Severity Of Periventricular Leukomalacia Research Articles

Dystonia in individuals with spastic cerebral palsy and isolated periventricular leukomalacia.

To determine the prevalence of dystonia in individuals with periventricular leukomalacia (PVL) and spastic cerebral palsy (CP), but without basal ganglia and thalamic injury (BGTI) on brain magnetic resonance imaging (MRI). This was a retrospective study of individuals with spastic CP and PVL on MRI evaluated between 2005 and 2018 in a CP center. Individuals with non-PVL brain lesions on MRI, including BGTI, were excluded. Dystonia was assessed via blinded review of neurological exam videos by pediatric movement disorders specialists. Eighty-five participants (45 males, 40 females; mean age at videotaping 12 years [standard deviation 5 years 6 months], range 4-26 years) met inclusion and exclusion criteria. Of these participants, 50 (59%) displayed dystonia in their exam videos. The most common locations of dystonia were the fingers and hip adductors. The prevalence of dystonia was unaffected by the gestational age or severity of PVL, and was affected by Gross Motor Function Classification System level. Dystonia is common in individuals with spastic CP and PVL, even without BGTI on MRI. Our findings suggest vigilance for dystonia in individuals with spastic CP should remain high, even without MRI evidence of BGTI. Individuals with spastic cerebral palsy and isolated periventricular leukomalacia on magnetic resonance imaging commonly display dystonia. Common sites of dystonia are in the fingers and hip adductors.

Prevalence of the Infantile Strabismus Complex in Premature Children With and Without Periventricular Leukomalacia

To determine whether rates of strabismus and associated visuomotor deficits differed among children with different severities of periventricular leukomalacia (PVL). Retrospective, case-control study. Brain magnetic resonance images (MRI) obtained from 98 children aged ≥2 years were analyzed using a standardized scoring system: 67 of 98 had PVL (mean GA 31 weeks) and 31 of 98 did not have PVL (mean GA 29 weeks). Severity of PVL was scored as degree of damage to the posterior optic radiations and the splenium of the corpus callosum on MRI. Ophthalmologic examination data were collated to assess the prevalence of visuomotor deficits and the relationship to PVL severity (grades 1-3, mild to severe). Infantile strabismus was documented in 61% of children with mild, 74% with moderate, and 88% with severe PVL (esotropia: exotropia ratio 3.5:1). Associated ocular motor deficits also increased systematically with PVL severity: latent ("fusion maldevelopment") nystagmus (20%, 47%, and 40%, respectively), dissociated vertical deviation (13%, 28%, and 30%), and nasotemporal pursuit/optokinetic nystagmus asymmetry (23%, 38%, and 54%). Additionally, the prevalence of retrograde optic neuropathy increased with PVL severity (5%, 26%, and 38%). The prevalence of each of these signs was substantially lower in children who had no PVL. Children who suffer PVL are likely to develop the deficits of the infantile strabismus complex. The deficits tend to increase systematically as a function of PVL severity. These findings provide evidence that infantile strabismus is linked to perinatal damage to cerebral vergence and gaze pathways.

Alteration in the number and integrity of white matter tracts in the preterm: A quantitative diffusion tensor imaging and diffusion fibre tractography in children.

Purpose:Periventricular white matter is most commonly injured in preterm babies with hypoxia. To assess white matter damage, we decided to perform diffusion tensor imaging (DTI) in preterm children with history of hypoxia and magnetic resonance imaging (MRI) features of periventricular leukomalacia (PVL) (PTH). We hypothesized that the PTH have reduced number of white matter fibres compared to age matched pre term children without hypoxia (PTHO), and also depending on the severity of PVL, there could be reduction in the number of fibres as well.Materials and Methods:The present study was carried out at the Government Medical College, Thiruvananthapuram. DTI was performed on 15 PTH and 15 PTHO. We measured number of fibres and fractional anisotropy of corpus callosum (CC) and optic radiations (OR).Results:There was significant difference between two groups with regard OR (P < 0.001). The mean number of OR fibres in cases and control was 104 ± 28.44 (mean ± SD) and 578 ± 286 (mean ± SD), respectively. The mean number of CC in cases was 953 ± 429 and in controls was 1625 ± 116 with a P value <0.56. No significant difference in FA was seen between cases and controls (P = 0.94).Conclusions:Preterm children with history of hypoxia and MRI features of PVL show reduced number of CC and OR compared to preterm children without hypoxia. There was significant correlation between PVL severity and number of OR fibres which could be due to the preferential involvement of periventricular white matter, in which OR has a major contribution.

