Mineralizing Lenticulostriate Vasculopathy: An Emerging Risk Factor for Basal Ganglia Stroke After Minor Head Trauma in Young Children.

Mineralizing lenticulostriate vasculopathy is a well-recognized risk factor for basal ganglia stroke after minor head trauma in infants and young children; it is diagnosed on head computed tomography by the presence of basal ganglia calcification, seen as punctate hyperdensities on axial and linear hyperdensities on reconstructed coronal and sagittal images. In children with anterior fontanel window, its presence is suggested by branching hyperechogenic stripes in the basal ganglia region on cranial ultrasound. Brain magnetic resonance imaging, including susceptibility-weighted sequences and brain magnetic resonance angiography, fail to detect calcification or vascular abnormalities. Although its etiology remains unknown, mineralizing lenticulostriate vasculopathy is considered to represent end-stage pathology of lenticulostriate vasculopathy, a neonatal radiographic condition detected during routine neonatal cranial ultrasonographic examination and represents nonspecific finding associated with a multitude of etiologies. The significance of mineralizing lenticulostriate vasculopathy lies in the fact that it has emerged as one of the most common risk factors for basal ganglia stroke in Indian children, accounting for one-fourth to one-half of all causes of stroke in some studies. The outcome of stroke in children with mineralizing lenticulostriate vasculopathy appears to be favorable with the majority achieving complete or nearly complete recovery of their motor functions. Stroke recurrence following repeat head trauma is seen in a small proportion of children despite aspirin treatment.

Improved characterization of lenticulostriate arteries using compressed sensing time-of-flight at 7T.

OBJECTIVES: To evaluate the feasibility of 0.2-mm isotropic lenticulostriate arteries (LSAs) imaging using compressed sensing time-of-flight (CS TOF) at around 10 min on 7T, and compare the delineation and characterization of LSAs using conventional TOF and CS TOF. METHODS: Thirty healthy volunteers were examined with CS TOF and conventional TOF at 7T for around 10 min each. CS TOF was optimized to achieve 0.2-mm isotropic LSA imaging. The numbers of LSA stems and branches were counted and compared on a vascular skeleton. The length and distance were measured and compared on the most prominent branch in each hemisphere. Another patient with intracranial artery stenosis was studied to compare LSA delineation in CS TOF and digital subtraction angiography (DSA). RESULTS: The number of stems visualized with CS TOF was significantly higher than with conventional TOF in both left (p = 0.002, ICC = 0.884) and right (p < 0.001, ICC = 0.938) hemispheres. The number of branches visualized by conventional TOF was significantly lower than that by CS TOF in both left (p < 0.001, ICC = 0.893) and right (p < 0.001, ICC = 0.896) hemispheres. The lengths were statistically higher in CS TOF than in conventional TOF (left: p < 0.001, ICC = 0.868; right: p < 0.001, ICC = 0.876). CONCLUSIONS: The high-resolution CS TOF improves the delineation and characterization of LSAs over conventional TOF. High-resolution LSA imaging using CS TOF can be a promising tool for clinical research and applications in patients with neurologic diseases. KEY POINTS: • 0.2-mm isotropic LSA imaging for around 10 min using CS TOF at 7T is feasible. • More stems and branches of LSAs with longer lengths can be delineated with CS TOF than with conventional TOF at the same scan time. • High-resolution CS TOF can be a promising tool for research and applications on LSA.

CMV-induced Hearing Loss.

