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Few reports address the role of decompressive craniectomy in children with space-occupying cerebral edema attributable to severe ischemic infarction of the posterior cerebral arterial circulation. We describe four children with posterior circulation arterial ischemic stroke who required decompressive craniectomy. These children accounted for 11% of all cases of posterior circulation ischemic stroke at our institution from 2002-2010. Three manifested large, cerebellar hemispheric infarcts, and one manifested a large, temporo-occipital posterior cerebral artery infarct. Deterioration occurred within 72 hours of stroke onset. Two patients demonstrated minimal functional deficits at follow-up, and two demonstrated moderate deficits with functional limitations. Because decompressive craniectomy can be lifesaving in children with severe posterior circulation arterial ischemic stroke, early neurosurgical referral should be considered.

Childhood stroke is increasingly recognized as a major cause of childhood morbidity and mortality; however, limited information exists regarding neurobehavioral sequelae. Executive function (EF), important for problem solving, reasoning, social awareness, and adaptive behavior, may be particularly vulnerable to early brain lesions such as stroke, due to its protracted development. This study investigated: (i) the impact of childhood stroke on EF; and (ii) the impact of lesion size on EF outcome. Twenty-eight children diagnosed with stroke at least 12 months prior to assessment were recruited. Neurobehavioral assessment focused on cognitive and behavioral aspects of EF. Lesion volume was determined from standard ratings of brain pathology based on magnetic resonance imaging (MRI) scans. Deficits in cognitive aspects of EF were detected in attentional control, cognitive flexibility and information processing. Difficulties in behavioral aspects of EF were most striking, with problems identified across a wide range of behaviors. Lesion size impacted on EF, with large lesions (greater than 25% of brain volume) proving the most detrimental.

Objective To describe presumptive risk factors (RFs) for childhood arterial ischemic stroke (AIS) and explore their relationship with presentation, age, geography, and infarct characteristics. Methods Children (29 days–18 years) were prospectively enrolled in the International Pediatric Stroke Study. Risk factors, defined conditions thought to be associated with childhood AIS, were divided into 10 categories. Chi-square tests were used to compare RFs prevalence across regions and age; logistic regression was used to determine whether RFs were associated with particular features at presentation or infarct characteristics. Results A total of 676 children were included. No identifiable RFs was present in 54 (9%). RFs in others included arteriopathies (53%), cardiac disorders (CDs) (31%), infection (24%), acute head and neck disorders (AHNDs) (23%), acute systemic conditions (ASCs) (22%), chronic systemic conditions (CSCs) (19%), prothrombotic states (PTSs) (13%), chronic head and neck disorders (CHNDs) (10%), atherosclerosis-related RFs (2%), and other (22%). Fifty-two percent had multiple RFs. There was lower prevalence of arteriopathy in Asia, lower prevalence of CSCs in Europe and Australia, higher prevalence of PTSs in Europe, and higher prevalence of ASCs in Asia and South America. Prevalence of CDs and ASCs was highest in preschoolers, arteriopathies in children 5 to 9 years old, and CHNDs were highest in children aged 10 to 14 years. Arteriopathies were associated with focal signs and ASCs, CHNDs, and AHNDs with diffuse signs. Arteriopathies, CSCs, and ASCs were associated with multiple infarcts and CDs with hemorrhagic conversion. Interpretation RFs, especially arteriopathy, are common in childhood AIS. Variations in RFs by age or geography may inform prioritization of investigations and targeted preventative strategies. Ann Neurol 2011;69:130–140.

Child stroke is a major cause of death in children, although limited information exists on neurobehavioral functioning of stroke survivors. Executive function (important for goal-directed behavior) is thought to be vulnerable to early insults such as stroke because of its widespread representation in the immature brain. This study investigated the impact of lesion location on executive skills. Twenty-eight children diagnosed with stroke at least 18 months before assessment were recruited. Lesion characteristics were coded from magnetic resonance imaging (MRI) scans. Neurobehavioral assessment focused on cognitive and everyday executive skills. Deficits were found in the context of overall normal intellectual functioning (M = 91.60; SD = 19.40). Generally, insults involving frontal and extra-frontal regions impacted equally on cognitive performance. Everyday deficits were marginally more prominent following frontal insult. Subcortical frontal lesions were associated with impairments in everyday executive skills. Results provide further support for the diffuse representation of executive function in the immature brain.