Strabismus in Children with Periventricular leukomalacia: MRI correlation

PurposeTo investigate the correlation between strabismus and the severity of PVL based on magnetic resonance imaging (MRI) findings. Although strabismus is commonly associated with periventricular leukomalacia (PVL), its clinical features are not well established.MethodsIn this cross‐sectional study, 73 consecutive patients who visited the Department of Ophthalmology and were diagnosed with PVL were included. The severity of PVL was graded based on the MRI findings of the patients. All of the patients underwent complete ophthalmic examination, and strabismus was characterized in terms of direction, constancy, and angle of deviation. The prevalence and the characteristics of strabismus and their correlation with the grade of PVL were investigated.ResultsThe perinatal characteristics did not differ between different grades of PVL. Refractive errors, found in 56 (76.7%) patients, did not differ between the grades of PVL either. Strabismus was observed in 38 (52.1%) patients, and its prevalence increased with the grade of the disorder; 20 patients had exotropia and 18 had esotropia. Constant strabismus was found more frequently in patients with higher grade PVL. However, the direction and angle of deviation did not differ depending on the grade of PVL.ConclusionsThe prevalence of strabismus was higher among patients with PVL than among healthy individuals and increased with the severity of PVL. The severity of PVL might be related to the presence and constancy of strabismus. Early ophthalmic evaluation is necessary in children with PVL, especially in those with higher grades of the disease.

The significance of periventricular leukomalacia on ophthalmic outcome

Aim: Periventricular leukomalacia (PVL) is a type of cerebral white matter damage commonly arising within neonates born prematurely. This review aims to evaluate the literature relating to the long-term ophthalmic outcomes following PVL, focussing on the relationship between neuro-imaging and visual out- come. Methods: A literature review was undertaken between October 2013 and January 2015. Articles were sourced using PubMed, ResearchGate and forward citation searches. Results: PVL is shown to increase an individual’s chance of developing ocular defects, namely cerebral visual impairment, strabismus, visual field defects and visuoperceptual anomalies. The severity and extent of PVL is directly proportional to both the chance of developing an ocular defect, and the severity of said ocular defect; however, there is not a perfect correlation and ophthalmic outcome is specific to each individual. There have also been reports of strabismus being the presenting factor, leading to an investigation which revealed the presence of PVL that had been missed during the neonatal period. Neuro-imaging has been shown to have some predictive ability, varying depending on the area of the visual pathway examined, and the aspect of visual outcome predicted. Conclusions: While predictive results gathered via neuro-imaging can offer insight into visual outcome, these must be consolidated through non-radiological clinical testing. Strabismus has been documented as a presenting factor in patients with PVL. Therefore the initial presentation of a patient with PVL, where the diagnosis has been missed during the neonatal period, may be through the orthoptic department.

Scoring system for periventricular leukomalacia in infants with congenital heart disease.

Currently two magnetic resonance imaging (MRI) methods have been used to assess periventricular leukomalacia (PVL) severity in infants with congenital heart disease: manual volumetric lesion segmentation and an observational categorical scale. Volumetric classification is labor intensive and the categorical scale is quick but unreliable. We propose the quartered point system (QPS) as a novel, intuitive, time-efficient metric with high interrater agreement. QPS is an observational scale that asks the rater to score MRIs on the basis of lesion size, number, and distribution. Pre- and postoperative brain MRIs were obtained on term congenital heart disease infants. Three independent observers scored PVL severity using all three methods: volumetric segmentation, categorical scale, and QPS. One-hundred and thirty-five MRIs were obtained from 72 infants; PVL was seen in 48 MRIs. Volumetric measurements among the three raters were highly concordant (ρc = 0.94-0.96). Categorical scale severity scores were in poor agreement between observers (κ = 0.17) and fair agreement with volumetrically determined severity (κ = 0.26). QPS scores were in very good agreement between observers (κ = 0.82) and with volumetric severity (κ = 0.81). QPS minimizes training and sophisticated radiologic analysis and increases interrater reliability. QPS offers greater sensitivity to stratify PVL severity and has the potential to more accurately correlate with neurodevelopmental outcomes.

The Relationship between the Severity of Periventricular Leukomalacia and the Fractional Anisotropy Values of Diffusion Tensor Imaging

The Relationship between the Severity of Periventricular Leukomalacia and the Fractional Anisotropy Values of Diffusion Tensor Imaging

Does perinatal asphyxia contribute to neurological dysfunction in preterm infants?