Congenital cytomegalovirus (cCMV) infection is the most common fetal viral infection and contributes to about 25% of childhood hearing loss by the age of 4 years. It is the leading nongenetic cause of sensorineural hearing loss (SNHL). Infants born to seroimmune mothers are not completely protected from SNHL, although the severity of their hearing loss may be milder than that seen in those whose mothers had a primary infection. Both direct cytopathic effects and localized inflammatory responses contribute to the pathogenesis of cytomegalovirus (CMV)-induced hearing loss. Hearing loss may be delayed onset, progressive or fluctuating in nature, and therefore, a significant proportion will be missed by universal newborn hearing screening (NHS) and warrants close monitoring of hearing function at least until 5-6 years of age. A multidisciplinary approach is required for the management of hearing loss. These children may need assistive hearing devices or cochlear implantation depending on the severity of their hearing loss. In addition, early intervention services such as speech or occupational therapy could help better communication, language, and social skill outcomes. Preventive measures to decrease intrauterine CMV transmission that have been evaluated include personal protective measures, passive immunoprophylaxis and valacyclovir treatment during pregnancy in mothers with primary CMV infection. Several vaccine candidates are currently in testing and one candidate vaccine in phase 3 trials. Until a CMV vaccine becomes available, behavioral and educational interventions may be the most effective strategy to prevent maternal CMV infection.

Lenticulostriate vasculopathy in routine brain ultrasonography in infants: next step?

Lenticulostriate vasculopathy (LSV) is a relatively common fi nding in routine cranial ultrasound examination that has been associated with many infectious and non-infectious conditions. The aim of this review was to provide a better understanding of LSV ultrasound fi nding, as well as the need for further laboratory and imaging examinations in infants. The most of the published studies represented small series, with few prospective long-term studies involving the control groups. Authors have mostly found an association between LSV, especially higher-grade (although there is no universally accepted classifi cation) with congenital cytomegalovirus (CMV) infection, classifying those children as at risk for sensorineural hearing loss. In contrast, some authors pointed out that LSV could be found relatively often, and believe that isolated LSV, especially lower-grade, is not predictive for an unfavourable outcome and a long-term prognosis. Therefore, although 35 years have passed since the first publication of LSV, there is still no consensus among experts on the clinical signifi cance of isolated LSV, but caution is certainly needed given the fact that most infants with congenital CMV are asymptomatic.

Lenticulostriate Vasculopathy in Very-Low-Birth-Weight Preterm Infants: A Longitudinal Cohort Study.

BACKGROUND: The pathogenesis and clinical significance of lenticulostriate vasculopathy (LSV) are unclear. Our study aimed to determine the prevalence, presentation, and evolution of LSV, and the perinatal risk factors associated with LSV among very-low-birth-weight (VLBW) preterm infants. METHODS: One-hundred-and-thirty VLBW preterm infants were retrospectively enrolled in this study. Serial cranial ultrasound examinations were performed regularly from birth until a corrected age of 1 year. Infants with LSV were assigned to early-onset (≤10 postnatal days) and late-onset (>10 postnatal days) groups. Data describing the infants' perinatal characteristics, placental histopathology, and neonatal morbidities were collected, and the groups were compared. RESULTS: Of the VLBW infants, 39.2% had LSV before they were 1 year old. Linear-type LSV was the most common presentation, and >50% of the infants had bilateral involvement. LSV was first detected at 112 ± 83 postnatal days, and its detection timing correlated negatively with gestational age (GA) (R2 = 0.153, p = 0.005) and persisted for 6 months on average. The infants with and without LSV had similar perinatal characteristics, placental pathologies, cytomegalovirus infection rates, and clinical morbidities. The late-onset LSV group comprised 45 (88.2%) infants who had a significantly higher rate of being small for gestational age (SGA) and used oxygen for longer than the infants without LSV. After adjusting a multivariable regression model for GA and SGA, analysis showed that the duration of oxygen usage was an independent risk factor for late-onset LSV development in VLBW infants (odds ratio: 1.030, p = 0.032). CONCLUSION: LSV may be a nonspecific marker of perinatal insult to the developing brains of preterm infants. Prolonged postnatal oxygen usage may predispose VLBW preterm infants to late-onset LSV development. The long-term clinical impacts of LSV should be clarified.

