, y desemboco en una cascada de multiples intervenciones quirurgicas, entre las que se incluyeron procedimientos endoscopicos y derivativos. Conclusiones. Se trata de un ejemplo mas de asociacion entre patologia de los plexos coroideos y cromosomopatia 9. Debido a su escasa incidencia, es dificil establecer el diagnostico de hidrocefalia secundaria a hiperplasia de los plexos y, por tanto, el tratamiento mas adecuado. En este tipo de hidrocefalia existe un doble mecanismo fisiopatologico, que implica un aumento de produccion de liquido cefalorraquideo y una disminucion de su reabsorcion. A pesar de tener en cuenta dicha consideracion, el tratamiento de la hidrocefalia secundaria a hiperplasia de los plexos supone un verdadero reto que habitualmente pasa por multiples procedimientos quirurgicos, desde la plexectomia o coagulacion de los plexos coroideos hasta la implantacion de dispositivos de derivacion de liquido cefalorraquideo.

Outbreak of brainstem encephalitis associated with enterovirus-A71 in Catalonia, Spain (2016): a clinical observational study in a children's reference centre in Catalonia.

OBJECTIVES: To describe the characteristics of an outbreak of brainstem encephalitis and encephalomyelitis related to enterovirus (EV) infection in Catalonia (Spain), a setting in which these manifestations were uncommon. METHODS: Clinical and microbiological data were analysed from patients with neurological symptoms associated with EV detection admitted to a reference paediatric hospital between April and June 2016. RESULTS: Fifty-seven patients were included. Median age was 27.7 months (p25-p75 17.1-37.6). Forty-one (72%) were diagnosed with brainstem encephalitis, seven (12%) with aseptic meningitis, six (11%) with encephalitis, and three (5%) with encephalomyelitis (two out of three with cardiopulmonary failure). Fever, lethargy, and myoclonic jerks were the most common symptoms. Age younger than 12 months, higher white-blood-cell count, and higher procalcitonin levels were associated with cardiopulmonary failure. Using a PAN-EV real-time PCR, EV was detected in faeces and/or nasopharyngeal aspirate in all the patients, but it was found in cerebrospinal fluid only in patients with aseptic meningitis. EV was genotyped in 47 out of 57 and EV-A71 was identified in 40 out of 47, being the only EV type found in patients with brainstem symptoms. Most of the detected EV-A71 strains were subgenogroup C1. Intravenous immunoglobulins were used in 34 patients. Eight cases (14%) were admitted to the intensive care unit. All the patients but three, those with encephalomyelitis, showed a good clinical course and had no significant sequelae. No deaths occurred. CONCLUSIONS: The 2016 outbreak of brainstem encephalitis in Catalonia was associated with EV-A71 subgenogroup C1. Despite the clinical manifestations of serious disease, a favourable outcome was observed in the majority of patients.

Resultados de la colocación del estimulador del nervio vago en epilepsia y calidad de vida en un hospital pediátrico / Vagus nerve stimulator implantation for epilepsy in a paediatric hospital: outcomes and effect on quality of life

Introducción: La epilepsia es uno de los trastornos neurológicos más frecuentes de la infancia, presentándose en un 0,5-1%. Aproximadamente un 20-30% de los pacientes son farmacorresistentes. El objetivo de este trabajo es describir en 30 pacientes el impacto sobre las crisis y la calidad de vida del estimulador del nervio vago (ENV). Métodos: Se trata de un estudio descriptivo, retrospectivo, mediante revisión de las historias clínicas de todos los pacientes a quienes se les colocó el ENV entre el 2008 y 2013 en nuestro centro. La calidad de vida fue valorada mediante la escala de calidad de vida en el niño con epilepsia (CAVE), obtenida por medio de una entrevista telefónica. Resultados: Se incluyeron 19 niños (64%) y 11 niñas (36%) con una mediana de comienzo de las crisis de 21 meses (1-144 meses). La edad promedio de colocación del ENV fue de 11,89 años. El tiempo de seguimiento fue de 6-36 meses. A los 6 meses la reducción de las crisis en promedio fue del 38%, a los 12 meses del 43%, a los 24 meses del 42% y a los 36 meses del 54%. De todos los pacientes evaluados al menos un 50% se catalogaron como respondedores. Según la CAVE un 54% de las familias encontró el efecto del ENV como bueno o muy bueno y un 39% como regular. Conclusiones: El ENV es un tratamiento paliativo, generalmente bien tolerado, parcialmente efectivo para el control de la epilepsia refractaria en pediatría y con repercusiones positivas sobre la calidad de vida

