Children born at 32 to 35 weeks with birth asphyxia and later cerebral palsy are different from those born after 35 weeks.

OBJECTIVE: The objectives of this study were to (1) establish the proportion of cerebral palsy (CP) that occurs with a history suggestive of birth asphyxia in children born at 32 to 35 weeks and (2) evaluate their characteristics in comparison with children with CP born at ⩾36 weeks with such a history. STUDY DESIGN: Using the Canadian CP Registry, children born at 32 to 35 weeks of gestation with CP with a history suggestive of birth asphyxia were compared with corresponding ⩾36 weeks of gestation children. RESULTS: Of the 163 children with CP born at 32 to 35 weeks and 738 born at ⩾36 weeks, 26 (16%) and 105 (14%) had a history suggestive of birth asphyxia, respectively. The children born at 32 to 35 weeks had more frequent abruptio placenta (35% vs 12%; odds ratio (OR) 4.1, 95% confidence interval (CI) 1.5 to 11.2), less frequent neonatal seizures (35% vs 72%; OR 0.20, 95% CI 0.08 to 0.52), more frequent white matter injury (47% vs 17%; OR 4.3, 95% CI 1.3 to 14.0), more frequent intraventricular hemorrhage (IVH) (40% vs 6%; OR 11.2, 95% CI 3.4 to 37.4) and more frequent spastic diplegia (24% vs 8%; OR 1.8, 95% CI 1.2 to 12.2) than the corresponding ⩾36 weeks of gestation children. CONCLUSIONS: Approximately 1 in 7 children with CP born at 32 to 35 weeks had a history suggestive of birth asphyxia. They had different magnetic resonance imaging patterns of injury from those born at ⩾36 weeks and a higher frequency of IVH. Importantly, when considering hypothermia in preterm neonates with suspected birth asphyxia, prospective surveillance for IVH will be essential.

Clinical profile of children with cerebral palsy born term compared with late- and post-term: a retrospective cohort study.

OBJECTIVE: To determine whether cerebral palsy (CP) risks factors, neurological subtype, severity and co-morbidities differ between early/full-term-born children with CP compared with those born late/post-term. DESIGN: Retrospective cohort study. SETTING: Children with CP born between 1998 and 2014, residing in Canada, and registered in the Canadian Cerebral Palsy Registry (CCPR) (n = 1691), a database with information from 15 participating centres across six Canadian provinces. POPULATION: Children with CP from the CCPR born at 37 weeks of gestation and later (n = 802). METHODS: The clinical profile of children with CP born at 37-40 weeks of gestation was compared with those born at 41 weeks and later using the Pearson chi-square test (or Fisher's exact test) for univariate analyses of categorical data. A P value <0.05 was considered significant a priori. MAIN OUTCOME MEASURES: CP neurological subtype, Gross Motor Function Classification System (GMFCS) severity, risk factors and co-morbidities. RESULTS: Neonatal encephalopathy was found in 23.9% of children with CP born early/full-term and in 33.6% of those born late/post-term (P = 0.026). Neonatal hyperbilirubinaemia was found in 10.2% of children born in the earlier period and in 2.6% of those born in the later period (P = 0.008). Apgar score at 5 minutes, but not 10 minutes, was significantly higher in the early/full-term group (9) compared with its late/post-term counterpart (7; P = 0.046). Rates of CP subtype, severity (GMFCS) and co-morbidities did not differ significantly between the two gestational periods. CONCLUSIONS: In children with CP, neonatal encephalopathy was significantly less frequent and neonatal hyperbilirubinaemia was significantly more frequent in those born early/full-term compared with their later-born counterparts. However, clinical outcomes of CP were not significantly different between these two gestational epochs. TWEETABLE ABSTRACT: Children with cerebral palsy born early/full-term have similar outcomes to those born late/post-term.

Evidence report: Genetic and metabolic testing on children with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.