Visualization of lenticulostriate artery by intracranial dark-blood vessel wall imaging and its relationships with lacunar infarction in basal ganglia: a retrospective study.

OBJECTIVES: There is close relationship between lenticulostriate arteries (LSAs) and lacunar infarctions (LIs) of the basal ganglia. The study aims to visualize the LSAs using high-resolution vessel wall imaging (VWI) on 3T system and explore the correlation between LSAs and LIs. METHODS: Fifty-six patients with LIs in basal ganglia, and 44 age-matched control patients were enrolled and analyzed retrospectively. The raw VWI images were reformatted into coronal slices in minimum intensity projection for further observation of LSAs. The risk factors of LIs in basal ganglia were analyzed by univariate and multivariate logistic regression. The correlation and linear regression analysis between the LSAs and LIs, ipsilateral MCA-M1 plaques were investigated. RESULTS: The total number (p < 0.01) and length (p < 0.01) of LSAs were statistically different between basal ganglias with and without LIs. The total number of LSAs and ipsilateral MCA-M1 plaques were independently related to LIs in basal ganglias. The mean length of LSAs were negatively correlated with number (r = - 0.33, p = 0.002) and volume (r = - 0.37, p = 0.001) of LIs. Age, drinking history, and mean length of LSAs were associated with LI occurrence in basal ganglia, and mean length of LSAs was correlated with larger volume of LIs. CONCLUSIONS: Number of LSA reduction and ipsilateral MCA-M1 plaques were associated with the presence of LIs in basal ganglias. Age increasing, drinking history, and shorter LSAs were correlated with the increasing of LIs. KEY POINTS: • Patients with LIs tend to have shorter LSAs. • The characteristics of LSAs and ipsilateral MCA-M1 plaques are associated with LIs in basal ganglias. • Age, drinking history, and mean length of LSAs are correlated with LI features in basal ganglias.

Riesgo de daño cerebral en prematuros menores de 34 semanas expuestos a corioamnionitis histológica, Lima, Perú / Risk of brain damage in premature infants under 34 weeks of gestational age exposed to histological chorioamnionitis Lima, Peru

RESUMEN Objetivos: Evaluar el riesgo de daño cerebral en prematuros menores de 34 semanas expuestos a corioamnionitis histológica (CAH). Materiales y métodos: Se realizó un estudio de cohortes en el Hospital Cayetano Heredia, durante el 2015. Fueron incluidos prematuros menores de 34 semanas que tuvieran examen histopatológico de la placenta. Los tipos de CAH evaluados fueron subcorionitis, corionitis, corioamnionitis, con o sin funisitis. El daño cerebral se evaluó en tres periodos de edad, entre 0 y 7 días, entre 7 y 30 días y a las 40 semanas gestacionales corregidas. Se realizó un seguimiento neurológico y controles con ecografía cerebral. Resultados: Se estudiaron 85 prematuros, 47,1% eran mujeres y la media de la edad gestacional fue de 30,9 semanas. El 42% (36/85) nacieron expuestos a CAH. La ruptura prematura de membrana fue la principal generatriz de sepsis, y la sepsis se relacionó con daño neurológico. La CAH estuvo asociada con hemorragia intraventricular (HIV) durante la primera semana y con lesiones de la sustancia blanca entre los 7 y 30 días de edad (p = 0,035). El tipo corioamnionitis de CAH se asoció al daño neurológico durante la primera semana (RR = 2,11; IC 95%: 1,09-4,11) y entre los 7 y 30 días de vida (RR = 2,72; IC 95%: 1,07-6,88). Conclusiones: La corioamnionitis fue un factor de riesgo para desarrollar lesiones cerebrales en prematuros menores de 34 semanas, para HIV durante los primeros 7 días y lesiones de sustancia blanca entre los 7 y los 30 días de edad. A las 40 semanas de edad corregida, los prematuros extremos con CAH tuvieron lesiones cerebrales más extensas.

