Accidentes cerebrovasculares prenatales diagnosticados en la etapa de lactante: serie de 10 pacientes / Prenatal cerebrovascular accidents diagnosed in the early infant stage: a series of 10 patients

Introducción. El accidente cerebrovascular (ACV) fetal o prenatal se define como un suceso isquémico, trombótico o hemorrágico arterial o venoso que acontece entre las 14 semanas de gestación y el inicio del parto. Pacientes y métodos. Estudio retrospectivo de una serie de 10 pacientes afectos de un ictus, presumiblemente fetal, desapercibido durante el embarazo y diagnosticado en la etapa de lactante; se destacan cuáles han sido los síntomas y la edad en que se han identificado. Resultados. De los 10 pacientes estudiados, ninguno presentaba antecedentes maternos relevantes, pero se detectaron cuatro amenazas de parto pretérmino que se resolvieron con las medidas habituales y sin hallazgos de alteración fetal posterior. Entre el segundo y tercer trimestre de vida es cuando se han realizado los estudios que han llevado al diagnóstico, y se ha indicado por la familia una menor movilidad de un hemicuerpo respecto al contralateral como motivo de consulta. Dos pacientes presentaban una trombofilia. Con una media de seguimiento de seis años, todos asocian una parálisis cerebral infantil, la tercera parte una epilepsia y el 75% tiene dificultades de aprendizaje o discapacidad intelectual. Conclusión. Cuando los ACV no se detectan prenatalmente, es importante que en la atención primaria se busquen y detecten los signos de alarma del desarrollo psicomotor del lactante de forma precoz para iniciar su estudio y procurar una rehabilitación lo más pronto posible (AU)

Introduction. A foetal or prenatal cerebrovascular accident (CVA) is defined as an ischaemic, thrombotic or arterial or venous haemorrhagic event that occurs between the 14th week of gestation and the onset of labour. Patients and methods. We report a retrospective study of a series of 10 patients suffering from a, presumably foetal, stroke that went unnoticed during the pregnancy and was diagnosed in the early infant stage. The symptoms and the age at which they were identified are highlighted. Results. None of the 10 patients studied presented any relevant events in the mothers’ medical history, but there were four threats of a preterm birth that were solved using the usual means and without the occurrence of any alterations that later affected the foetus. The studies that led to the diagnosis were carried out between the sixth and ninth months of life, and the reason for visiting was reported by the family as being a lower degree of mobility on one side of the body with respect to the other. Two patients presented thrombophilia. With a mean follow-up time of six years, all the patients have an associated infantile cerebral palsy, a third of them have epilepsy and 75% have learning difficulties or intellectual disability. Conclusions. When CVA are not detected in the prenatal period, it is important in primary care to look for and detect the warning signs of the psychomotor development of the infant at an early stage in order to begin a study of the case and to undertake rehabilitation as early as possible (AU)

Cerebral palsy in a term population: risk factors and neuroimaging findings.

OBJECTIVE: The purpose of this work was to study risk factors and neuroimaging characteristics of cerebral palsy in term and near-term infants. PATIENTS AND METHODS: Among a cohort of 334,339 infants > or = 36 weeks' gestation born at Kaiser Permanente Medical Care Program in northern California in 1991-2003, we identified infants with cerebral palsy and obtained clinical data from electronic and medical charts. Risk factors for cerebral palsy among infants with different brain abnormalities were compared using polytomous logistic regression. RESULTS: Of 377 infants with cerebral palsy (prevalence: 1.1 per 1000), 273 (72%) received a head computed tomography or MRI. Abnormalities included focal arterial infarction (22%), brain malformation (14%), and periventricular white matter abnormalities (12%). Independent risk factors for cerebral palsy were maternal age > 35, black race, and intrauterine growth restriction. Intrauterine growth restriction was more strongly associated with periventricular white matter injury than with other neuroimaging findings. Nighttime delivery was associated with cerebral palsy accompanied by generalized brain atrophy but not with cerebral palsy accompanied by other brain lesions. CONCLUSIONS: Cerebral palsy is a heterogeneous syndrome with focal arterial infarction and brain malformation representing the most common neuroimaging abnormalities in term and near-term infants. Risk factors for cerebral palsy differ depending on the type of underlying brain abnormality.

Unique anomalous origin of the left anterior cerebral artery.