OBJECTIVE: To systematically review the evidence concerning the diagnostic yield of genetic and metabolic evaluation of children with global developmental delay or intellectual disability (GDD/ID). METHODS: Relevant literature was reviewed, abstracted, and classified according to the 4-tiered American Academy of Neurology classification of evidence scheme. RESULTS AND CONCLUSIONS: In patients with GDD/ID, microarray testing is diagnostic on average in 7.8% (Class III), G-banded karyotyping is abnormal in at least 4% (Class II and III), and subtelomeric fluorescence in situ hybridization is positive in 3.5% (Class I, II, and III). Testing for X-linked ID genes has a yield of up to 42% in males with an appropriate family history (Class III). FMR1 testing shows full expansion in at least 2% of patients with mild to moderate GDD/ID (Class II and III), and MeCP2 testing is diagnostic in 1.5% of females with moderate to severe GDD/ID (Class III). Tests for metabolic disorders have a yield of up to 5%, and tests for congenital disorders of glycosylation and cerebral creatine disorders have yields of up to 2.8% (Class III). Several genetic and metabolic screening tests have been shown to have a better than 1% diagnostic yield in selected populations of children with GDD/ID. These values should be among the many factors considered in planning the laboratory evaluation of such children.

Current understandings and challenges in the management of cerebral palsy.

Cerebral palsy is a long recognized ''symptom-complex'' that is the most common cause of childhood physical impairment. A neurodevelopmental disability sub-type, it has an essential core of objective neuro-motor impairment and its present conceptualization includes explicit recognition of activity limitations and varying associated co-morbidities. This review explores this disorder's incidence, risk factors, complementary classification schemes, clinical evaluation, spectrum of heterogeneous causal etiologies, management challenges related to associated spasticity, movement disorders and co-existing conditions, outcomes and lifespan related changes. The goal of this review was to improve the clinician's understanding of what is presently known regarding cerebral palsy, its clinical challenges and appropriate case management.

Childhood absence epilepsy requiring more than one medication for seizure control.

INTRODUCTION: Absence epilepsy is the most common primary generalized epilepsy syndrome encountered in pediatric practice. Treatment is pharmacologically specific and usually successful with a single medication. The objective of this study was to identify any clinical or electroencephalographic features at initial presentation in a consecutive cohort of children with absence epilepsy that may be associated with the need for a second medication. METHODS: A computerized pediatric neurology database (1991-2007 inclusive) was retrospectively searched for all patients with typical absence seizures, 3 Hz spike and wave on EEG and no apparent symptomatic etiology who were over the age of two years at seizure onset with at least one year of follow-up. All such children were then divided into two groups; a) those requiring a single medication for seizure control (Group 1), and b) those requiring two medications for seizure control despite optimal management with the initial medication as determined by serum drug monitoring (Group 2). Clinical and electrographic features evident at diagnosis were then contrasted between Group 1 and 2. RESULTS: Seventy-five children with absence seizures were initially identified with 52 meeting the study's inclusion and none of the exclusion criteria. Of these 52 children, 43 required a single medication for seizure control (Group 1), while 9 required two or more medications for seizure control (Group 2). A significant difference (p < 0.05) was apparent between Group 1 and 2 with respect to gender (16/43 males vs 8/9 males) and mean age of diagnosis (8.19 years +/- 3.00 vs 6.06 years +/- 2.22). Age of onset of seizures, interval duration of seizures prior to treatment initiation, duration of seizures, presence of automatisms, family history, presence of co-morbid conditions and EEG findings were not found to be significantly different between the two Groups. CONCLUSIONS: Male gender and an earlier age of diagnosis is associated with the need for two medications for seizure control in children with absence epilepsy. This observation may suggest the need for more intensive early programmatic follow-up for young male children with newly diagnosed absence epilepsy to effect more rapid attainment of seizure control.

Array comparative genomic hybridization in global developmental delay.

OBJECTIVE: Array-based comparative genomic hybridization (array CGH) is an emerging technology that allows for the genome-wide detection of DNA copy number changes (CNC) such as deletions or duplications. In this study, array-based CGH was applied to a consecutive series of children with previously undiagnosed non-syndromal global developmental delay (GDD) to assess potential etiologic yield. METHODS: The children in this study were drawn from a previously reported consecutive series of children with well-defined GDD. Almost all subjects had undergone prior karyotyping and neuroimaging studies with non-diagnostic results. Array-based CGH was undertaken using the SignatureChip(R) (1887 BACs representing 622 loci) with abnormalities verified by subsequent FISH analysis and testing of parents to distinguish between pathogenic and familial non-pathogenic variants. RESULTS: On CGH analysis in our study, 6 of 94 children (6.4%) had a causally related pathogenic CNC. Three were sub-telomeric in location. An analysis of a variety of clinical factors revealed that only the presence of minor dysmorphic features (<3) was predictive of etiologic yield on CGH analysis (4/26 vs. 2/68, P = 0.05). Severity of delay was not found to be predictive. INTERPRETATION: In children with non-syndromal GDD, array-based CGH has an etiologic yield of 6.4%. This suggests that this emerging technology may be of diagnostic value when applied subsequent to detailed history, physical examination, and targeted laboratory testing. Array CGH may merit consideration as a first-tier test in the context of a child with unexplained GDD.