ABSTRACT Objectives: To assess the risk of brain damage in premature infants under 34 weeks of gestational age exposed to histological chorioamnionitis (HCA). Materials and methods: A cohort study was conducted at the Hospital Cayetano Heredia, during 2015. Premature infants under 34 weeks of gestational age, who had histopathological examination of the placenta, were included. The types of HCA evaluated were sub-chorionitis, chorionitis, chorioamnionitis, with or without funisitis. Brain damage was evaluated in three age periods, between 0 and 7 days, between 7 and 30 days and at 40 weeks of corrected gestational age. A neurological follow-up and regular controls were performed with brain ultrasound. Results: A total of 85 premature infants were included, 47.1% were women and the mean gestational age was 30.9 weeks. From the total, 42% (36/85) were born exposed to HCA. Premature rupture of membranes was the main cause of sepsis, which was related to neurological damage. HCA was associated with intraventricular hemorrhage (IVH) during the first week and with white matter lesions between 7 and 30 days of age (p = 0.035). The chorioamnionitis type of HCA was associated with neurological damage during the first week (RR = 2.11, 95% CI: 1.09-4.11) and between 7 and 30 days of age (RR = 2.72, 95% CI: 1.07-6.88). Conclusions: Chorioamnionitis was a risk factor for developing brain injuries in premature infants under 34 weeks of gestational age. It was also a risk factor for HIV during the first 7 days and for white matter injuries between 7 and 30 days of age. At 40 weeks of corrected gestational age, extreme premature infants with HCA had more extensive brain damage.

An extremely severe case of Aicardi-Goutières syndrome 7 with a novel variant in IFIH1.

We describe herein an extremely severe case of Aicardi-Goutières syndrome 7 (AGS7). The female patient was the daughter of nonconsanguineous parents and developed cardiomegaly, pericardial effusion, splenomegaly, and intracranial calcification during the fetal period. Because her cardiotocogram showed a non-reassuring fetal status, she was delivered at 29 weeks and 4 days of gestation by an emergency cesarean section. After birth, she suffered from respiratory distress, pulmonary hypertension, refractory fever, recurrent thrombocytopenia, and abdominal distention caused by hepatomegaly and ascites. She showed a lenticulostriate vasculopathy, which was compatible with the fetal intracranial calcification. Despite various intensive care procedures, she died of gradually progressive pulmonary hypertension at 3 months of age. After her death, whole exome sequencing on the patient and the parents was performed and revealed a novel, de novo, heterozygous mutation in the IFIH1 gene (IFIH1:NM_022168:exon12:c.2439A > T:p.Glu813Asp). On the basis of the mutation and the clinical features, the diagnosis was AGS7. Although AGS7 has been regarded as a relatively mild subtype of Aicardi-Goutières syndrome, this case indicates that the c.2439A > T variant of AGS7 can be fatal in early infancy.

7T TOF-MRA shows modulated orifices of lenticulostriate arteries associated with atherosclerotic plaques in patients with lacunar infarcts.

PURPOSE: To characterize the orifices of lenticulostriate arteries (LSAs) in vivo by using three-dimensional (3D) time-of-flight magnetic resonance angiography (TOF-MRA) and to investigate the spatial relationship between LSA orifices and atherosclerotic plaques in patients with lacunar infarcts (LI). METHOD: Seventeen healthy volunteers and fifteen patients with LI underwent 3D TOF-MRA and 3D vessel wall imaging (VWI) at 7 T. The orifices of LSAs and the locations of atherosclerotic plaques on MCA walls were categorized based on the involvement of the superior, inferior, ventral or dorsal sides of MCA wall. The distribution quadrants of LSA orifices on MCA walls were compared among different groups. RESULTS: Most orifices were located on the superior side of MCA firstly (46 of 95, 48.4%), followed by the dorsal side (22 of 95, 23.2%). In patients with LI, the visible numbers of ventral and inferior orifices on the ipsilateral side were significantly lower than healthy controls (p = 0.039 for ventral side, p = 0.002 for inferior side). Similarly, plaques occurred more frequently at the ventral (7 of 20, 35.0%) and the inferior (7 of 20, 35.0%) sides of MCA walls. CONCLUSIONS: TOF-MRA at 7 T is capable of imaging orifices of LSA on MCA. In patients with LI, the decreased number of LSA orifices on the ventral and inferior sides corresponded with the distribution of MCA plaques. The results may indicate the vulnerability of LSA orifices in intracranial atherosclerosis, which was supposed to be the cause of LI in basal ganglia.

