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Charles Glasier - Top 30 Publications

Sturge-Weber Syndrome.

Injured Children Receive Twice the Radiation Dose at Nonpediatric Trauma Centers Compared With Pediatric Trauma Centers.

Use of cranial CT scans in children has been increasing, in part due to increased awareness of sports-related concussions. CT is the largest contributor to medical radiation exposure, a risk factor for cancer. Long-term cancer risks of CT scans can be two to three times higher for children than for adults because children are more radiosensitive and have a longer lifetime in which to accumulate exposure from multiple scans.

The Clinical Impact of a Web-Based Image Repository on Radiation Exposure in Injured Children.

The long-term cancer risks for children exposed to radiologic images can be two to three times higher than for adults because children are more sensitive to radiation and have a longer lifetime in which to accumulate exposure from CT scans. Injured children often undergo repeat CT imaging if they are transferred from non-pediatric hospitals to a Level I pediatric trauma center (PTC). This study determined the impact of a statewide web-based image repository (WBIR) on repeat imaging among transferred injured children.

Compression of the posterior fossa venous sinuses by epidural hemorrhage simulating venous sinus thrombosis: CT and MR findings.

Posterior fossa dural venous sinus thrombus is a well-described complication of head trauma, especially when fracture crosses the dural sinus grooves or in association with epidural hemorrhage. We have found that post-traumatic posterior fossa epidural hematoma compressing a dural venous sinus can mimic dural venous thrombus.

Strategies for Computed Tomography Radiation Dose Reduction in Pediatric Neuroimaging.

Radiation exposure from diagnostic imaging is a significant concern, particularly in the care of pediatric patients. Computed tomography (CT) scanning is a significant source of radiation.

Amplitude-integrated EEG in newborns with critical congenital heart disease predicts preoperative brain magnetic resonance imaging findings.

The study aims are to evaluate cerebral background patterns using amplitude-integrated electroencephalography in newborns with critical congenital heart disease, determine if amplitude-integrated electroencephalography is predictive of preoperative brain injury, and assess the incidence of preoperative seizures. We hypothesize that amplitude-integrated electroencephalography will show abnormal background patterns in the early preoperative period in infants with congenital heart disease that have preoperative brain injury on magnetic resonance imaging.

White matter injury in newborns with congenital heart disease: a diffusion tensor imaging study.

Brain injury is observed on cranial magnetic resonance imaging preoperatively in up to 50% of newborns with congenital heart disease. Newer imaging techniques such as diffusion tensor imaging provide sensitive measures of the white matter integrity. The objective of this study was to evaluate the diffusion tensor imaging analysis technique of tract-based spatial statistics in newborns with congenital heart disease.

Brain ketones detected by proton magnetic resonance spectroscopy in an infant with Ohtahara syndrome treated with ketogenic diet.

Atypical resonances on proton magnetic resonance spectroscopy (MRS) examinations are occasionally found in children undergoing a metabolic evaluation for neurological conditions. While a radiologist's first instinct is to suspect a pathological metabolite, usually the origin of the resonance arises from an exogenous source. We report the appearance of distinct resonances associated with a ketogenic diet in a male infant presenting with Ohtahara syndrome. These resonances can be confused in interpretation with lactate and glutamate. To confirm assignments, the basis set for quantification was supplemented with simulations of β-hydroxybutyrate, acetone and acetoacetate in LCModel spectroscopy processing software. We were able to quantitate the levels of end products of a ketogenic diet and illustrate how to distinguish these resonances.

Diffusion tensor imaging in extremely low birth weight infants managed with hypercapnic vs. normocapnic ventilation.

Permissive hypercapnia is a ventilatory strategy used to prevent lung injury in ventilated extremely low birth weight (ELBW, birth weight ≤1,000 g) infants. However, there is retrospective evidence showing that high CO2 is associated with brain injury.

Author reply: To PMID 23972277.

Reliability of magnetic resonance imaging for the detection of hypopituitarism in children with optic nerve hypoplasia.

It is essential to identify hypopituitarism in children with optic nerve hypoplasia (ONH) because they are at risk for developmental delay, seizures, or death. The purpose of this study is to determine the reliability of neurohypophyseal abnormalities on magnetic resonance imaging (MRI) for the detection of hypopituitarism in children with ONH.

Multi-tiered analysis of brain injury in neonates with congenital heart disease.