Therapy programmes aim to provide services that are family-centred and address the specific needs of children. The Canadian Occupational Performance Measure (COPM) and the Perceived Efficacy and Goal Setting system (PEGS) are two measures that are available to assist in determining therapy priorities for children and their parents; however, the use of these measures has not been documented for children who have sustained a stroke. This project aimed to describe the functional concerns identified by children and their parents following paediatric stroke.A cross-sectional design was used. A total of 26 children were recruited from a paediatric stroke outpatient clinic, and functional concerns were identified using either the COPM or the PEGS.Children and their parents identified similar issues. Of the 26 participants, 23 (88.5%) reported ongoing functional concerns at three months or more following stroke, whereas three participants did not identify any ongoing functional concerns. Functional concerns were grouped into categories of self-care, productivity and leisure as outlined in the Canadian Model of Occupational Performance. Concerns were identified across all functional domains by both children and their parents.The COPM and PEGS provided useful information about functional issues that are important to children and their parents following paediatric stroke. Use of these client-centred measures provides an opportunity to better understand the impact of paediatric stroke on children's functional abilities, and allows greater scope for service provision and planning for this group of children.

Background and Purpose— Limited data exist on childhood posterior circulation arterial ischemic stroke (PCAIS). We describe clinical and radiological features of childhood PCAIS to determine whether there are differences in infarct topography, vascular abnormalities, risk factors, and stroke subtypes when compared to adults. Methods— Children with radiologically confirmed PCAIS were prospectively identified from August 2002 to February 2008. Infarcts were divided into proximal, middle, and distal posterior circulation segments utilizing an adult topographical classification system. Vascular abnormalities were described in terms of location, severity, and evolution over time. A pediatric modification of the TOAST classification system was used to define stroke subtypes. Results— Twenty seven (37%) of 73 children recruited to our registry had 34 radiologically confirmed PCAIS events. Infarct location was distal (25), middle (2), proximal (1), and multiple segments (6). Fourteen events were associated with single infarcts and 20 were associated with multiple infarcts. Magnetic resonance angiography was abnormal in 16 of 25 children with PCAIS at presentation with stenosis (8) or occlusion (8). Vascular lesions progressed (5), transiently worsened before stabilizing (2), remained stable or improved (8), or normalized (1) over 12 months. Stroke subtypes included nonprogressive steno-occlusive cerebral arteriopathy (9), cardioembolic (4), dissection (3), Moyamoya syndrome (3), other determined (4), probable (1), and undetermined etiology (3). Fifty-two percent of children had recurrent posterior (6) or anterior (8) strokes. Conclusions— Nonprogressive arteriopathies are the most common cause of childhood PCAIS, usually affecting distal segments. Atherosclerosis-related risk factors do not play an important role in stroke causation. PCAIS is frequently associated with recurrent events.

The aim of this study was to describe the frequency, risk factors, manifestations, and outcome of epilepsy in children with hemiplegic cerebral palsy (CP) due to perinatal arterial ischaemic stroke (AIS).The study group comprised 63 participants (41 males, 22 females) from a population-based CP register whose brain imaging showed perinatal AIS. Information collected included occurrence of neonatal seizures, family history of epilepsy, motor function and epilepsy onset, treatment, and outcome. Electroclinical findings were classified according to seizure semiology, seizure type, and epilepsy syndrome.Mean age of participants at the time of study was 10 years 6 months (SD 4 y 7 mo, range 4-20 y). Gross Motor Function Classification System levels I and II were reported in 96% of participants, and Manual Ability Classification System levels I and II were reported in 79% of children. Thirty-four children (54%) developed epilepsy. Term delivery and more severe motor impairment were associated with epilepsy, but neonatal seizures and family history of epilepsy were not. Initial seizures were epileptic spasms, focal seizures, or myoclonic seizures. Focal seizure semiology suggested Rolandic or occipital seizure origin in the majority of children. Focal epileptic discharges in children with focal seizures had features of idiopathic partial epilepsy. Only 15% of children had active epilepsy 10 years after onset.Despite a high incidence of epilepsy in children with hemiplegic CP due to AIS, the prognosis for seizure remission is good. Many children have clinical features, electroencephalography findings, and remission typical of idiopathic partial epilepsy.

Stroke is an important cause of mortality and long-term morbidity in children. The aetiology of stroke in childhood differs from that of adults, with vasculopathies and congenital heart disease being the most commonly identified risk factors. Recognition and diagnosis are often delayed, limiting access to acute medical interventions such as thrombolysis. Optimal management of stroke in children is still not known and existing guidelines are at the level of expert consensus. Interdisciplinary childhood stroke programmes are required to meet the needs of this population and to contribute to the development of evidence-based therapies.