Early rehabilitation service utilization patterns in young children with developmental delays.

OBJECTIVE: There is a growing demand for interdisciplinary rehabilitation services for children with developmental disabilities, however, information is lacking on service utilization patterns. This study describes the type, frequency and location of initial rehabilitation services provided to young children recently diagnosed with a developmental delay. METHODS: A telephone survey was conducted on a consecutive cohort of children referred to subspecialists for the first investigation of their delay. RESULTS: Out of 129 respondents, only 30 (23%) did not receive any rehabilitation services within the first 6 months following medical evaluation, and 61 (47%) received two or more services. Services were provided most frequently in the hospital setting, especially for occupational or physical therapy (73-80%). Services were less often rendered in a rehabilitation centre (5-13%) or in the community (< 10%). Services were provided privately for a subset, particularly for speech language pathology and psychology (34% and 30% respectively). Interventions were typically given weekly or bi-weekly. Children receiving physical therapy were significantly younger whereas children referred to speech language pathology were older than the age of our cohort at intake. Overall, parents' educational level did not influence services received with the exception of private services, which were more likely to occur in children of fathers with university education. CONCLUSIONS: The findings would suggest that long waiting times and lack of resources may limit access to comprehensive services, particularly in community settings. Service utilization patterns were not consistent within types of developmental disability, suggesting that formal and co-ordinated interdisciplinary programmes are not in place for this population of interest.

Long-term developmental outcome of asphyxiated term neonates.

Asphyxia remains one of the main causes of later disability in term infants. Despite many publications identifying possible predictors of outcome in this population of interest, little is known of the long-term developmental outcome of asphyxiated term neonates. Observational studies have largely focused on short-term outcomes, with an emphasis on significant neurologic sequelae and intellectual impairments. This article reviews the literature that has described the developmental outcome of asphyxiated term newborns. As part of this review, we have also highlighted the evolution of the definition of asphyxia and delineated appropriate markers that should be used in future research on this population.

Functional limitations in young children with congenital heart defects after cardiac surgery.

UNLABELLED: With the recent dramatic decline in mortality rates of infants undergoing open-heart surgery (OHS), there is growing concern regarding neurodevelopmental sequelae. Outcome studies have primarily focused on delineating developmental impairments; however, the impact on function and family burden has not been investigated. The objective of this study was to determine the prevalence of functional limitations and burden of care of young children with congenital heart defects (CHD) after OHS. STUDY DESIGN: One hundred thirty-one eligible infants with CHD undergoing their first OHS were recruited prospectively. Patients were assessed pre- and postoperatively, and again 12 to 18 months after surgery. Functional assessments included the WeeFIM (Functional Independence Measure) and the Vineland Adaptive Behavior Scale. RESULTS: For the WeeFIM, mean quotients were 84.3 +/- 23.8 (self-care), 77.2 +/- 30.0 (mobility), and 92.4 +/- 27.8 (cognition), with an overall quotient of 83.8 +/- 23.4. Only 21% of the cohort was functioning within their expected age range. Moderate disability was noted in 37%, while only 6% demonstrated a severe disability. For the Vineland scale, mean score for daily living skills was 84.4 +/- 17.6, and 80.3 +/- 15.9 for socialization. Functional difficulties in daily living skills were documented in 40%, whereas >1/2 had poor socialization skills. Factors enhancing risk for functional disabilities included perioperative neurodevelopmental status, microcephaly, length of deep hypothermic circulatory arrest, length of stay in the intensive care unit, age at surgery, and maternal education. CONCLUSIONS: The high prevalence of functional limitations and dependence in activities of daily living is currently underappreciated in the clinical setting, and deserves additional attention by pediatricians and developmental specialists.