Visualization of the lenticulostriate arteries at 3T using black-blood T1-weighted intracranial vessel wall imaging: comparison with 7T TOF-MRA.

OBJECTIVES: The objective of this study was to explore the feasibility of using intracranial T1-weighted vessel wall imaging (VWI) to visualize the lenticulostriate arteries (LSAs) at 3T. MATERIAL AND METHODS: Thirteen healthy volunteers were examined with VWI at 3T and TOF-MRA at 7T during the same day. On the vascular skeletons obtained by manual tracing, the number of stems and branches of LSAs were counted. On the most prominent branch in every hemisphere, the contrast-to-noise ratio (CNR), the full length and the local length (5-15 mm above MCAs) were measured and compared between the two methods. Nine stroke patients with intracranial artery stenosis were also recruited into the study. The branches of LSAs were compared between the symptomatic and asymptomatic side. RESULTS: The extracted vascular trees were in good agreement between 7T TOF-MRA and 3T VWI. The two acquisitions showed similar numbers of the LSA stems. The number of branches revealed by 3T VWI was slightly lower than 7T TOF. The full lengths were slightly lower by VWI at 3T (p = 0.011, ICC = 0.917). The measured local lengths (5-15 mm from MCAs) showed high coherence between VWI and TOF-MRA (p = 0.098, ICC = 0.970). In stroke patients, 12 plaques were identified on MCA segments, and nine plaques were located on the symptomatic side. The average numbers of LSA visualized by 3T VWI were 4.3±1.3 on the symptomatic side and 5.0±1.1 on the asymptomatic side. CONCLUSION: 3T VWI is capable of depicting LSAs, particularly the stems and the proximal segments, with comparable image quality to that of 7T TOF-MRA. KEY POINTS: • T1-weighted intracranial VWI at 3T allows for black-blood MR angiography of lenticulostriate artery. • 3T intracranial VWI depicts the stems and proximal segments of the lenticulostriate arteries comparable to 7T TOF-MRA. • It is feasible to assess both large vessel wall lesions and lenticulostriate vasculopathy in one scan.

Fetal stroke and cerebrovascular disease: Advances in understanding from lenticulostriate and venous imaging, alloimmune thrombocytopaenia and monochorionic twins.