The clinical and radiological features of childhood acute transverse myelitis are compared to those of acute disseminated encephalomyelitis with spinal cord involvement in 22 children with acute transverse myelitis and 12 children with acute disseminated encephalomyelitis with spinal cord involvement. Children with acute transverse myelitis were more likely to have a sensory level (55%) and areflexia. Sixty-eight percent of the children with acute transverse myelitis, and 92% of children with acute disseminated encephalomyelitis had longitudinally extensive transverse myelitis. Demyelination was more extensive in acute disseminated encephalomyelitis (mean 15.6 vertebral segments) than in acute transverse myelitis (mean 8.0 vertebral segments). The outcome was normal to good in 82% with acute transverse myelitis and in 100% with acute disseminated encephalomyelitis. Persistent bladder dysfunction was uncommon in both. Poor prognostic factors in acute transverse myelitis are flaccid paraparesis, respiratory failure, and age less than 6 months. These clinical and radiological differences suggest acute transverse myelitis and acute disseminated encephalomyelitis are separate entities.

Aim: Hypoglycaemic seizures are common in children with diabetes and electroencephalogram abnormalities are well recognised in this patient group. Elevated antibodies to glutamic acid decarboxylase, a major auto-antigen in Type 1 diabetes, are also implicated in a number of neurological disorders. Despite these associations, the question of whether children with diabetes are more prone to epilepsy, possibly as a result of lowered seizure threshold, has not been previously studied. We aimed to determine the prevalence and type of epilepsy in a large paediatric diabetes clinic. Methods: An audit by chart review was carried out at the Diabetes Clinic at the Royal Children's Hospital, Melbourne, Australia. Clinical, demographical, biochemical, EEG and neuro-imaging data were recorded. Results: Of 1384 children and adolescents aged 0–19 years with Type 1 diabetes, we identified 12 with active epilepsy (prevalence of 8.7/1000), the majority of whom had idiopathic generalised epilepsy and benign focal epilepsy of childhood. These findings are similar to those in the general population. Conclusion: Childhood epilepsy is no more frequently encountered in children and adolescents with Type 1 diabetes than in the general paediatric population.

We report a 14-year-old girl with a delayed diagnosis of stroke, highlighting one of the most significant obstacles to offering acute thrombolytic therapies in teenagers and children. Feasibility of treatment is further limited by a lack of dosage and safety data in the paediatric population. Improved community awareness and more rapid recognition of stroke may reduce lag time to diagnosis.

Publisher Summary The chapter discusses the occurrence of stroke among women, ethnic groups, young adults, and children. There are a number of different groups of people among whom the incidence and etiology of stroke differ. Incidence of stroke is lower among women than men, and is lower among young adults and children. Women have a number of unique risk factors for stroke that involve their reproductive capacity, although other factors may also be involved. People aged less than 45 years with stroke require special consideration. The frequencies of underlying etiology differ from the older stroke population and a greater breadth of investigation is required. Although considered rare, stroke is more common than brain tumors in children. Arteriopathies are the most commonly identified risk factor for arterial ischemic stroke, followed by cardiac and thromboembolic disorders. Neurological sequelae are common in arterial ischemic stroke and sinovenous thrombosis.

Stroke is a major cause of disability and death in children. It can have devastating consequences for families and enormous costs to society. Although considered rare, stroke is more common in children than brain tumours. Ten percent of children suffering stroke will die as a result, and at least 50% of survivors are left with significant neurological disabilities, learning difficulties or seizures.This article discusses risk factors, investigation, management and outcomes of ischaemic and haemorrhagic stroke in neonates and children.Arteriopathies and cardiac disease are the commonest risk factors for childhood arterial ischaemic stroke (AIS). The cause of perinatal AIS is poorly understood, despite affecting 1 in 4000 newborns. Sinovenous thrombosis is commonly associated with head and neck infections, and haemorrhagic stroke with arterio-venous malformations. Magnetic resonance imaging is the diagnostic investigation of choice. Treatment recommendations are extrapolated from adults due to a lack of prospective interventional studies.

N-terminal protein acetylation is common in eukaryotes and halophilic archaea, but very rare in bacteria. We demonstrate that some of the most abundant proteins present in the crenarchaeote Sulfolobus solfataricus, including subunits of the thermosome, proteosome and ribosome, are acetylated at the N-terminus. Modification was observed at the N-terminal residues serine, alanine, threonine and methionine-glutamate. A conserved archaeal protein, ssArd1, was cloned and expressed in Escherichia coli, and shown to acetylate the same N-terminal sequences in vitro. The specific activity of ssArd1 is sensitive to protein structure in addition to sequence context. The crenarchaeota and euryarchaeota apparently differ in respect of the frequency of acetylation of Met-Glu termini, which appears much more common in S. solfataricus. This sequence is acetylated by the related Nat3 acetylase in eukarya. ssArd1 thus has a relaxed sequence specificity compared with the eukaryotic N-acetyl transferases, and may represent an ancestral form of the enzyme. This represents another example where archaeal molecular biology resembles that in eukaryotes rather than bacteria.