Introduction: Epilepsy, which is present in 0.5% to 1% of the paediatric population, is one of the most frequent childhood neurological disorders. Approximately 20% to 30% of these cases will be drug-resistant. The objective of this study is to describe the impact of vagal nerve stimulation (VNS) on seizures and quality of life in a sample of 30 patients. Methods: Descriptive, retrospective study of all patients with a VNS device implanted between 2008 and 2013 in a single paediatric hospital, based on patients’ medical records. Quality of life was assessed using the Spanish scale for quality of life in children with epilepsy, completed by means of a telephone interview. Results: We describe a population of 19 boys (64%) and 11 girls (36%) with a mean age at seizure onset of 21 months (1-144 months). The mean age of VNS implantation was 11.89 years. Follow-up periods ranged from 6 to 36 months. Mean reduction in seizures at 6 months was 38%, with a reduction of 43% at 12 months, 42% at 24 months, and 54% at 36 months. At least half of all patients were classified as responders. According to the quality of life scale, 54% of the families rated the effect of VNS as either very good or good while 39% rated it as fair. Conclusions: VNS is a safe palliative treatment that is generally well tolerated. It is partially effective for controlling drug-resistant epilepsy and exerts a positive effect on quality of life

Tratamiento de la sialorrea en niños con patología neurológica / Drooling therapy in children with neurological disorders

Introducción. La sialorrea es la incapacidad para retener la saliva dentro de la boca y su progresión al tracto digestivo, y es un problema frecuente en pacientes pediátricos con patología neurológica, por lo que se están utilizando diferentes medidas para su tratamiento. Objetivo. Evaluar la eficacia y seguridad del trihexifenidilo, la escopolamina y la infiltración de toxina botulínica en el tratamiento del babeo en niños con patología neurológica. Pacientes y métodos. Es un estudio de tipo abierto y prospectivo. Incluye pacientes atendidos en el servicio de neurología que presentaban babeo excesivo, con repercusión en su calidad de vida, entre 2009 y 2013. Resultados. En 46 pacientes se indicó tratamiento con trihexifenidilo oral, y se obtuvo buena respuesta en 15 (32,6%), tres con efecto transitorio y el resto mantenido. Presentaron efectos secundarios tres pacientes (6,5%). De los 11 pacientes a los que se indicaron parches de escopolamina, se halló efecto beneficioso en cuatro (36,36%), uno fue retirado por falta de eficacia y seis por efectos secundarios. Veinticinco pacientes fueron infiltrados con toxina botulínica, con disminución significativa del babeo en 16 (64%) tras la primera infiltración. No observamos cambios significativos en nueve casos. Sólo uno presentó efectos secundarios (disfagia leve). Conclusiones. Por no haber una opción terapéutica totalmente eficaz para los pacientes con sialorrea, recomendamos iniciar el tratamiento con trihexifenidilo; como segunda opción, los parches de escopolamina, y como tercera opción, la toxina botulínica. La infiltración de toxina botulínica en glándulas salivales se muestra como una alternativa eficaz y segura según nuestra serie (AU)

Introduction. Drooling is the inability to retain saliva in the mouth and its progression to the digestive tract, being a common problem in pediatric patients with neurological disorders. Three different treatment options are available. Aim. To assess the effectiveness and safety of trihexyphenidyl, scopolamine and botulinum toxin infiltration in the treatment of drooling in children with neurological disorders. Patients and methods. This is an open and prospective type study. We include patients treated in the Neurology Service that present excessive drooling, affecting their quality of life, between 2009 and 2013. Results. We enrolled 46 patients in the study. The treatment with oral trihexyphenidyl was indicated in 46, obtaining good result in 15 (32.6%), three with temporary effect and the rest with lasting effect. Three patients presented side effects (6.5%). Four out of 11 (36.36%) patients treated with scopolamine patch had beneficial effects. One was withdrawn due to lack of efficacy and six due to side effects. Twenty-five patients were infiltrated with botulinum toxin, with a significant decrease of drooling in 16 patients (64%) after the first injection. We observed no significant changes in nine patients. Only one out of 25 showed side effects (mild dysphagia). Conclusions. Currently there is not a fully effective therapeutic option for drooling. We recommend starting treatment with trihexyphenidyl. A second option could be the scopolamine patch and botulinum toxin as a third option. Botulinum toxin infiltration in salivary glands is shown as an effective and safe alternative in our study (AU)

Vagus nerve stimulator implantation for epilepsy in a paediatric hospital: outcomes and effect on quality of life.