Fetal stroke is an important cause of cerebral palsy but is difficult to diagnose unless imaging is undertaken in pregnancies at risk because of known maternal or fetal disorders. Fetal ultrasound or magnetic resonance imaging may show haemorrhage or ischaemic lesions including multicystic encephalomalacia and focal porencephaly. Serial imaging has shown the development of malformations including schizencephaly and polymicrogyra after ischaemic and haemorrhagic stroke. Recognised causes of haemorrhagic fetal stroke include alloimmune and autoimmune thrombocytopaenia, maternal and fetal clotting disorders and trauma but these are relatively rare. It is likely that a significant proportion of periventricular and intraventricular haemorrhages are of venous origin. Recent evidence highlights the importance of arterial endothelial dysfunction, rather than thrombocytopaenia, in the intraparenchymal haemorrhage of alloimmune thrombocytopaenia. In the context of placental anastomoses, monochorionic diamniotic twins are at risk of twin twin transfusion syndrome (TTTS), or partial forms including Twin Oligohydramnios Polyhydramnios Sequence (TOPS), differences in estimated weight (selective Intrauterine growth Retardation; sIUGR), or in fetal haemoglobin (Twin Anaemia Polycythaemia Sequence; TAPS). There is a very wide range of ischaemic and haemorrhagic injury in a focal as well as a global distribution. Acute twin twin transfusion may account for intraventricular haemorrhage in recipients and periventricular leukomalacia in donors but there are additional risk factors for focal embolism and cerebrovascular disease. The recipient has circulatory overload, with effects on systemic and pulmonary circulations which probably lead to systemic and pulmonary hypertension and even right ventricular outflow tract obstruction as well as the polycythaemia which is a risk factor for thrombosis and vasculopathy. The donor is hypovolaemic and has a reticulocytosis in response to the anaemia while maternal hypertension and diabetes may influence stroke risk. Understanding of the mechanisms, including the role of vasculopathy, in well studied conditions such as alloimmune thrombocytopaenia and monochorionic diamniotic twinning may lead to reduction of the burden of antenatally sustained cerebral palsy.

Intraventricular haemorrhage as the first manifestation of congenital Cytomegalovirus infection.

Congenital Cytomegalovirus infection (CCMV) is the most common intrauterine infection. Early diagnosis of CCMV is hindered by three factors: There is no screening programme for CMV infection in pregnant women; a high percentage of infections in neonates are asymptomatic; the clinical signs of CCMV infection are uncharacteristic. The aim of this article is to analyse the clinical picture and course of CCMV treatment in a 3-week-old newborn, analyse adverse events in 14-week-long antiviral therapy and also assess intraventricular bleeding as an early indicator for the diagnosis of CCMV.

Hyperechogenicity of lenticulostriate vessels: A poor prognosis or a normal variant? A seven year retrospective study.

BACKGROUND: Lenticulostriate vasculopathy (LSV) is a hyperechogenicity of the lenticulostriate branches of the basal ganglia and/or thalamus' middle cerebral arteries and is frequently seen in neonatology. Our study primarily describes the perinatal data and long-term follow-up of newborns with lenticulostriate vessel hyperechoic degeneration. Secondly, it describes the cerebral imaging data as a function of perinatal factors and neurodevelopmental follow-up of these newborns. METHODS: This retrospective study assesses the outcome of newborns with LSV hyperechogenicity on cerebral ultrasound (two grades). These children were born between January 2008 and September 2015 and were treated in a large level III neonatal intensive care unit. Thirty-four term-equivalent age children underwent MRIs using a standardized protocol of T2, T1 3D, diffusion and spectro-MRI sequences. The MRIs retrospectively measured the white matter and basal ganglia apparent diffusion coefficients (ADC). RESULTS: Fifty-eight neonates, ranging from 25 to 42 weeks gestational age (GA), were diagnosed with LSV. There was a significantly increased high-grade LSV when accompanied by fetal heart rate abnormalities (p = 0.03) and the neonate's need for respiratory support at birth (P = 0.002). The mean ADC score was substantially superior in the high-grade versus the low-grade LSVs (p = 0.023). There were no noteworthy outcome differences between a high and low grade LSV. The mean ADC for basal ganglions was appreciably higher in children with a severe prognoses (death or developmental disorder) as compared to children with no abnormalities (p < 0.01). CONCLUSION: From the results of our study, it appears that a low-grade LSV could be considered as a normal variant. There are no unifying diagnostic criteria for LSV on cerebral ultrasound. With a cerebral MRI, the use of ADC values of basal ganglia may well underscore the importance of such data in predicting long-term outcomes.

Stroke After Minor Head Trauma in Infants and Young Children With Basal Ganglia Calcification: A Lenticulostriate Vasculopathy?