Infantile spasms (IS) are a serious epileptic encephalopathy that occur most commonly between 2 and 14 months. The spasms occur in association with hypsarrhythmia or similar on EEG. There is often coincident psychomotor arrest or regression. Between March 2007 and May 2014, infants with IS and a compatible EEG were enrolled in a multicenter treatment trial. Participating countries were the UK, Germany, Switzerland, Australia and New Zealand. Infants were randomised to receive either hormonal therapy and vigabatrin or hormonal therapy alone. A second stage randomization allowed hormonal treatment to be allocated as either prednisolone or tetracosactide depot. Minimum doses were: vigabatrin 100 mg/kg/day, prednisolone 40 mg per day, or IM tetracosactide depot 0.5 mg on alternate days. Hormonal treatment was continued for 2 weeks and then weaned over 2 weeks. Vigabatrin was continued for 3 months and then weaned over a month. The early primary outcome measure was cessation of spasms on and between days 14 and 42. Analysis is by intention to treat. 377 children were enrolled and early clinical outcome data will be available on 376 (1 case withdrew). 186 were allocated hormonal therapy and vigabatrin and 191 were allocated hormonal therapy alone. We will report on the primary clinical outcome and serious adverse clinical events. Developmental outcome at 18 months of age will be reported in a subsequent paper. To date this is by far the largest treatment study of infantile spasms ever undertaken.

OBJECTIVE To report the efficacy and tolerability of the ketogenic diet (KD) in refractory paediatric epilepsy. METHODS Twenty-six consecutive children were treated with the classical KD from 1996 to 2001. The epilepsy syndromes included symptomatic generalized epilepsy (15), idiopathic generalized epilepsy (4), symptomatic partial epilepsy (1) and unclassified epilepsy (6). One child was lost to follow up. RESULTS Median age at initiation of the KD was 6.1 years. Median duration of the treatment was 9 months. Twelve children (48%) were treated for >12 months; one still remains on the KD. Four children (16%) became seizure-free. Five children (20%) had 50-99% reduction in seizures, seven (28%) had

Objective: Arterial ischaemic stroke (AIS) in childhood is a serious disorder about which little is published. The aim of this study is to determine the epidemiology and outcome of AIS in Australian children. Methods: Cases of childhood AIS occurring at the Royal Children's Hospital, Melbourne 1993−2001, were identified by medical record search using International Classification of Disease Codes. Information was collected on demographics, risk factors, arterial distribution, results of thrombophilic testing, management and outcome. Results: During the 8 years of review 95 patients presented with 98 cases of AIS calculating an incidence of 1.8 per 100 000 children per year. Children less than 12 months of age represented greater than one third of all cases. Identifiable risk factors were present in 64% of cases with congenital heart disease the major risk factor. Thrombophilic testing was incomplete with initial abnormalities present in 18% of cases tested. The estimated stroke-related mortality was 8.4%. Of the patients who survived and who had follow-up details available, 78% had a neurological deficit. Twenty-six patients (26%) received anticoagulation. There was no statistically significant association between treatment with anticoagulation and normal neurological outcome. Conclusion: AIS is over-represented in children under 12 months of age and results in death or residual neurological impairment in the majority of cases. Further prospective studies are needed to identify risk factors for poor outcome. The recently established Australian and New Zealand Stroke and Thrombophilia Registry should provide important information on clinical and laboratory based risk factors and create a basis for international clinical trials to improve the outcome of childhood AIS.

Objective: To determine the current best practice for treatment of infantile spasms in children. Methods: Database searches of MEDLINE from 1966 and EMBASE from 1980 and searches of reference lists of retrieved articles were performed. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, and presence or absence of epilepsy or an epileptiform EEG. One hundred fifty-nine articles were selected for detailed review. Recommendations were based on a four-tiered classification scheme. Results: Adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms, but there is insufficient evidence to recommend the optimum dosage and duration of treatment. There is insufficient evidence to determine whether oral corticosteroids are effective. Vigabatrin is possibly effective for the short-term treatment of infantile spasm and is possibly also effective for children with tuberous sclerosis. Concerns about retinal toxicity suggest that serial ophthalmologic screening is required in patients on vigabatrin; however, the data are insufficient to make recommendations regarding the frequency or type of screening. There is insufficient evidence to recommend any other treatment of infantile spasms. There is insufficient evidence to conclude that successful treatment of infantile spasms improves the long-term prognosis. Conclusions: ACTH is probably an effective agent in the short-term treatment of infantile spasms. Vigabatrin is possibly effective.

