A Case of Tuberculosis-Associated Acute Disseminated Encephalomyelitis in a Seven-Month-Old Infant.

A seven-month-old previously healthy female infant presented with acute onset encephalopathy and left focal weakness in the setting of three months of non-productive cough. She was diagnosed with pulmonary tuberculosis (TB), and neuroimaging showed multifocal non-enhancing T2 hyperintensities in the brain and longitudinal T2 hyperintensity in the spinal cord consistent with acute disseminated encephalomyelitis (ADEM). However, her cerebrospinal fluid (CSF) did not show evidence of TB infection. She was treated with high-dose steroids for five days with a steroid taper along with antitubercular medications with a remarkable recovery in gross motor function. This case suggests a previously unreported association between TB and an immune-mediated demyelinating syndrome in children that is clinically distinct from other more common forms of TB-associated central nervous system (CNS) complications.

Abnormal cochleovestibular anatomy and imaging: Lack of consistency across quality of images, sequences obtained, and official reports.

OBJECTIVES: There are significant variations across centers on how to acquire and interpret imaging of children with congenital sensorineural hearing loss and cochleovestibular abnormalities. This study assesses the quality of imaging, sequences included, and accuracy of official radiology reports, to determine if these children are being assessed appropriately. METHODS: This study is retrospective review of CTs and MRIs from 40 pediatric patients diagnosed with profound sensorineural hearing loss and cochleovestibular structure/nerve abnormalities presenting to a tertiary referral academic center. Images were reviewed by two experienced neuroradiologists and a neurotologist. Findings were compared to official reports, when available. RESULTS: Twelve (30%) patients had an MRI only, while 28 (70%) had both an MRI and a CT. There were 3 (10.7%) CTs and 7 (17.5%) MRIs noted to be of poor quality. Children received an average of 6.8 (±2.7) CT acquisitions and 10.9 (±5.7) MRI acquisitions. There was non-concordance between the official report and expert review for 27 (71.1%) ears on CT and 27 (56.3%) ears on MRI. CONCLUSIONS: These data demonstrate high variability in protocols and quality of medical imaging of children with sensorineural hearing loss. Interpretation of images is highly discordant between official reports and tertiary review. Given these results, we recommend that these children be imaged and evaluated at centers with neuroradiologists who are experienced in interpreting congenital abnormalities of the cochleovestibular system.

Evaluation of Fetal First and Second Cervical Vertebrae: Normal or Abnormal?

OBJECTIVES: To use 3-dimensional sonographic volumes to evaluate the variable appearance of the normal fetal cervical spine and craniocervical junction, which if unrecognized may lead to misdiagnosis of malalignment at the first and second cervical vertebrae (C1 and C2). METHODS: Three-dimensional sonographic volumes of the fetal cervical spine were obtained from 24 fetuses at gestational ages between 12 weeks 6 days and 35 weeks 1 day. The volumes were reviewed on 4-dimensional software, and the vertebral level was determined by labeling the first rib-bearing vertebra as the first thoracic vertebra. The ossification centers of the cervical spine and occipital condyles were then labeled accordingly and evaluated for alignment and structure by rotating the volumes in oblique planes. The appearance on multiplanar images was assessed for possible perceived anomalies, including malalignment, particularly at the C1 and C2 levels. Evidence of head rotation was correlated with the presence of possible malalignment at C1-C2. Head rotation was identified in the axial plane by measuring the angle of the anteroposterior axis of C1 to the anteroposterior axis of C2. RESULTS: Of the 24 fetuses, 16 had adequate quality to assess the entire cervical spine and craniocervical junction. All 16 cases showed an osseous component of C1 that did not align directly with C2 on some of the multiplanar images when the volumes were rotated, which could lead to suspected diagnosis of spinal malalignment or a segmental abnormality, as occurred in 2 clinical cases in our practice. All 16 cases showed at least some degree of head rotation, ranging from 2° to 36°, which may possibly explain the apparent malalignment. The lateral offset from C1 to C2 ranged from 0.0 to 3.3 mm. CONCLUSIONS: The normal C1 and C2 ossification centers may appear to be malaligned due to normal offsetting (lateral displacement) of C1 on C2. An understanding of the normal development of the cervical spine is important in assessing spinal anatomy.

Appropriateness of radiology procedures performed in children with gastrointestinal symptoms and conditions.

BACKGROUND & AIMS: Exposure to ionizing radiation from diagnostic imaging procedures (DIPs) has been associated with an increased risk of cancer in children. In particular, gastrointestinal imaging has been identified as a significant factor that contributes to exposure of children to radiation during diagnostic procedures. We performed a longitudinal assessment of gastrointestinal-associated DIPs to identify practices that might be targeted to reduce exposure of pediatric patients to radiation. METHODS: DIP insurance claims from 2001 through 2009 were obtained from an Independent Physicians Association in a large US metropolitan area. We retrieved and analyzed Current Procedural Terminology codes, associated International Classification of Diseases, 9th Revision, codes specific for gastrointestinal symptoms and conditions, and patient demographics associated with DIPs from insurance claims data. RESULTS: Overall, 11,473 DIPs were performed on 6550 children with gastrointestinal symptoms; 1 in 30 patients received a DIP for a gastrointestinal complaint. Over the study period, the proportion of higher-radiation DIPs (computed tomography, fluoroscopy, and angiography) increased. Higher-radiation DIPs for gastrointestinal symptoms were performed more frequently in older children and in boys in the emergency department and in inpatient settings for diagnoses of abdominal pain, appendicitis, and noninfectious gastroenteritis. CONCLUSIONS: Higher-radiation diagnostic imaging accounts for an increasing proportion of imaging procedures among children with gastrointestinal symptoms, even though these often are not recommended for evaluation of gastrointestinal disorders. Clinicians should be aware of these findings when ordering DIPs for gastrointestinal complaints, and clinical practice guidelines should be created to reduce diagnostic imaging-related radiation exposure in children.