The authors retrospectively reviewed charts of the children with basal ganglia stroke who either had preceding minor head injury or showed basal ganglia calcification on computed tomography (CT) scan. Twenty children, 14 boys and 6 girls were identified. Eighteen were aged between 7 months to 17 months. Presentation was with hemiparesis in 17 and seizures in 3. Preceding minor head trauma was noted in 18. Family history was positive in 1 case. Bilateral basal ganglia calcification on CT scan was noted in 18. Brain magnetic resonance imaging done in 18 infants showed acute or chronic infarcts in basal ganglia. Results of other laboratory and radiological investigations were normal. Four infants were lost to follow-up, 9 achieved complete or nearly completely recovery, and 7 had persistent neurological deficits. Basal ganglia calcification likely represents mineralized lenticulostriate arteries, a marker of lenticulostriate vasculopathy. Abnormal lenticulostriate vessels are vulnerable to injury and thrombosis after minor head trauma resulting in stroke.

Clinical Significance of Neonatal Lenticulostriate Vasculopathy: Association with Congenital Cytomegalovirus Infection

PURPOSE: To investigate clinical characteristics of neonates with lenticulostriate vasculopathy (LSV) and determine the correlation between LSV and clinical characteristics, especially congenital cytomegalovirus (CMV) infection. METHODS: We retrospectively reviewed the medical records of neonates with LSV, born at Cheil General Hospital between January 2005 and December 2015. LSV was graded into three groups based on the number of the LSV lesions and classified into an isolated and combined group showing LSV with coexistent abnormalities noted on brain sonography. We compared clinical data based on the LSV classification. RESULTS: Our study included 102 neonates with LSV, which showed an unilateral pattern in 10 and bilateral pattern in 92 neonates. The numbers of neonates studied based on LSV grading were 33, 53, and 16 in grade 1, 2, and 3, respectively. We observed the isolated LSV in 62 and the combined type in 40 neonates. We observed that 93 (91.2%) of the neonates with LSV did not show specific underlying cause for this condition. Congenital CMV infection was detected in 7 neonates, including 0, 5, and 2 neonates belonging to grade 1, 2, and 3, respectively. Among these, 2 neonates showed the isolated, and 5 showed the combined type of LSV. Statistically, congenital CMV infection was more significantly associated with LSV in grade 2 and 3 than in grade 1 (P < 0.05). Additionally, congenital CMV infection was more commonly observed in the combined than in the isolated LSV type showing a marginal association (P=0.07). CONCLUSION: We observed that LSV was not clinically significant except when associated with CMV infection. We suggest that neonates presenting with a grade 2 or higher of LSV or a combined type of LSV detected via neonatal brain ultrasonography should be evaluated for CMV infection.

Fetal Infection by Zika Virus in the Third Trimester: Report of 2 Cases.

Zika virus (ZIKV) infection acquired during pregnancy is associated with congenital microcephaly. We describe 2 cases of ZIKV infection in women in their 36th week of pregnancy whose fetuses had preserved head circumference at birth and findings of subependymal cysts and lenticulostriate vasculopathy in postnatal imaging. These represent the first signs of congenital brain injury acquired due to ZIKV in the third trimester.

Lenticulostriate Vasculopathy in Brain Ultrasonography is Associated with Cytomegalovirus Infection in Newborns.