To assess the relationship between leg length and glucose tolerance in pregnancy.The leg length and leg-to-height percentage were prospectively determined on 161 glucose-tolerant women during pregnancy and 61 women with gestational diabetes mellitus (GDM).Women with GDM were a mean of 2.8 cm shorter than women who were glucose tolerant, due entirely to their leg lengths being a mean of 3.2 cm shorter. With respect to the 2-h result on the glucose tolerance test (GTT), there were negative correlations for height (r = -0.161, P = 0.017), leg length (r = -0.266, P0.0005), and the leg-to-height percentage (r = -0.294, P0.0005). The correlation between the leg-to-height percentage and the 2-h result on the GTT remained significant after adjustment for age (r = -0.252, P0.0005) and for age and BMI (r = -0.224, P = 0.001).Women with GDM are shorter than glucose-tolerant women and have a lower leg-to-height percentage. Consideration of short stature as a risk factor for GDM is not valid without taking into account the leg-to-height percentage.

Objective: Cerebral sinus venous thrombosis (cerebral SVT) is rare in children. Information on clinical characteristics, radiological findings and outcome is emerging. Methods: Cases of cerebral SVT diagnosed between 1995 and 2001 were identified by a computer-assisted search using International Classification of Disease codes. Medical records were reviewed to collect information on clinical presentation, investigations, treatment and outcome. Results: Sixteen cases of cerebral SVT were identified. All cases presented in association with head and neck pathology. The majority of cases presented with symptoms of raised intracranial pressure and focal neurological signs. Magnetic resonance imaging identified all cases of cerebral SVT whilst CT scanning failed to demonstrate the diagnosis in two cases. Management with anticoagulation was associated with radiological resolution of the thrombosis and normal neurological outcome. Long-term follow up demonstrated neurological deficits in greater than 40% of patients. Conclusion: Cerebral SVT in children is associated with significant residual neurological morbidity. Prospective studies to identify predictors of outcome and effective management interventions are required.

The objective of this study was to compare the efficacy of corticotropin (ACTH) versus vigabatrin in treating infantile spasms and to determine which medication has a more favorable long-term outcome in terms of cognitive function, evolution of epilepsy, and incidence of autism. Patients with infantile spasms were included in the study if they were 3 to 16 months old, had hypsarrhythmia, and had no previous treatment with vigabatrin or corticosteroids. Patient evaluation included electroencephalographic and psychometric measures before and after treatment. Patients were stratified based on etiology (idiopathic or symptomatic) and sex and then randomized between the ACTH and vigabatrin treatment groups. Each of the treatment groups received either ACTH or vigabatrin for 2 weeks. At the end of 2 weeks of treatment, patients were considered responders if spasms and hypsarrhythmia resolved. Nonresponders were crossed over and treated with the alternate drug. Nine patients were included in the study. Three patients received ACTH, one of whom was a responder. Six patients received vigabatrin, three of whom were responders. The five nonresponders received both therapies. All patients had some degree of developmental plateau or regression before the initiation of treatment. Four patients with idiopathic infantile spasms showed improved cognitive function following treatment. The remaining five patients remained significantly delayed. Five patients with symptomatic infantile spasms had epilepsy following treatment; three of them were in the autistic spectrum. The small number of infants in this pilot study is insufficient to determine which of the two drugs is more effective. However, the following trends were identified: vigabatrin may be more effective for patients with symptomatic infantile spasms; patients with idiopathic infantile spasms tend to have a better cognitive outcome; and patients with symptomatic infantile spasms tend to develop both epilepsy and autism. ( J Child Neurol 2003; 18: 165—170).

The object of this work was to subject established empirical medical treatment regimens for infantile spasms to evidence-based medicine analysis in order to determine the current best practice for the treatment of infantile spasms in children. Clinical studies of infantile spasms reported during the presteroid era were reviewed critically to define the natural history of the disorder. Treatment trials of infantile spasms conducted since 1958 were rigorously assessed using MEDLINE and hand searches of the English language literature. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, the presence or absence of epilepsy, and/or an epileptiform electroencephalogram. Evidence was defined as class I, II, or III, and practice parameter recommendations were made using the framework devised by the American Academy of Neurology. Class I and III evidence support a standard of practice recommendation for the use of vigabatrin in the treatment of infantile spasms in children with tuberous sclerosis. Class I and III evidence support a guidelines recommendation for the use of either ACTH or vigabatrin in infantile spasms in nontuberous sclerosis patients. There is no strong evidence that successful treatment of infantile spasms improves the long-term prognosis for cognitive outcome or decreases the incidence of later epilepsy. A practice option recommendation for the use of oral corticosteroids in the treatment of infantile spasms is supported by limited and inconclusive class I and III data. Based on the evidence, no recommendation can be made for the use of pyridoxine, benzodiazepines, or the newer antiepileptic drugs in the treatment of infantile spasms. ACTH and vigabatrin are the most effective agents in the treatment of infantile spasms, but concerns remain about the risk/benefit profiles of these drugs.