We present the case of an anomalous origin of the left anterior cerebral artery (ACA) from the supraclinoid segment of the right internal carotid artery. Because of improved imaging quality, anomalies of the ACA-anterior communicating artery (AComA) complex are increasingly recognized on transaxial images. Although most of these anomalies are incidental findings of little clinical significance, some ACA-AComA complex anomalies are clinically significant. Recognition of these anomalies may be instrumental in developing a differential diagnosis or for improved surgical planning.

Major brain lesions detected on sonographic screening of apparently normal term neonates.

Major congenital or acquired structural abnormalities of the brain, with significant prognostic implications have rarely been emphasised in apparently normal infants. Our purpose was to investigate the prevalence and types of major brain lesions in clinically normal term neonates using sonography. From January 1999 to December 2001, we examined 2309 clinically normal term neonates within 3 days of birth. Of these, six (0.26%) had major brain lesions, including three cases of intracerebral haemorrhage (two grade II intraventricular and one temporal lobe haemorrhage), two of corpus callosum agenesis and one lacunar infarct in the territory of the left middle cerebral artery. One child was lost to follow-up, because of adoption, and four had borderline to significant developmental delay at a mean age of 24 months (range 12-36 months). Although the prevalence of major brain lesions was low, these infants have a higher risk of later neurodevelopmental disability, despite an early asymptomatic period. Universal neonatal brain ultrasound screening may help early diagnosis of rare major lesions, some of which have prognostic implications.

Combination of event-related fMRI and diffusion tensor imaging in an infant with perinatal stroke.

Focal ischemic brain injury, or stroke, is an important cause of later handicap in children. Early assessment of structure-function relationships after such injury will provide insight into clinico-anatomic correlation and potentially guide early intervention strategies. We used combined functional MRI (fMRI) with diffusion tensor imaging (DTI) in a 3-month-old infant to explore the structure-function relationship after unilateral perinatal stroke that involved the visual pathways. With visual stimuli, fMRI showed a negative BOLD activation in the visual cortex of the intact right hemisphere, principally in the anterior part, and no activation in the injured hemisphere. The functional activation in the intact hemisphere correlated clearly with the fiber tract of the optic radiation visualized with DTI. DTI confirmed the absence of the optic radiation in the damaged left hemisphere. In addition, event-related fMRI (ER-fMRI) experiments were performed to define the characteristics of the BOLD response. The shape is that of an inverted gamma function (similar to a negative mirror image of the known positive adult BOLD response). The maximum decrease was reached at 5-7 s with signal changes of -1.7 +/- 0.4%.Thus, this report describes for the first time the combined use of DTI and event-related fMRI in an infant and provides insight into the localization of the fMRI visual response in the young infant and the characteristics of the BOLD response.

General movements detect early signs of hemiplegia in term infants with neonatal cerebral infarction.

Background. Studies have reported that infants with hemiplegia of congenital origin may have a period between birth and up to 12 months when clinical signs of hemiplegia are not evident. The aim of this study was to establish whether the assessment of general movements (GMs) may help in the earlier detection of signs of hemiplegia. Subjects and Methods. Eleven infants with cerebral infarction on brain MRI, and eleven normal controls were enrolled in the study. Quality of GMs was assessed from videotapes between 3 and 6 weeks and between 9 and 16 weeks. Neurological outcome was evaluated at least at two years. Results. Seven of the 11 infants had an assessment performed between 3 and 6 weeks: abnormal GMs were observed in all the infants who developed hemiplegia, but one child had abnormal GMs and a normal outcome. All 11 infants had a scorable assessment between 9 and 16 weeks. In all a specific type of GMs, fidgety movements (FMs), were predictive of neurological outcome. The presence of early asymmetries at both 3 - 6 and 9 - 16 weeks was also significantly associated with later signs of hemiplegia. Conclusions. The assessment of GMs after the neonatal period appears to be very useful in the early identification of hemiplegia in infants with cerebral infarction. Whilst the prediction of hemiplegia should be possible from early neonatal MRI brain scans, this facility is not always available. Observation of GMs is a bedside clinical approach that allows confirmation of early prediction from MRI, early rehabilitation if needed and reassurance that neurological outcome will be good where that is appropriate.

Origin and timing of brain lesions in term infants with neonatal encephalopathy.