BACKGROUND: Lenticulostriate vasculopathy is associated with various disorders, in particular cytomegalovirus infection, which can cause neurological consequences. We wanted to evaluate the association of lenticulostriate vasculopathy and cytomegalovirus infection. We retrospectively collected data on lenticulostriate vasculopathy from 858 neonatal ultrasonography scans. METHODS: Fifty-five patients with lenticulostriate vasculopathy were diagnosed. Lenticulostriate vasculopathy was classified as severe and mild according to the ultrasonographic findings. We analyzed gender, unilateral and bilateral lenticulostriate vasculopathy, mild and severe lenticulostriate vasculopathy, intrauterine growth retardation, and lenticulostriate vasculopathy associated with other brain malformations to determine whether they were correlated with cytomegalovirus infection. RESULTS: Neonatal cytomegalovirus infections correlated primarily with lenticulostriate vasculopathy that was associated with brain structure anomalies p < 0.0001, followed by severe lenticulostriate vasculopathy (p = 0.029). Cytomegalovirus urine polymerase chain reaction ratios were 69% for severe and 23% for mild lenticulostriate vasculopathy (p = 0.002; odds ratio = 7.33). Of 72 newborns with intrauterine growth retardation without lenticulostriate vasculopathy, 33 were analyzed for cytomegalovirus, of whom only one was positive, which was significantly different from the newborns with lenticulostriate vasculopathy (p = 0.003; odds ratio = 11.64). CONCLUSION: Lenticulostriate vasculopathy on neonatal ultrasonography is useful for predicting cytomegalovirus infection, particularly in severe lenticulostriate vasculopathy. When severe lenticulostriate vasculopathy is associated with a brain structure anomaly, cytomegalovirus infection should be considered. The outcomes for the cases in which cytomegalovirus infection was associated with other brain structure anomalies were significantly worse than the outcomes in cases associated with lenticulostriate vasculopathy only.

Lenticulostriate vasculopathy in neonates: Perspective of the radiologist.

Lenticulostriate vasculopathy (LSV) is a diagnosis dependent on neonatal cranial ultrasound (US). The diagnosis of LSV requires the presence of linear or branching echogenicities in the area of the basal ganglia and/or thalamus on gray scale cranial US. Although the diagnosis of LSV is dependent on cranial US, there are no convincing correlates observed on either computerized tomography or magnetic resonance imaging. Moreover, the radiographic criteria for LSV on cranial US remain vague, and intra-observer correlations are generally reported to be poor. The purpose of this review is to examine the issues associated with the use of cranial US and the diagnosis of LSV, including alternative imaging, clinical abnormalities and the significance of LSV on cranial US.

Imaging patterns of sonographic lenticulostriate vasculopathy and correlation with clinical and neurodevelopmental outcome.

PURPOSE: To evaluate the relationship between the imaging patterns of lenticulostriate vasculopathy (LSV) and clinical outcomes. METHODS: We performed cranial sonography (US) in 110 neonates and evaluated the patterns of visible lenticulostriate vessels with three grades: 0: no vessel seen; 1 (low grade): one or two thin branches seen; and 2 (high grade): more than three prominent branches seen. Color Doppler US was performed on these vessels to evaluate the presence of flow. Associated underlying diseases and the presence of neurodevelopmental delay on follow-up were reviewed retrospectively. RESULTS: There were 51 neonates with associated underlying diseases, including congenital heart diseases (CHD) (n = 34) and neonatal hypoxia (n = 13). Sonographic LSV was detected in 29.1% cases (22 low- and 10 high-grade cases). Doppler flow was not detected in three patients with CHD (p = 0.028). CHD (odds ratio [OR], 25.73; p < 0.001), neonatal hypoxia (OR, 7.00; p = 0.020), two underlying diseases (OR, 73.232; p < 0.001), high-grade LSV (OR, 16.29; p = 0.005), and absent color Doppler flow (OR, 40.80; p = 0.046) were significantly associated with neurodevelopmental delay in univariate analysis. In multivariate analysis, underlying diseases and absent color Doppler flow were associated with neurodevelopmental delay. Both high LSV grade (area under the receiver operating characteristic curves of 0.901; 95% confidence interval, 0.823-0.979) and absent color Doppler flow (area under the receiver operating characteristic curves of 0.874; 95% confidence interval, 0.803-0.945) had a high predictive power for neurodevelopmental delay. CONCLUSIONS: High-grade sonographic LSV and absent color Doppler flow on lenticulostriate vessels were significantly associated with neurodevelopmental delay.