therapies have not been trialled in children and there is little information about long-term survival in treated children. Methods: A multicentre, web-based Australian and New Zealand registry for children with PAH was established to better understand the profile of children with IPAH, aiming to describe demographics, presenting features, clinical course and outcomes of children aged three months to 18years at timeofPAHdiagnosis, from1/1/2002. A group of historic controlswith IPAHdiagnosed between 1970 and 1990 were used for comparison. Results:Of the 24 patients enrolled with IPAH, 54% are female and 75% Caucasian. The mean (SD) age at diagnosis was 9.3 (4.90) years and 50% were WHO functional class III at presentation. Baseline cardiac catheterisation data for both groups is shown. During follow-up, 20 (83%) subjects were treated with an endothelin receptor antagonist (exclusively Bosentan monohydrate), 17 (71%) with a phosphodiesterase 5 inhibitor (sildenafil) and 10 (42%) with either epoprostenol or iloprost; 19 (79%) are anticoagulated with warfarin. Themean duration of follow-up was 3.45 years, with an annualisedmortality of 6%. Study endpoints of death/transplant occurred in 6/24, compared to 10/12 controls (p= .001). Severity of PAH from cardiac catheterisationwas similar between patients and controls. Conclusions: Multiple medical therapies have resulted in improved survival for children with IPAH diagn c d r

The congenital muscular dystrophies are a heterogeneous, recessively inherited group of disorders that have been subclassified on the basis of clinical central nervous system involvement. We report two children with "pure" congenital muscular dystrophy, one merosin negative and one merosin positive with extensive white matter and occipital cortical neuromigration abnormalities on magnetic resonance imaging (MRI). The first patient (merosin-negative congenital muscular dystrophy) presented with hypotonia and weakness in the neonatal period and subsequently was found to have a leukoencephalopathy and occipital cortical dysplasia on magnetic resonance imaging. The second patient presented with developmental delay without definite weakness. Initial investigations revealed a leukoencephalopathy and cortical dysplasia, but the patient subsequently was shown to have merosin-positive congenital muscular dystrophy. These patients illustrate that white-matter changes are not specific for merosin-negative congenital muscular dystrophy alone and that extensive cortical abnormality can be found in both groups of patients. In addition, our second patient illustrates a non-muscular mode of congenital muscular dystrophy presentation that should be considered in patients with a "nonprogressive leukodystrophy." (J ChiLd Neurol 1998;13:481-487).

Teaching the low-vision patient with an ostomy to manage a stoma independently provides a significant challenge for the WOC nurse. Although guide strips, lighted mirrors, and other handheld devices are available to patients with impaired eyesight, these products, when used alone, may be inadequate for the patient with an ostomy. In addition, there is a paucity of literature available to the WOC nurse describing specific interventions for the low-vision patient with an ostomy. Common visual problems encountered when managing people with an ostomy are reviewed. Specific interventions are discussed, including products designed to assist the low-vision patient with an ostomy with stoma care. A case study is provided that underscores the importance of consulting a low-vision specialist in the care of the patient with a new ostomy who has low vision as a result of albinism.

We emphasize again that the prevalence of maternal infections may vary in different populations, and others might arrive at different estimates about the percentage of infants colonized and, concerning those infants colonized, about the percentage with adverse outcomes. Additionally, many women are colonized or infected simultaneously with several of the organisms discussed in this review, and this may result in different projections of morbidity and mortality rates than those presented here. We realize also that new information is generated continually describing the relationship between various maternal colonizations and preterm birth, and that screening and treatment protocols for several diseases may reduce the prevalence of adverse outcomes reported here. Therefore, we emphasize that the prevalences of various adverse pregnancy outcomes, as presented in this article, are only approximations and may change as new information becomes available. Nevertheless, we believe it is reasonable to estimate the relative effect of various maternal sexually transmitted diseases on adverse pregnancy outcome as we have done in this article. By comparing the effect of direct transmission of sexually transmitted organisms on adverse outcomes with the effect on overall outcome through an increase in the rate of preterm births, we should be able to use this type of analysis to establish some basis for allocation of resources to future research as well as intervention programs aimed at reducing sexually transmitted disease-related adverse outcomes of pregnancy. Finally, the appreciation of the effect of bacterial vaginosis on outcomes of pregnancy associated with preterm birth gives bacterial vaginosis a greater public health importance than has been attributed to it in the past as the subject of sexually transmitted disease research and prevention.