BACKGROUND: The role of intrapartum asphyxia in neonatal encephalopathy and seizures in term infants is not clear, and antenatal factors are being implicated in the causal pathway for these disorders. However, there is no evidence that brain damage occurs before birth. We aimed to test the hypothesis that neonatal encephalopathy, early neonatal seizures, or both result from early antenatal insults. METHODS: We used brain MRI or post-mortem examination in 351 fullterm infants with neonatal encephalopathy, early seizures, or both to distinguish between lesions acquired antenatally and those that developed in the intrapartum and early post-partum period. We excluded infants with major congenital malformations or obvious chromosomal disorders. Infants were divided into two groups: those with neonatal encephalopathy (with or without seizures), and evidence of perinatal asphyxia (group 1); and those without other evidence of encephalopathy, but who presented with seizures within 3 days of birth (group 2). FINDINGS: Brain images showed evidence of an acute insult without established injury or atrophy in 197 (80%) of infants in group 1, MRI showed evidence of established injury in only 2 infants (<1%), although tiny foci of established white matter gliosis, in addition to acute injury, were seen in three of 21 on post-mortem examination. In group 2, acute focal damage was noted in 62 (69%) of infants. Two (3%) also had evidence of antenatal injury. INTERPRETATION: Although our results cannot exclude the possibility that antenatal or genetic factors might predispose some infants to perinatal brain injury, our data strongly suggest that events in the immediate perinatal period are most important in neonatal brain injury.

[Bilateral hypoxic-ischaemic thalamic lesions in newborns]. / Bilaterale hypoxisch-ischämische Thalamusläsionen im Neugeborenenalter.

Bilateral hypoxic-ischaemic thalamic lesions are a rare type of neonatal encephalopathy with characteristic but often misinterpreted clinical features. This article describes history, clinical and diagnostic findings in a preterm and a term infant with hypoxic-ischaemic thalamic lesions after severe and abrupt intrauterine hypoxia. Both neonates presented with absent suckling and swallowing whereas other cranial nerves were unaffected. Characteristic findings in both newborns were profound muscular hypotonia and weak facial expressions together with feeding difficulties and frequent episodes of aspiration pneumonias. The term infant died at the age of 14 weeks following presumed aspiration pneumonia, the preterm infant, however, has developed severe extrapyramidal cerebral palsy. Absent suckling and swallowing with otherwise normal cranial nerve function appears to be characteristic of a bilateral hypoxic-ischaemic lesion of the thalamus. Animal experiments suggest that these lesions originate from a short but total ischaemia as it can be observed in cord prolapse or total placental abruption. Specific findings may initially be absent on ultrasound examination. Hence, magnetic resonance imaging of the brain should be performed on all patients with characteristic clinical symptoms even if the ultrasound scan is unremarkable. Evidence of bilateral ischaemic lesions of the thalamus usually indicates a poor prognosis.

Diffusion imaging in neonates.

Diffusion imaging is a useful technique for the evaluation of many normal and pathologic processes occurring in the neonate and often provides complementary information for conventional MR and other imaging techniques.

Cerebral infarctions in the fetus and neonate: maternal-placental-fetal considerations.

Historical data, clinical examination findings, and laboratory information must be integrated along a variable timeline that includes antepartum, intrapartum, and postnatal time periods when cerebral infarction can occur, in the context of the neonates genetic endowment. Genetic susceptibility or prenatal acquired vulnerabilities regarding stroke syndromes may set in motion a cascade of molecular pathways that ultimately cause or exacerbate brain injury when the vulnerable child experiences adverse medical conditions. The clinician must consider maternal, placental, and fetal conditions on which a stroke syndrome may be superimposed, with or without additional brain injury from other pathogenic mechanisms. Evaluation of fetal and neonatal cerebral infarction requires knowledge of mechanisms of brain injury that cross medical disciplines and may involve consultation with maternal/fetal specialists, placental and pediatric pathologists, neonatologists, geneticists, and other pediatric subspecialties. Comprehensive evaluations of survivors of cerebral infarction are needed to better understand structural and functional plasticity of the developing brain after a cerebrovascular event in the fetal and neonatal periods.

Advances in postnatal neuroimaging: relevance to pathogenesis and treatment of brain injury.