Early brain injury occurs in newborns with congenital heart disease (CHD) placing them at risk for impaired neurodevelopmental outcomes. Predictors for preoperative brain injury have not been well described in CHD newborns. This study aimed to analyze, retrospectively, brain magnetic resonance imaging (MRI) in a heterogeneous group of newborns who had CHD surgery during the first month of life using a detailed qualitative CHD MRI Injury Score, quantitative imaging assessments (regional apparent diffusion coefficient [ADC] values and brain volumes), and clinical characteristics. Seventy-three newborns who had CHD surgery at 8 ± 5 (mean ± SD) days of life and preoperative brain MRI were included; 38 also had postoperative MRI. Thirty-four (34 of 73, 47 %) had at least one type of preoperative brain injury, and 28 of 38 (74 %) had postoperative brain injury. The 5-min APGAR score was negatively associated with preoperative injury, but there was no difference between CHD types. Infants with intraparenchymal hemorrhage, deep gray matter injury, and/or watershed infarcts had the highest CHD MRI Injury Scores. ADC values and brain volumes were not different in infants with different CHD types or in those with and without brain injury. In a mixed group of CHD newborns, brain injury was found preoperatively on MRI in almost 50 %, and there were no significant baseline characteristic differences to predict this early brain injury except 5-min APGAR score. We conclude that all infants, regardless of CHD type, who require early surgery should be evaluated with MRI because they are all at high risk for brain injury.

Variable manifestations of familial hemiplegic migraine associated with reversible cerebral edema in children.

Three children with familial hemiplegic migraine presented with right-sided weakness, speech difficulty, altered mental status, and gait abnormalities. These persistent aura signs were accompanied by left-sided slowing and cerebral dysfunction, documented by electroencephalograms. Cranial magnetic resonance imaging revealed cortical edema restricted to the left cerebral hemisphere. Follow-up electroencephalogram and imaging studies produced normal results 1-4 months afterward. However, cognitive changes persisted. Genetic testing demonstrated variable results: one child manifested a CACNA1A mutation compatible with familial hemiplegic migraine type 1, whereas another demonstrated an ATP1A2 sequence alteration. No known mutations were evident in the third child, with minor head trauma thought to precipitate the familial hemiplegic migraine. These findings demonstrate the variable clinical and genetic heterogeneity of childhood familial hemiplegic migraine.

Decreased activation and increased lateralization in brain functioning for selective attention and response inhibition in adolescents with spina bifida.

We used functional magnetic resonance imaging to evaluate functional activity in the brain of adolescents with spina bifida when performing selective attention and response inhibition tasks. We then compared the results to that of age-matched controls. Our results showed that adolescents with spina bifida had decreased frontal and superior parietal activation and more apparently low involvement of left brain hemisphere during these tasks. Our results indicated activation deficits and possibly abnormal functional organization in adolescents with spina bifida and associated pathologies such as hydrocephalus.

Ketamine as a neuroprotective and anti-inflammatory agent in children undergoing surgery on cardiopulmonary bypass: a pilot randomized, double-blind, placebo-controlled trial.

Infants are potentially more susceptible to cell death mediated via glutamate excitotoxicity attributed to cardiopulmonary bypass. We hypothesized that ketamine, via N-methyl D-aspartate receptor blockade and anti-inflammatory effects, would reduce central nervous system injury during cardiopulmonary bypass.

Cavernous sinus syndrome from an internal carotid artery aneurysm in an infant with tuberous sclerosis.

Aneurysms are associated with tuberous sclerosis complex. We describe the first case of cavernous sinus syndrome from an intracavernous internal carotid artery aneurysm in a 9-month-old boy with tuberous sclerosis. The presence of an intracranial aneurysm should be considered in the differential diagnosis of children with tuberous sclerosis who develop cranial nerve deficits.

Diffusion tensor imaging evaluation of white matter in adolescents with myelomeningocele and Chiari II malformation.

Macrostructural abnormalities in cerebral white matter in patients with myelomeningocele are well known, but microstructural abnormalities are not as well studied.

Nerve root enhancement on spinal MRI in pediatric Guillain-Barré syndrome.

Guillain-Barré syndrome diagnosis is based on clinical presentation and supportive diagnostic testing. In its early stage, no single, reliable diagnostic test is available. However, a finding of nerve root enhancement on spinal magnetic resonance imaging may be useful. We evaluated the frequency of nerve root enhancement on spinal magnetic resonance imaging in children with Guillain-Barré syndrome. At a single tertiary pediatric center, we conducted a retrospective chart review of children with Guillain-Barré syndrome who had complete spinal or lumbosacral spinal magnetic resonance imaging with gadolinium administration from January 2002-January 2009. Twenty-four consecutive patients were identified. Spinal nerve root enhancement with gadolinium was present in 92% (22/24) of children with Guillain-Barré syndrome on initial spinal magnetic resonance imaging (95% confidence interval, 0.745-0.978). This finding increased to 100% of patients, after two patients underwent repeat spinal magnetic resonance imaging that did reveal nerve root enhancement. Patterns of enhancement were variable, but involved the thoracolumbar nerve roots in all patients. Enhancement of nerve roots with gadolinium on initial spinal magnetic resonance imaging was frequently present in these children with Guillain-Barré syndrome. Spinal magnetic resonance imaging is a sensitive diagnostic test and should be considered an additional diagnostic tool in select cases.

Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor: report of the first case.

Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.