Doppler ultrasound detection of abnormally high-pitched signals within the arterial waveform offers a new method for diagnosis, and potentially for prediction, of embolic complications in at-risk patients. The nature of Doppler "microembolic" signals is of particular interest in patients with prosthetic heart valves, where a high prevalence of these signals is observed. Monitoring the middle cerebral artery with 2-MHz transcranial Doppler ultrasound (TC-2000, Nicolet Biomedical; Warwick, UK), we looked for microemboli signals in 150 patients (95 women and 55 men), and found 1 or more signals during a 30-min recording in 89% of 70 patients with Bjork-Shiley valves (principally monostrut), 54% of 50 patients with Medtronic-Hall valves, and 50% of 30 patients with Carpentier-Edwards valves (p < 0.001, chi 2). In the patients with Bjork-Shiley valves, the mean number of signals per hour was 59 (range, 42-86; 95% confidence interval), which was significantly higher than the mean in patients with Medtronic-Hall and Carpentier-Edwards valves (1.5[range, 0.5-2.5] and 1 [range, 0-5.3], respectively; both p < 0.04, multiple comparisons. Bonferroni correction). In the patients undergoing serial pre- and postoperative studies, the causative role of the valve implant was emphasized. There was no correlation between the number of emboli signals and a prior history of neurologic deficit, cardiac rhythm, previous cardiac surgery, or the intensity of oral anticoagulation, in patients with prosthetic heart valves. In Bjork-Shiley patients, dual (mitral and aortic) valves were associated with more signals than were single valves. In Medtronic-Hall patients, the signal count was greater for valves in the aortic position than it was for valves in the mitral position. Comparative studies of Doppler emboli signals in other clinical settings suggest a difference in composition or size of the underlying maternal between prosthetic valve patients and patients with carotid stenosis. These studies also suggest that the signals are of gaseous origin in valve patients. The clinical significance of continuing microembolism remains to be determined.

Transfusion practice and blood ordering policy for major joint replacement was studied during 1989-93. Existing practice requiring automatic preoperative cross-match was prospectively audited. A blood ordering policy rationalising transfusion practice, using the group and screen technique, was then introduced. This resulted in a decrease in the total number of units cross-matched and increased the transfusion fraction significantly. The percentage of blood returned to the blood bank decreased by 46% for knees and 41% for hip arthroplasty. After the introduction of low molecular weight heparin (LMWH) as routine deep vein thrombosis (DVT) prophylaxis for all major total joint arthroplasties, the total transfusion requirement did not increase. In fact, the average number of units transfused per case after its introduction was marginally less at 1.3 units per hip, and 0.9 units per knee.

According to a 1991-1992 survey of program directors of obstetrics and gynecology residency programs in the United States, the overall percentage of programs providing any training in first-trimester (70%) or second-trimester abortion (66%) has changed very little since 1985. However, the proportion of programs providing routine training in first-trimester abortion decreased from 23% in 1985 to 12% in 1991-1992, and the proportion providing routine training in second-trimester abortion declined from 21% to 7%. The majority of the programs that dropped routine abortion training continued to offer optional training, but residents in programs with optional training were less likely to receive training. More than 80% of programs in private, non-Catholic hospitals and public hospitals provided some form of abortion training in 1991-1992, but only 6% of programs in Catholic hospitals and no military programs did so. In 45% of programs offering abortion training, residents performed one or fewer abortions per week.

Shallow traps that affect exciton dynamics in undoped and Ce doped yttrium aluminum garnet (CeYAG) single crystals were studied by low temperature thermo-luminescence spectroscopy. The nature of traps was identified and their energy levels in the band gap were measured. Three types of traps were found: two very shallow traps with activation energy of less than 0.2 eV associated with electronic defects, one trap with activation energy of 0.5 eV associated with oxygen vacancies, and one relatively deeper trap with activation energy of 0.7 eV associated with hydrogen impurities. The association of oxygen vacancies with shallow traps as evident from this work and with deep traps as reported by Varney et al. [J. Appl. Phys. 111, 063505 (2012)] suggest their presence in the lattice in more than one charge state.