Julius Hallervorden's wartime activities: implications for science under dictatorship.

The eponym Hallervorden-Spatz syndrome recalls Julius Hallervorden's and Hugo Spatz's original description of this pediatric neurodegenerative disorder. Julius Hallervorden's important contribution to the practice of neuropathology over a long career cannot be underestimated. However, his work as a pathologist during the Third Reich put him in close proximity with the implementation of biologic solutions (i.e., euthanasia) targeting those individuals with significant intellectual or physical disabilities in chronic-care facilities. The Nazi program of active euthanasia provided a scientific opportunity to gain quick access to pathologic materials. This opportunity was recognized and used by Hallervorden to achieve personal scientific objectives and research efforts. These efforts resulted in a number of postwar scientific publications using materials obtained through the euthanasia program. The participation of distinguished academic physicians in such a program provides a cautionary tale of the potential results of ethical compromise and the effects of the abrogation of personal autonomy in the setting of a totalitarian dictatorship.

Profile of referrals for early childhood developmental delay to ambulatory subspecialty clinics.

The objective of this study was to determine the profile and pattern of referral to subspecialty clinics of young children with suspected developmental delay together with the factors prompting their referral. All children under 5 years of age referred to either developmental pediatrics or pediatric neurology clinics at a single tertiary hospital over an 18-month period were prospectively identified. Standardized demographic and referral information were collected at intake, final developmental delay subtype diagnosed was identified, and referring physicians were surveyed regarding factors prompting referral. A total of 224 children met study criteria. There was a marked male preponderance (166/224), especially among those with either cognitive or language delay. Two delay subtypes, global developmental delay and developmental language disorder, accounted for two thirds of the diagnoses made. For slightly more than one third of the children (75/224), the delay subtype diagnosed following specialty evaluation was different from that initially suspected by the referring physician. A mean delay of 15.5 months was observed for the cohort as a whole between initial parental concern and specialty assessment. For referring physicians, the major factor prompting referral was the severity of the observed delay. The most important aspects of the specialty evaluation according to referral sources were the identification of a possible etiology and confirmation of delay. A profile of referrals and the rationale thereof for a cohort of children with suspected developmental delay is presented that, although locale specific, has implications for service provision and training.

Diagnostic profile of neonatal hypotonia: an 11-year study.

The profile of disorders presenting with neonatal hypotonia to the neonatal intensive care unit has not been studied previously. An 11-year retrospective cohort study of neonates, who were identified through computer database records and were admitted to the Neonatal Intensive Care Unit from January 1989 to December 1999 at the Montreal Children's Hospital (Montreal, Québec), is presented. The final diagnoses, tests obtained, and outcome were determined from a structured review of the subject's hospital record. The database search generated 95 records, of which 50 neonates met the inclusion criteria. The hypotonia was classified as central in 33 patients (66%) and peripheral in 17 (34%). Hypoxic-ischemic encephalopathy (n = 13), Prader-Willi syndrome (n = 6), myotonic dystrophy (n = 6), other muscle disorders (n = 6), chromosomal disorders (n = 4), and peripheral nerve disorders (n = 3) were the most common diagnoses. The genetic tests of highest yield were fluorescent in situ hybridization for Prader-Willi syndrome, DNA methylation studies for Prader-Willi syndrome, trinucleotide repeat testing for myotonic dystrophy, and karyotype analysis. A diagnostic approach is proposed based on the results.

Association between electroencephalographic findings and neurologic status in infants with congenital heart defects.

Neurologic status is of concern in infants with congenital heart defects undergoing open heart surgery. The association between perioperative electroencephalography (EEG) with acute neurologic status and subsequent outcome was examined in a cohort of 60 infants. Preoperative EEG and neurologic examinations were performed within 1 to 2 days prior to surgery (n = 27) and postoperatively (n = 47). Prior to surgery, 15 of 27 infants had normal EEG, whereas 5 had epileptiform activity and 9 had disturbances in background activity that were primarily moderate (8/9) and diffuse (7/9). Postoperatively, only 17 of 47 infants had normal recordings. Newborns (<1 month) were more likely (P< .001) to demonstrate EEG abnormalities than infants. Epileptiform activity was documented in 15, whereas 28 had background abnormalities that were moderate-severe (22/28) and diffuse (20/28) in most. Epileptiform activity prior to surgery was always associated with an abnormal neurologic examination, and this association persisted postoperatively (86%). Moderate to severe background abnormalities in the postoperative EEG was also strongly associated with acute neurologic abnormalities (93%). Severe background abnormalities (n = 5) were 100% predictive of death or severe disability. Long-term follow-up revealed that all children with normal postoperative EEGs had positive neurologic outcomes (P = .04); however, there were many false positives. Perioperative EEG abnormalities increased the likelihood for acute neurologic findings, whereas normal recordings following surgery were reassuring with regard to a favorable outcome.