The human brain is susceptible to a wide variety of insults. The permanent residua of these abnormalities are represented in dysfunction of one or more areas of neurodevelopment. A full understanding of normal brain development, mechanisms of brain injury, and consequences for subsequent brain development is required to determine which infants are at risk for neurodevelopmental handicap, and to monitor the effects of new treatments and management regimens designed to prevent these disabilities. Advanced magnetic resonance techniques, such as quantitative morphometric magnetic resonance techniques, diffusion-weighted magnetic resonance techniques, and magnetic resonance spectroscopy applied to the study of early human brain development have given us a better understanding of the pathophysiologic mechanisms of brain injury and its effects on subsequent brain development. Magnetic resonance imaging has provided an invaluable tool for the study of the fetal and newborn brain in vivo.

Parenchymal brain injury in the preterm infant: comparison of cranial ultrasound, MRI and neurodevelopmental outcome.

AIM: Magnetic resonance imaging (MRI) is increasingly being used in high-risk preterm neonates. Cranial ultrasound (US) was compared with MRI in preterm patients with parenchymal injury and related to neurodevelopmental outcome. PATIENTS AND METHODS: Studies were performed in 61 patients. Twelve infants with normal US (Group 1) had an MRI within the first 4 weeks of life (early MRI), and 10 also at term age (late MRI). Eight out of 20 infants with intraventricular haemorrhage with parenchymal involvement (IVH + PI) (Group 2) had an early as well as a late MRI and 12 a late MRI. Of the 20 patients with cystic-periventricular leukomalacia (c-PVL) (Group 3), 7 had an early MRI, 1 had an MRI on both occasions and 12 had a late MRI. All 9 children with focal infarction (FI) (Group 4) had a late MRI. RESULTS: MRI was conform with cranial US in Group 1. Early MRI in Group 2 showed contralateral c-PVL in one infant and an additional contralateral occipital parenchymal haemorrhage and blood in the posterior fossa in another infant. Late MRI showed an asymmetrical posterior limb of the internal capsule (PLIC) (n=6), which predicted later hemiplegia. Early MRI in Group 3 showed more cysts (n = 5), punctate white matter lesions (n = 6), lesions in the basal ganglia (n = 1) and once involvement of the cerebellum. Late MRI showed involvement of the centrum semiovale (n = 2) lesions in the basal ganglia (n = 2) and bilateral abnormal signal intensity of the PLIC in 7 infants who all went on to develop cerebral palsy. In Group 4 MRI showed signal intensity changes suggestive of cystic lesions compared to persisting echogenicity on US (n = 3) and an asymmetrical PLIC (n = 5), which predicted hemiplegia in 4. CONCLUSION: Early MRI especially provided additional information in those with c-PVL. MRI at term age could assess the PLIC, which was useful in children with unilateral parenchymal involvement, for prediction of subsequent hemiplegia and, to a lesser degree, in bilateral c-PVL for prediction of diplegia or quadriplegia.

Clinical manifestations of cerebrovascular hypoplasias in childhood.

The aim of the study was to analyze cerebrovascular hypoplasia in childhood and its clinical manifestations in a clinical population of 205 children aged 3 to 14 years. Cerebrovascular hypoplasias were diagnosed using angiography (n=63), magnetic resonance angiography, and transcranial Doppler ultrasonography. Hypoplasias were localized in the internal carotid artery in 41.9% of patients, in the middle cerebral artery in 54.1%, the anterior cerebral artery in 1.0%, and in the vertebro-basilar system in 3.0%. Clinical manifestations included transient ischemic attacks (21% of patients), cerebral infarcts (17%), progressive unilateral cerebral hemisphere atrophy (1.0%), focal and secondary generalized epileptic seizures (56.1%), and migraine-like headache (4.9%). Hypoplasias of the internal carotid artery and middle cerebral artery manifested as focal and secondary generalized epileptic seizures, transient ischemic attacks, cerebral infarcts, migraine-like headache, and progressive unilateral cerebral hemisphere atrophy, in descending order of frequency. Hypoplasias in the anterior cerebral artery or the basilar artery caused cerebral infarcts, and hypoplasias in the vertebral arteries caused transient ischemic attacks. This article discusses the pathophysiology of ischemia in the territory of the hypoplastic cerebral artery in childhood, as well as possibilities for noninvasive neuroimaging for diagnosis of cerebrovascular hypoplasias.

Neonatal cerebral infarction: an under recognised/unreconised cause of neonatal seizures?