Sporadic bilateral optic neuropathy in children: the role of mitochondrial abnormalities.

To evaluate a group of patients with isolated, early-onset, bilateral optic neuropathy for genetic and biochemical evidence of mitochondrial diseases.

Pediatric neurodegenerative white matter processes: leukodystrophies and beyond.

Pediatric neurodegenerative white matter processes are complex, numerous and result from a vast array of causes ranging from white matter injury or inflammation to congenital metabolic disorders. When faced with a neurodegenerative white matter process on neuroimaging, the first step for the radiologist is to determine whether the findings represent a congenital metabolic leukodystrophy or one of various other white matter processes. In this review we first describe a general approach to neurodegenerative white matter disorders. We will briefly describe a few white matter diseases that mimic metabolic leukodystrophies. In the second half of the review we discuss an approach to distinguishing and classifying white matter leukodystrophies.

Neuro-ophthalmologic findings in patients with rhombencephalosynapsis.

Rhombencephalosynapsis is a rare, sporadic, posterior fossa malformation characterized by agenesis of the cerebellar vermis, dorsal fusion of the cerebellar hemispheres, and fusion of the dentate nuclei and superior cerebellar peduncles. Ophthalmologic findings in patients with rhombencephalosynapsis have not been described in detail. Optic nerve and chiasmal hypoplasia were reported in two patients. Strabismus has been noted in several patients; however, specific details were not provided. We describe the association of A-pattern strabismus and superior oblique overaction with rhombencephalosynapsis.

Diffusion-weighted imaging of the brain in infants and children.

During the last decade, diffusion-weighted imaging (DWI) has become an important tool in the evaluation of a variety of disorders of the central nervous system in children. DWI relies on variability in the diffusivity of water molecules in the presence of a supplemental diffusion-sensitizing gradient to produce image contrast. Pathologic states alter the diffusion characteristics of brain water in a reproducible fashion. In this review, the DWI appearances of a number of common abnormalities of the brain in infants and children are presented.

Somatostatin receptor scintigraphy in surveillance of pediatric brain malignancies.

Somatostatin receptor scintigraphy (SRS) has been successfully used in imaging PBTs and, as a functional imaging modality, may be better able to differentiate tumor from scar/necrosis. This retrospective study evaluates the role of SRS in post-treatment surveillance of PBTs.

Profound infantile neuroretinal dysfunction in a heterozygote for the CLN3 genetic defect.

The neuronal ceroid-lipofuscinoses are a group of diseases that are characterized by progressive neuroretinal symptomatology, progressive accumulation of autofluorescing waxy lipopigments (ceroid-lipofuscin) within the brain and other tissues, and cerebral atrophy. Juvenile neuronal ceroid-lipofuscinosis, or Batten disease, is a form of neuronal ceroid-lipofuscinosis that is characterized by onset of neuroretinal symptoms between 4 and 10 years. Juvenile neuronal ceroid-lipofuscinosis is the most common type of neuronal ceroid-lipofuscinosis in the United States and Europe and is inherited as an autosomal recessive genetic disorder. Research in the last decade has led to the identification of the responsible gene for juvenile neuronal ceroid-lipofuscinosis, which is designated as CLN3. CLN3 is located on chromosome 16p11.2-12.1. The major mutation is a 1.02 kb deletion, which removes exons 7 and 8. Both homozygotic and heterozygotic deletions at the CLN3 gene site have been associated with the clinical syndromes of juvenile neuronal ceroid-lipofuscinosis. We report a possible atypical case of neuronal ceroid-lipofuscinosis, an infant, who presented at 5 months of age with a lack of developmental milestones, poor vision, severe retinopathy, intractable seizures, and progressive cerebral atrophy. Extensive laboratory investigations, including thorough metabolic evaluations, were unremarkable except for neuroimaging studies, electroencephalography, and electroretinography, all of which showed abnormalities confirming both cerebral and retinal degeneration. Although skin and conjunctival biopsies did not show classic fingerprint cytosomes by electron microscopic study, which characterize juvenile neuronal ceroid-lipofuscinosis, a diagnosis of an atypical form of juvenile neuronal ceroid-lipofuscinosis was suspected on the basis of the clinical picture. The retinal abnormalities, surprisingly, were those believed to be diagnostic of juvenile-onset neuronal ceroid-lipofuscinosis, or Batten disease. Subsequently, a heterozygous mutation for the common 1.02 kb deletion characteristic of juvenile neuronal ceroid-lipofuscinosis was established.

Chiasmal enlargement and optic nerve enhancement on magnetic resonance imaging in leber hereditary optic neuropathy.

Perinatal cortical and subcortical visual loss: mechanisms of injury and associated ophthalmologic signs.

To determine whether term and preterm injuries to the retrogeniculate visual system are associated with recognizable patterns of ophthalmologic abnormalities and whether these patterns can be attributed to cortical (gray matter) or subcortical (white matter) injury.