Etiologic yield of autistic spectrum disorders: a prospective study.

At present, the etiologic yield in community-derived samples of young children with an autistic spectrum disorder is not known. To address this question, all young children (under 5 years of age) referred for an initial assessment to ambulatory pediatric neurology or developmental pediatric clinics at a tertiary university center over an 18-month period for a suspected developmental delay were prospectively identified. Specific diagnostic testing was left to the discretion of the evaluating physician. In all, 50 children with an autistic spectrum disorder were assessed. Detailed history or physical examination was informative with respect to suggesting the possibility of an underlying etiology in a minority (10/50,20%). Genetic studies (FMR-1, karyotype), electroencephalography (EEG), and neuroimaging were carried out in a majority (42/50, 34/50, and 33/50, respectively) of the children, for the most part on a screening rather than an indicated basis (31/42, 34/34, and 28/33, respectively). Etiologic yield was low (1/50, 2%), with only a single child identified with a possible Landau-Kleffner variant on sleep EEG tracing. The results suggest an evaluation paradigm with reference to etiologic determination for young children with autistic spectrum disorder that does not presently justify metabolic or neuroimaging on a screening basis. Recurrence risk and treatment implications, however, suggest that strong consideration be given to genetic (FMR-1, karyotype) testing and EEG study despite a relatively low yield.

The pediatric neurologist as expert witness with particular reference to perinatal asphyxia.

The frequency of litigation related to alleged medical malpractice is increasing in Canada. For the neurologist, involvement in such litigation most often takes place in the context of acting as an expert witness and, for the pediatric neurologist, the most common clinical situation for which expertise is requested is that of possible perinatal asphyxia. The medical expert's primary role is to provide necessary guidance and assistance to the court, which may permit the rendering of decisions that are scientifically valid. This article will review the attributes of the medical expert witness. Aspects of perinatal asphyxia cases under litigation that commonly require the assistance of pediatric neurology expertise such as etiology, timing, extent of disability and life expectancy will also be reviewed in detail. The aim is to provide for the neurologist a clearer understanding of the responsibilities inherent in this increasing professional role.

Etiologic determination of childhood developmental delay.

To determine the etiologic yield in young children with developmental delay referred to sub-specialty clinics for evaluation. Over an 18-month period, all children less than 5 years of age referred to the ambulatory pediatric neurology or developmental pediatrics clinics of the Montreal Children's Hospital for initial evaluation of a suspected developmental delay were enrolled. Features evident on history or physical examination were determined at intake as were the laboratory tests (and their rationale) requested by the evaluating physicians. Six months post initial assessment, detailed chart review was undertaken to determine if an etiology was found and the basis for such a determination. Bivariate and multivariate logistic regression was used to test for associations between factors present at intake and successful ascertainment of an underlying etiology. Two hundred and twenty-four children met study criteria. Etiologic yield varied across childhood developmental delay subtypes, and was 44/80 for global developmental delay [GDD] (55%), 13/22 for motor delay [MD] (59.1%), 3/72 for developmental language disorders [DLD] (4.2%), and 1/50 for autistic spectrum disorders [ASD] (2%). For GDD, the presence of historical features or findings on physical examination was associated with greater likelihood for successful etiologic determination with the following items significant in multiple logistic regression analysis; microcephaly, antenatal toxin exposure, focal findings. For MD, physical findings or the co-existence of a cerebral palsy symptom complex predicted a successful search for etiology. For both groups, the severity of the delay did not predict etiologic yield. For both groups, a small number of etiologic categories accounted for the majority of diagnoses made. Etiologic yield in childhood developmental delay is largely dependent on the specific developmental delay subtype. Paradigms for systematic evaluation of this common child health problem can be suggested, however they await validation.