Neonatal cerebral infarction or neonatal stroke is a well recognised cause of neonatal seizures. The awareness of its existence among healthcare workers is low and it is not even mentioned as a cause of neonatal seizures in most books. Many of the affected neonates are well, without any evidence of neurological disturbances between seizures. Awareness of its existence among healthcare workers is critical for the diagnosis of this problem. Early diagnosis can lead to early intervention, which may mean a better prognosis for these children.

Extremity gangrene in utero.

A neonate with extremity gangrene resulting from intrauterine embolization of infarcted placental substances is discussed. This rare clinical entity is thought to be most commonly a manifestation of embolic phenomenon during maturation of the neonatal circulatory system. Management of neonatal gangrene is conservative, delaying amputation as long as possible since the line of demarcation tends to migrate distally. Evidence of multiple emboli should be carefully sought prior to definitive treatment.

Ischaemic and haemorrhagic brain lesions in newborns with seizures and normal Apgar scores.

Serial ultrasound scans and conventional and diffusion weighted magnetic resonance imaging (MRI) were performed on 16 neonates who presented with seizures. The Apgar scores were normal and subsequently no metabolic or infective cause could be found. The aim of the study was to evaluate the extent to which early sequential imaging can elucidate the cause of seizures in apparently neurologically normal infants. Fourteen of the infants had haemorrhagic or ischaemic lesions on MRI and these were detected by ultrasound scanning in 11. Early ultrasound scanning detected the haemorrhagic lesions but the ischaemic lesions were often not seen until the end of the first week of life. Early MRI, however, was able to detect all the ischaemic lesions. The evolution of the insult could be timed by using serial ultrasound scans and a combination of diffusion weighted and conventional MRI during the first week of life, confirming a perinatal insult even in the absence of fetal distress. Although the aetiology of these lesions remains obscure, serial ultrasound scans will detect the presence of cerebral lesions in neonates presenting with isolated seizures but additional MRI sequences will give better definition on type, site, and extent of the pathology.

Acute subdural hematoma associated with cerebral infarction in the full-term neonate.

Acute subdural hematomas in the full-term neonate are usually thought to be a manifestation of birth trauma. Most commonly, these hematomas are associated with a tentorial tear, and less frequently are secondary to damage to the occipital sinus accompanying occipital osteodiastasis, or to rupture of bridging superficial cerebral convexity veins. We report 6 cases of acute subdural hematoma associated with cerebral infarction (SDH/CI) in term neonates, an entity which has not been well-documented previously. This combination accounted for half the cases of neonatal acute subdural hematomas referred to a pediatric neurosurgical service. All children presented within 72 h of birth, usually after an uneventful delivery, and typically with seizures (5/6), apneic spells (3/6) and a full or bulging fontanel (5/6). Although the patients were referred to the neurosurgical servive for management of a convexity subdural hematoma, the CT scans showed a large associated cerebral infarct, which was hemorrhagic in 5 of the 6 cases, most commonly in the distribution of the middle cerebral artery. Craniotomy was performed in 4 patients, needle aspiration of the subdural hematoma in 1, and no surgical intervention in 1. The outcome in 3 patients, who have been followed for more than 1 year, has been good, with some mild focal neurologic deficits, but no seizure activity. The pathogenesis of this entity is unclear, but the acute subdural hematoma in these cases is probably secondary to the cerebral infarction. Recognition of SDH/CI as a specific entity in term neonates has significant management and possibly medicolegal implications.

Computerized electroencephalographic assessment of congenital brain infarction.

We studied 12 children (8 female and 4 male) aged 2.2-14.3 years, whose computed tomographic (CT) examination had shown evidence of malacic and/or porencephalic outcomes of early vascular brain infarction. Topographic spectral electroencephalographic (EEG) analysis was performed in all patients in the awake state. The following spectral EEG variables were studied: topography, absolute and relative power of delta, theta, alpha, beta bands, overall power, and peak alpha frequency asymmetries. The results of topographic spectral EEG analysis were compared with the localization and nature of lesions as detected by CT scans. Depending on the nature of the lesions, we were able to identify two different spectral patterns. Porencephalic cysts were characterized by an increase in delta and theta bands in the areas surrounding the lesion sites, as identified by CT. Spectral EEG patterns of malacic outcomes resulted in a focal increase in theta and delta band power, corresponding to the topography of lesions. Moreover, in 9/12 subjects an asymmetry of alpha rhythm in occipital leads was found homolaterally to the lesion sites, associated with a decrease in power, without any CT evidence of an occipital lesion.