INTRODUCTION: Epilepsy, which is present in 0.5% to 1% of the paediatric population, is one of the most frequent childhood neurological disorders. Approximately 20% to 30% of these cases will be drug-resistant. The objective of this study is to describe the impact of vagal nerve stimulation (VNS) on seizures and quality of life in a sample of 30 patients. METHODS: Descriptive, retrospective study of all patients with a VNS device implanted between 2008 and 2013 in a single paediatric hospital, based on patients' medical records. Quality of life was assessed using the Spanish scale for quality of life in children with epilepsy, completed by means of a telephone interview. RESULTS: We describe a population of 19 boys (64%) and 11 girls (36%) with a mean age at seizure onset of 21 months (1-144 months). The mean age of VNS implantation was 11.89 years. Follow-up periods ranged from 6 to 36 months. Mean reduction in seizures at 6 months was 38%, with a reduction of 43% at 12 months, 42% at 24 months, and 54% at 36 months. At least half of all patients were classified as responders. According to the quality of life scale, 54% of the families rated the effect of VNS as either very good or good while 39% rated it as fair. CONCLUSIONS: VNS is a safe palliative treatment that is generally well tolerated. It is partially effective for controlling drug-resistant epilepsy and exerts a positive effect on quality of life.

Rombencefalosinapsis, una rara alteración del desarrollo del cerebelo de fácil diagnóstico / Rhombencephalosynapsis, a rare congenital abnormality easy to diagnose

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Hipoplasia adenohipofisiaria congénita aislada / Isolated congenital adenohypophyseal hypoplasia

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Mild clinical and biochemical phenotype in two patients with PMM2-CDG (congenital disorder of glycosylation Ia).

Phosphomannomutase 2 deficiency (PMM2-CDG) patients may present as mild phenotypes, with the cerebellum frequently involved. In those cases, false-negative results in screening may occur when applying conventional biochemical procedures. Our aim was to report two patients with a diagnosis of PMM2-CDG presenting with mild clinical phenotype. Patient 1-at 9 months of age, she presented with just psychomotor delay, tremor, hypotonia, and slight lipodystrophy. Patient 2-she presented at 8 months of age with psychomotor delay, hand stereotypes, hypotonia, convergent bilateral strabismus, and tremor but no lipodystrophy. Routine biochemical parameters including blood count, clotting factors, proteins, and thyroid hormone were normal in both cases. Cranial MRI evidenced mild cerebellar atrophy with moderate vermis hypoplasia. In case 1, sialotransferrin pattern showed very slightly increased disialotransferrin with no asialotransferrin, and in case 2, the transferrin pattern was impaired in the first study but nearly normal in the second. Nevertheless, in all the samples, quantification of the patterns obtained by capillary zone electrophoresis analysis gave results out of the control range. High residual PMM2 activity was observed in both cases and the genetic analysis showed that patient 1 was heterozygous for c.722G>C (p.C241S) and c.368G>A (p.R123Q) mutations, and patient 2 showed the c.722G>C and the c.470T>C (p.F157S) mutations in the PMM2 gene. We would like to stress the importance of the use of sensitive semiquantitative methods of screening for CDG in order to achieve early identification of patients with mild phenotypes. Intentional tremor was an atypical but remarkable clinical feature in both cases, and the global cerebellar atrophy with vermis hypoplasia reinforced the early clinical suspicion of a PMM2-CDG disease.

Granulosa cell tumor and endometrial cancer: a case report and review of the literature.

Granulosa cell tumors (GCTs) of the ovary are an uncommon type of ovarian cancer, representing only 2-5%. Frequently, their tumoral cells present some features of normal granulosa cells, like hormonal production. As a consequence, this neoplasia can be diagnosed either by common ovarian cancer symptoms or endometrial pathologies due to an estrogenic effect. Symptoms caused by estrogen production can also give rise to different clinical manifestations depending on whether they appear in postmenopausal or young women. In the case we present below, a patient was referred for presenting postmenopausal bleeding of one year's duration. Once endometrial cancer was diagnosed and subsequently staged, an ovarian mass was detected. We report an atypical case of ovarian cancer with the aim of reviewing the clinical features of GCT, as well as its prognosis, treatment and follow-up recommendations, according to the available literature.

Aneurisma de arteria renal diagnosticado a raíz de una emergencia hipertensiva reparado mediante colocación de Stent

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[Ischemic brain lesions following carotid revascularisation procedures: a comparative study using diffusion-weighted magnetic resonance imaging]. / Lesiones cerebrales isquémicas tras procedimientos de revascularización carotídea: estudio comparativo con resonancia magnética por difusión.