BackgroundChildren born preterm are known to be at risk for neurodevelopmental disorders. The role of perinatal asphyxia in this increased risk is still a matter of debate. AimTo analyze the contribution of perinatal asphyxia in a population of preterm infants admitted to a secondary paediatric setting to neurological dysfunction in the first months after birth and to the development of cerebral palsy. Methods17 preterm infants with perinatal asphyxia born before 35weeks postmenstrual age (PMA) and 34 carefully matched preterm controls without asphyxia were studied. Neuromotor outcome was examined by means of three assessments of the quality of general movements (GM) at “preterm” (around 34weeks PMA), “writhing” (around term age) and “fidgety” GM age (around 3months post term). Follow-up until at least 18months corrected age focused on the presence of cerebral palsy (CP). ResultsGM-quality of infants with asphyxia and of those without did not differ. Multivariate analysis revealed that abnormal GMs at “preterm” age were associated with respiratory problems, those at “writhing” age with none of the assessed risk factors, and those at “fidgety” age with the severity of periventricular leukomalacia (PVL) on neonatal ultrasound scan.Perinatal asphyxia was not associated with the development of CP. CP was associated with PVL and the presence of abnormal GMs at “fidgety” age. ConclusionPerinatal asphyxia in preterm infants is not associated with an increased risk for neurodevelopmental problems including CP. Respiratory problems during the neonatal period are associated with PVL and adverse neurological outcome.

Cortical Visual Impairment

1. Luis H. Ospina, MD* 1. *Assistant Professor, Pediatric Ophthalmology and Neuro-ophthalmology, Ste-Justine Hospital, University de Montreal, Montreal, Quebec, Canada After completing this article, readers should be able to: 1. Recognize cortical visual impairment (CVI) as an important cause of pediatric visual loss. 2. Identify the most frequent causes of pediatric CVI and, more specifically, understand the role of hypoxia as a cause of central visual damage. 3. Describe the different patterns and implications for future vision of hypoxic central visual damage occurring in preterm compared with term babies. 4. Know the clinical signs suggestive of CVI in children. 5. Discuss the involvement of visual functions other than visual acuity (ie, cognitive visual aspects) in CVI and correlate them with the likely affected neuroanatomic substrates. 6. Explain the value of using a multidisciplinary approach in rehabilitating children who have CVI early in their development. Vision loss caused by central nervous system damage often is referred to as CVI but also may be referred to as cerebral visual impairment or neurologic visual impairment. These terms refer to the fact that the primary defect may not necessarily be limited to the striate (primary visual) cortex and may affect other areas subserving vision, such as the visual associative cortex, optic radiations, and visual attention pathways. Although it is not yet certain which of these three terms best describes the visual deficit, it is clear that use of the label cortical blindness must be abandoned. That term not only evokes negative connotations for the child and the family but is inaccurate because in almost every instance, some degree of residual vision remains, and visual improvement can occur. CVI has become the greatest cause of pediatric visual impairment in developed countries (1)(2)(3)(4)(5) and is becoming increasingly prevalent in developing nations. CVI is a problem of increasing frequency for two primary reasons. First, the progress in neonatal care has resulted in improved survival of …

Chronologic Changes in Neonatal EEG Findings in Periventricular Leukomalacia

This study sought to clarify chronologic changes in neonatal electroencephalographic (EEG) findings in periventricular leukomalacia (PVL). We obtained serial EEG findings for all premature infants who were admitted to our hospital at gestational age of < or =33 weeks between 1997 and 2006. EEG recordings were obtained on days 1 to 4, 5 to 14, 15 to 28, 29 to 56, and 57 to 84. Abnormal EEG findings were classified as acute-stage abnormalities (ASAs) or chronic-stage abnormalities (CSAs) and were subclassified as mild, moderate, or severe. PVL was classified as noncystic, localized cystic, or extensive cystic. The final diagnosis of PVL was made through neurologic assessment and MRI findings at 24 months. Fifty-five infants were diagnosed as having PVL, including 23 with noncystic PVL, 9 with localized cystic PVL, and 23 with extensive cystic PVL. ASAs were observed most frequently on days 1 to 4 and were observed rarely thereafter in all groups. CSAs were observed most frequently on days 5 to 14, were most severe on days 5 to 14, and then resolved within 1 to 2 months in all groups. CSAs in patients with extensive cystic PVL were more severe and persisted longer, compared with other groups. ASA and CSA severity was correlated with PVL severity. EEG findings in PVL differed according to the severity of PVL and the time of recording. To detect PVL, > or =2 EEG recordings are recommended, 1 within 48 hours after birth, to detect ASAs, and 1 in the second week of life, to detect CSAs.