Etiologic yield of single domain developmental delay: a prospective study.

OBJECTIVE: To determine the etiologic yield in young children with single domain developmental delay (either developmental language disorder or isolated motor delay) after a specialty diagnostic evaluation. METHODS: During an 18-month period, all children <5 years of age, who were consecutively referred to pediatric neurology or developmental pediatric clinics at a single tertiary pediatric center, were prospectively enrolled. Etiologic yield was determined after completion of clinical assessments and selected laboratory studies requested by the evaluating physician. RESULTS: Seventy-two children (60 boys) were found to have a developmental language disorder, and 22 children (11 boys) had isolated motor delay, of whom 6 had an associated diagnosis of cerebral palsy. An etiologic diagnosis was rarely made in the children with developmental language disorder (3/72, 4.1%). Laboratory investigations (metabolic, cytogenetic, imaging), aside from audiometry, were uniformly uninformative. In those children with isolated motor delay, an etiology was apparent in more than half (13/22, 59%). Slightly more than half (7/13, 54%) of etiologies identified in this group were potentially preventable. Successful etiologic determination in children with motor delay often had an impact on recurrence risk estimation, medical management, or specific therapy offered (8/13, 62%). The presence of physical findings on initial assessment was found to be highly predictive of successful etiologic determination in children with isolated motor delay (13/17 vs 0/5, P =.002). CONCLUSION: Etiologic yield differs substantially according to the subgroup of single domain developmental delay.

Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery.

BACKGROUND: Neurodevelopmental disabilities in children with congenital heart defects (CHDs) have been primarily attributed to intraoperative events without consideration of preoperative and postoperative factors. OBJECTIVE: To describe the preoperative and postoperative neurodevelopmental status of newborns and infants with CHDs. STUDY DESIGN: One hundred thirty-one children (56 newborns and 75 infants) were evaluated before and after surgery by using standardized neurobehavioral (newborn) and motor assessments (infant) and neurologic examinations. RESULTS: In newborns, neurobehavioral abnormalities were documented in >50% before surgery, with abnormalities persisting in most after surgery. In infants, neurodevelopmental abnormalities were observed in 38% before surgery. There was a significant association between preoperative and postoperative neurodevelopmental status, with status remaining unchanged in most. Newborns with acyanotic heart lesions were more likely to demonstrate neurologic compromise than those with cyanotic defects. For infants, arterial oxygen saturations <85% were significantly associated with an abnormality. There was a trend for a longer circulatory arrest time to be associated with greater risk for neurologic sequelae in newborns, whereas prolonged cardiopulmonary bypass was an important risk factor for infants. CONCLUSIONS: Neurodevelopmental abnormalities are common in young infants with CHDs and are often present before open heart surgery. These developmental concerns are clinically underappreciated. Early systematic developmental screening may be warranted in this population of interest.

Neuromotor spectrum of periventricular leukomalacia in children born at term.

The spectrum of neuromotor abnormalities of term children with periventricular leukomalacia (PVL) has never been specifically defined. We report 12 term children with PVL to delineate its long-term clinical correlates. A retrospective review of a standardized computer database and files of a single pediatric neurologist during a 7-year period was performed. The imaging studies were reviewed independently by two neuroradiologists. The mean age of the patients at the initial neurologic assessment was 24. 4 months (range 5-60); nine were males. The reason for the assessment was developmental delay in 10 (83.3%), seizure in one, and attention-deficit-hyperactivity disorder in one. Three children (25%) had normal motor examinations, three (25%) were hypotonic, three (25%) had spastic diplegia, two (16.7%) had spastic quadriplegia, and one (8.3%) had fine-motor abnormalities. Nine children (75%) had developmental delay (severe global delay in six), and two children (16.7%) had epilepsy; electroencephalograms were abnormal in six (50%). PVL was mild in five (41.7%), moderate in two (16.6%), and severe in five (41.7%) children. Four of eight children with global developmental delay had enlargement of cerebral sulci in addition to white matter changes. PVL in term children presents with a spectrum of neurologic abnormalities, particularly developmental delay and heterogeneous motor findings not limited to classic spastic diplegia. The clinician should consider the diagnosis of PVL in the context of term children with developmental delay and motor abnormalities, even in the absence of perinatal difficulties.