INTRODUCTION: The risk of cerebral embolism following angioplasty-stent placement (AGP) is higher than that observed after a carotid endarterectomy (CE) using transcranial Doppler ultrasonography. Nevertheless, no studies have been conducted to compare the two revascularisation procedures with the latest neuroimaging techniques. AIMS: To analyse the presence and repercussion of acute cerebral ischemia detected by diffusion-weighted magnetic resonance imaging (DMR) following carotid revascularisation procedures. PATIENTS AND METHODS: Our prospective study included a sample of 20 consecutive patients with atherosclerotic stroke and symptomatic carotid stenosis > 70% according to NASCET criteria and treated by CE (n = 10) or AGP (n = 10). Patients were submitted to a DMR during the 48 hours prior to revascularisation and another within the 72 hours following the intervention in order to evaluate the existence of new acute cerebral ischemic lesions. Patients were explored by a neurologist before and after the intervention. RESULTS: DMR allowed new areas of cerebral ischemia to be detected in 10% of the CE and in 40% of the AGP patients. 50% of the post-AGP ischemic lesions were multiple and 10% of the lesions in each therapeutic group presented some clinical correlation in the form of transient neurological focal signs. No significant differences were observed with respect to the pattern of risk of complications between the two therapeutic groups and no factors associated to a higher risk of new ischemic lesions were detected by DMR in patients submitted to AGP. CONCLUSIONS: Carotid AGP is linked to a higher frequency of new ischemic lesions in the brain than in the case of CE. Nevertheless, these ischemic lesions detected by DMR are usually silent. Symptomatic complications were similar in the two procedures.

Lesiones cerebrales isquémicas tras procedimientos de revascularización carotídea: estudio comparativo con resonancia magnética por difusión / Ischemic brain lesions following carotid revascularisation procedures: a comparative study using diffusion-weighted magnetic resonance imaging

Introducción. El riesgo de embolismo cerebral tras una angioplastia-endoprótesis (AGP) es superior al encontrado tras la endarterectomía carotídea (EC) mediante estudio Doppler transcraneal. Sin embargo, no existen estudios comparativos entre ambos procedimientos de revascularización con las técnicas recientes de neuroimagen. Objetivo. Analizar la presencia y repercusión de la isquemia cerebral aguda detectada mediante resonancia magnética por difusión (RMD) tras procedimientos de revascularización carotídea. Pacientes y métodos. Se incluyeron, de forma prospectiva, 20 pacientes consecutivos con ictus aterosclerótico y estenosis carotídea sintomática superior al 70 por ciento según los criterios NASCET y tratados con EC (n = 10) o AGP (n = 10). Se les practicó una RMD en las 48 horas previas a la revascularización y otra dentro de las 72 horas posteriores, para evaluar la existencia de nuevas lesiones isquémicas cerebrales agudas. El neurólogo exploró a los pacientes antes y después del procedimiento terapéutico. Resultados. Se detectaron nuevas áreas de isquemia cerebral con RMD en un 10 por ciento de las EC y en un 40 por ciento de las AGP. El 50 por ciento de las lesiones isquémicas tras la AGP fue múltiple. El 10 por ciento de las lesiones en cada grupo terapéutico presentó una correlación clínica en forma de focalidad neurológica transitoria. No se observaron diferencias significativas respecto al perfil de riesgo de complicaciones entre los grupos terapéuticos, ni se detectaron factores asociados a un mayor riesgo de lesiones isquémicas nuevas por RMD en los pacientes sometidos a AGP. Conclusión. La AGP carotídea se asocia a nuevas lesiones isquémicas cerebrales con más frecuencia que la EC; sin embargo, estas lesiones isquémicas detectadas mediante RMD son, habitualmente, silentes. Las complicaciones sintomáticas en ambos procedimientos fueron similares (AU)

Introduction. The risk of cerebral embolism following angioplasty-stent placement (AGP) is higher than that observed after a carotid endarterectomy (CE) using transcranial Doppler ultrasonography. Nevertheless, no studies have been conducted to compare the two revascularisation procedures with the latest neuroimaging techniques. Aims. To analyse the presence and repercussion of acute cerebral ischemia detected by diffusion-weighted magnetic resonance imaging (DMR) following carotid revascularisation procedures. Patients and methods. Our prospective study included a sample of 20 consecutive patients with atherosclerotic stroke and symptomatic carotid stenosis > 70% according to NASCET criteria and treated by CE (n = 10) or AGP (n = 10). Patients were submitted to a DMR during the 48 hours prior to revascularisation and another within the 72 hours following the intervention in order to evaluate the existence of new acute cerebral ischemic lesions. Patients were explored by a neurologist before and after the intervention. Results. DMR allowed new areas of cerebral ischemia to be detected in 10% of the CE and in 40% of the AGP patients. 50% of the post-AGP ischemic lesions were multiple and 10% of the lesions in each therapeutic group presented some clinical correlation in the form of transient neurological focal signs. No significant differences were observed with respect to the pattern of risk of complications between the two therapeutic groups and no factors associated to a higher risk of new ischemic lesions were detected by DMR in patients submitted to AGP. Conclusions. Carotid AGP is linked to a higher frequency of new ischemic lesions in the brain than in the case of CE. Nevertheless, these ischemic lesions detected by DMR are usually silent. Symptomatic complications were similar in the two procedures (AU)