Progress in Periventricular Leukomalacia

Periventricular leukomalacia (PVL) is the predominant form of brain injury and the leading known cause of cerebral palsy and cognitive deficits in premature infants. The number of low-birth-weight infants who survive to demonstrate these neurologic deficts is increasing. Magnetic resonance imaging-based neuroimaging techniques provide greater diagnostic sensitivity for PVL than does head ultrasonography and often document the involvement of telencephalic gray matter and long tracts in addition to periventricular white matter. The neuropathologic hallmarks of PVL are microglial activation and focal and diffuse periventricular depletion of premyelinating oligodendroglia. Premyelinating oligodendroglia are highly vulnerable to death caused by glutamate, free radicals, and proinflammatory cytokines. Studies in animal models of PVL suggest that pharmacologic interventions that target these toxic molecules will be useful in diminishing the severity of PVL.

Impaired motor performance and movement quality in very-low-birthweight children at 6 years of age

Motor performance and movement quality were quantitatively examined (Zurich Neuromotor Assessment: timed motor performances and associated movements) in 87 prospectively enrolled very-low-birthweight (VLBW; <1250g) children (38 males, 49 females; mean birthweight 1016.2g [SD 141.5], range 720–1240g; mean gestational age 28.7wks [SD 2], range 25.7–33.4wks) at 6 years of age. All motor tasks were below the reference population: pure motor (median z-score) -0.46; adaptive fine motor (pegboard) -0.99; adaptive gross motor -0.88; static balance -0.48; and associated movements -1.90. All tasks correlated with the degree of neurological abnormalities (p≤0.004). VLBW children with no neurological abnormality also performed below the 10th centile and associated movements occurred more frequently than in the reference population (odds ratio 18, 95% confidence interval 6.7–47.9). Severity of periventricular leukomalacia and intraventricular haemorrhage assessed by ultrasound was associated with adaptive fine and gross motor tasks. We conclude that speed of motor performance and movement quality in particular were substantially impaired in VLBW children and are related to the degree of neurological abnormalities and neonatal cerebral injury.

Impaired motor performance and movement quality in very-low-birthweight children at 6 years of age

Motor performance and movement quality were quantitatively examined (Zurich Neuromotor Assessment: timed motor performances and associated movements) in 87 prospectively enrolled very-low-birthweight (VLBW; <1250g) children (38 males, 49 females; mean birthweight 1016.2g [SD 141.5]:, range 720-1240g; mean gestational age 28.7wks [SD 2], range 25.7-33.4wks) at 6 years of age. All motor tasks were below the reference population: pure motor (median z-score) -0.46; adaptive fine motor (pegboard) -0.99; adaptive gross motor -0.88; static balance -0.48; and associated movements -1.90. All tasks correlated with the degree of neurological abnormalities (p<or=0.004). VLBW children with no neurological abnormality also performed below the 10th centile and associated movements occurred more frequently than in the reference population (odds ratio 18, 95% confidence interval 6.7-47.9). Severity of periventricular leukomalacia and intraventricular haemorrhage assessed by ultrasound was associated with adaptive fine and gross motor tasks. We conclude that speed of motor performance and movement quality in particular were substantially impaired in VLBW children and are related to the degree of neurological abnormalities and neonatal cerebral injury.

Severe umbilical cord inflammation—a predictor of periventricular leukomalacia in very low birth weight infants

Chorioamnionitis has been associated with periventricular leukomalacia (PVL) in very low birth weight (VLBW) infants. We examined the association between the pathological severity of chorioamnionitis and PVL in VLBW infants. Thirty-four VLBW infants with PVL and 34 control infants matched for gestational age without a diagnosis of PVL or intraventricular hemorrhage were obtained from the Women and Infants' Hospital of Rhode Island's Neonatal Follow-up Clinic database. Placental samples, including the amnion/chorion, chorionic plate, and umbilical cord, were examined microscopically. Statistical analysis included Mantel–Haenszel chi-square, and Student's t-test. Severe inflammation in the umbilical cord was observed in 53% of infants with PVL and 32% without PVL ( p<0.05). Severe umbilical cord inflammation is one of the risk factors associated with the development of PVL in VLBW infants.