Optical absorption and photoluminescence (PL) measurements were performed on single crystals of undoped Y3Al5O12 (YAG) and a number of rare-earth-doped YAG to study the effect of dopant type and concentration, growth atmosphere, post-growth annealing and UV irradiation on the optical properties of YAG crystals. The presence of hydrogen in the growth atmosphere was found to be essential for enhancing the incorporation of Ce ions in the Ce3+ state in Ce-doped YAG (Ce : YAG). Annealing in air was shown to have no effect on the PL emission of Ce : YAG crystals. An absorption peak around 256 nm was observed in the undoped YAG and Ce : YAG crystals after air anneal at 1200 °C. Optical absorption and annealing experiments support the association of the 256 nm peak with Fe impurities and oxygen ions. UV irradiation modifies the valency of impurities and generates electronic defects leading to an increase in the optical density of YAG crystals. Optimizing the growth and annealing conditions is critical in order to develop Ce : YAG single crystals as efficient scintillators.

Anecdotal reports have suggested that the use of disposable contact lenses is an effective treatment for giant papillary conjunctivitis. In a prospective randomized double-masked study, we compared the clinical performance of the Acuvue (Vistakon, Jacksonville, Florida) disposable contact lens to the traditional daily-wear lens of choice, CSI (Pilkington Barnes Hind, Sunnyvale, California), in 37 patients with previously diagnosed giant papillary conjunctivitis who were examined at one-month intervals for six months. A survey questionnaire for grading symptoms disclosed significant improvement in itching, discharge, and blurred vision in both groups early in the study. Evaluation of patient preference showed that the CSI lens was strongly preferred with regard to lens handling (76% vs 8%). In contrast, the Acuvue lens was strongly preferred with regard to lens comfort (62% vs 11%). For the variable of overall lens preference, there was no significant difference between the two lenses (CSI, 43% and Acuvue, 41%). Multiple regression analysis disclosed that both lens comfort (P.002) and lens handling (P = .05) contributed significantly to the choices made regarding the dependent variable of overall lens preference. Lens comfort (beta = .71) was observed to be approximately 50% more powerful than lens handling (beta = .48). There was no significant difference in the rate or cost of torn lenses between groups, suggesting that the Acuvue lens can withstand the physical manipulation associated with daily-wear use for up to one month. The results of this study suggest that the use of disposable contact lenses for the treatment of giant papillary conjunctivitis is at least as effective as the traditional daily-wear lens of choice.

Background: Benign acute myositis of childhood is a disorder of midchildhood, typically affecting boys. Symptoms include calf pain and difficulty walking after a viral illness. There is an epidemiologic association with influenza. Objectives: To describe the clinical and laboratory features of benign acute myositis. Results: Thirty-eight children (32 boys, 6 girls) were seen with 41 episodes of myositis between 1978 and 1997. Two were siblings and three had recurrent episodes. Mean age at onset of symptoms was 8.1 years. Children remained ambulant during 33 of 41 episodes. Two characteristic gaits were noted: toe-walking in 13, with a wide-based stiff-legged gait in another 7. Muscle tenderness was isolated to the gastrocnemius–soleus muscles in 82% of episodes. Recovery occurred within 1 week. Creatine kinase levels were elevated during all episodes. Viral studies were positive in 10 of 24 episodes, 5 because of influenza B. Conclusion: Benign acute myositis is a syndrome of midchildhood that can be differentiated from more serious causes of walking difficulty by the presence of calf tenderness, normal power, intact tendon reflexes, and elevated creatine kinase. The gait patterns noted may minimize power generation of the calf muscles by splinting the ankles. Onset in childhood may reflect an age-related response to viral infection, and occurrence primarily in boys may reflect a genetic predisposition or an as-yet unknown metabolic defect.

A strong petroleum refinery odor in mackerel taken in late November 1971, near Halifax and on the outer edge of the Emerald Bank, has been identified as harmless dimethyl sulfide. It has been shown to originate from stomach contents and is specifically associated with the pteropod Spiratella retroversa. The dimethyl sulfide was not accompanied by any very large deposition in the fish of the known precursor of algal origin, dimethyl-β-propiothetin. Petroleum product pollution was not involved.

Mean counts of vertebrae, of anal and soft dorsal fin rays, and of peduncular finlets were identical in samples of Atlantic mackerel, Scomber scombrus, from the northern and southern breeding populations in the northwest Atlantic Ocean. This information, together with growth rates and biochemical evidence from the literature, suggests that although the populations occupy separate spawning regions there is sufficient exchange of individuals at other seasons to maintain considerable genetic continuity.