Intraventricular haemorrhage and periventricular leukomalacia in Nigerian infants of very low birth weight

Transfontanelle ultrasound scans were performed on 93 very low birth weight neonates admitted at the University of Ilorin Teaching Hospital (UITH), Ilorin, Nigeria, between January 1992 and April 1994, to ascertain the pattern and determinants of intraventricular haemorrhage and periventricular leukomalacia. Forty-seven percent of the infants had normal ultrasound; 22% had mild intraventricular haemorrhage (IVH), 7.5% had moderate to severe IVH and 23% had periventricular leukomalacia (PVL) and a 3.5 fold increase in IVH. Infants with apgar scores < or = 3 at 5 minutes had a 6-fold increase in IVH and 2-fold increase in PVL. There was no association with birth weight, gestational age, respiratory distress and place of delivery. Our study suggests that antepartum events proximate to delivery and the immediate post partum events play significant roles in the development of IVH and PVL in this population. Improvement in antenatal, labour and delivery, and immediate newborn care; including provision of basic resuscitation equipment and skills to midwives in the rural maternity centers will reduce the incidence and severity of IVH and PVL.

MRI measurements of the pons and cerebellum in children born preterm; associations with the severity of periventricular leukomalacia and perinatal risk factors.

Our purpose was to measure the size of the pons and cerebellum in preterm babies with periventricular leukomalacia (PVL), and to study their relationship with the severity of PVL and with perinatal risk factors. We examined 33 premature children, mean gestational age 31 weeks, range 26-36 weeks with PVL on MRI, and 27 full-term controls. On MRI at 0.4-5.5 years (mean 1.4 years) we measured the area of the corpus callosum and vermis, the anteroposterior diameter of the pons and the volume of the cerebellum. The area of the corpus callosum was used as a marker of white matter loss and PVL severity. All regional brain measurements except that of the vermis were significantly lower in patients than controls: corpus callosum (mm(2)): 239.6+/-92.5 vs 434.8+/-126.8, P <0.01; pons (mm): 14.8+/-3.0 vs 17.9+/-1.4, P <0.01]; cerebellum (cm(3)): 68.2+/-31.6 vs 100.6+/-28.3, P <0.01; vermis (mm(2)): 808.1+/-292.2 vs 942.2+/-246.2, NS. Significant reduction in the area of the vermis: 411.3+/-203.3 vs 935+/-252.6 mm(2); cerebellar volume: 16.3+/-12.5 vs 96.6+/-20.2 mm(3); and the diameter of the pons: 10.1+/-2.2 vs 17.5+/-1.3 mm ( P<0.01) were observed in seven children with gestational age < or =28 weeks, severe hypotension and large patent ductus arteriosus (PDA). There was a significant correlation between the duration of mechanical ventilation and the size of the vermis, pons and cerebellum (R=-0.65, -0.57 and -0.73, respectively, P <0.01).

Leucomalacia periventricular: secuelas neurológicas, radiológicas y repercusiones neuropsicológicas a largo plazo

Periventricular leukomalacia is an anatomical diagnosis made by neuroimaging technics, of hypoxic-ischemic origin and a characteristic neurological syndrome consisting in cerebral palsy, visual impairment and/or mental retardation. Is more frequently seen in pre-term infant (5-10%) as a spastic diplegia or tetraplegia. Real prevalence in a-term infant is unknown and is presented as hemiplegia. The correlation between periventricular leukomalacia severity and clinical severity is not absolutely clarified. Is not still known if we can predict mental retardation in an infant with periventricular leukomalacia on magnetic resonance image. This paper revise the clinical and radiological diagnosis of periventricular leukomalacia and shows the results of a series of 31 patients diagnosed radiologically of periventricular leukomalacia. We correlate the degree of neurological and mental disabilities with the severity of periventricular leukomalacia. We reach a good correlation between the severity of periventricular leukomalacia and the severity of neurological disturbances and mental retardation.

MRI findings in patients with spastic cerebral palsy. I: Correlation with gestational age at birth.

The authors studied MR images of the brain in 152 patients, aged 1 to 19 years (mean 3.3), who had spastic cerebral palsy (CP) and were attending two hospitals in Japan in 1993 and 1994. The relation was studied between the patients' gestational age at birth and their MRI findings, including the severity of periventricular leukomalacia (PVL) seen on MRI. In 119 of these patients, the CP was thought to be due to acquired, destructive brain injury. PVL was seen in 90 and posthemorrhagic porencephaly in 11. These preterm-type brain injuries were observed often in patients who had been born preterm but were also seen in those born at term. Fullterm-type border-zone infarct, bilateral basal ganglia-thalamic lesion, subcortical leukomalacia, and multicystic encephalomalacia were seen in 9, 14, 7, and 3 patients, respectively; these term-type brain injuries were observed only in patients born at or near term. Of the patients with PVL, 90% had been born preterm. Severe PVL was common in the patients whose gestational ages at birth were between 25 and 32 weeks; all patients with PVL who had been born at term had only mild PVL. The authors concluded that MRI findings for patients with spastic CP are closely correlated with gestational age.