Glioblastoma multiforme arising from dysembryoplastic neuroepithelial tumor in a child in the absence of therapy.

Dysembryoplastic neuroepithelial tumors (DNETs) are considered as low-grade tumors commonly associated with intractable seizures. We report a case of an unusual hemispheric DNET in a young child presenting with new-onset focal seizures. The tumor was notable for its atypical neuroimaging features and very rapid malignant transformation into a glioblastoma multiforme in the absence of radiation or chemotherapy, 1-year postdiagnosis. Our case highlights the malignant potential of atypical DNETs in the absence of therapy.

Behavioral effects of congenital ventromedial prefrontal cortex malformation.

BACKGROUND: A detailed behavioral profile associated with focal congenital malformation of the ventromedial prefrontal cortex (vmPFC) has not been reported previously. Here we describe a 14 year-old boy, B.W., with neurological and psychiatric sequelae stemming from focal cortical malformation of the left vmPFC. CASE PRESENTATION: B.W.'s behavior has been characterized through extensive review Patience of clinical and personal records along with behavioral and neuropsychological testing. A central feature of the behavioral profile is severe antisocial behavior. He is aggressive, manipulative, and callous; features consistent with psychopathy. Other problems include: egocentricity, impulsivity, hyperactivity, lack of empathy, lack of respect for authority, impaired moral judgment, an inability to plan ahead, and poor frustration tolerance. CONCLUSIONS: The vmPFC has a profound contribution to the development of human prosocial behavior. B.W. demonstrates how a congenital lesion to this cortical region severely disrupts this process.

Pediatric magnetoencephalography and magnetic source imaging.

Magnetoencephalography (MEG) and magnetic source imaging (MSI) together represent a uniquely powerful functional imaging modality because of their capabilities of directly observing the electrophysiologic activity of neurons with exquisite temporal detail and accurately localizing corresponding neuromagnetic field sources onto high-resolution MR images. These features have and should continue to advance our understanding of the complex spatiotemporal basis of normal and abnormal brain function and development in children. By more clearly delineating and characterizing epileptogenic foci and their relation to eloquent cortex, MSI enables earlier and more effective neurosurgery to be performed, thus resulting in improved seizure outcomes. Although MEG and MSI cannot replace scalp electroencephalography, neuropsychologic testing, and the need for meticulous intraoperative cortical mapping in patients undergoing excision of epileptogenic lesions, their increasing availability should ultimately persuade many clinicians of their key, if not essential, role in the evaluation and treatment of children with epilepsy.

Recurrent intractable seizures in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor.

The purpose of this study was to identify the pathologic features that predict postoperative outcome in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumors. We reviewed the records of children with dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery and who had at least 1 year of surgical follow-up. We divided the dysembryoplastic neuroepithelial tumors into three pathology classes (simple, complex, and nonspecific), categorized adjunctive cortical dysplasia into four types, and compared histopathology with seizure outcomes. We identified 26 children with dysembryoplastic neuroepithelial tumors. Dysembryoplastic neuroepithelial tumors were complex in 19 patients (73%), simple in 6 (23%), and nonspecific in 1 (4%). Cortical dysplasia was adjacent to dysembryoplastic neuroepithelial tumors in 18 patients. Six patients had type IA cortical dysplasia, 5 had type IB, 3 had type IIA, and 1 had type IIB. The 3 remaining patients had repeated surgeries; of these, 2 patients had cortical dysplasias of type IA/IB and 1 was type IIA/IIB. Eight (39%) of 18 patients with dysembryoplastic neuroepithelial tumors and cortical dysplasia required further surgery for recurrent intractable seizures (P < .05), whereas none of 8 patients without cortical dysplasia required additional surgery. Of 13 patients with type I cortical dysplasia, only 4 had a poor seizure outcome, whereas all 5 patients with type II had a poor seizure outcome postoperatively (P < .05). Children with dysembryoplastic neuroepithelial tumor and cortical dysplasia often had recurrent intractable seizures postoperatively and required further epilepsy surgery. Cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor can play a role in the epileptogenicity of dysembryoplastic neuroepithelial tumor. Complete resection of a dysembryoplastic neuroepithelial tumor and its adjacent cortical dysplasia should be considered.

Three-dimensionally reconstructed magnetic source imaging and neuronavigation in pediatric epilepsy: technical note.

OBJECTIVE: To determine the role of reconstructing three-dimensional magnetic source imaging (MSI) data on cortical resections for children undergoing epilepsy surgery using neuronavigation. METHODS: Magnetoencephalographic recordings were analyzed in 16 children under 18 years of age with intractable epilepsy. The data were transferred to the neuronavigation workstation for intraoperative localization of MSI spike sources in selected patients. With the aid of neuronavigation, the MSI spike sources were resected. Intraoperative electrocorticography was then used to survey the surrounding field for residual epileptiform activity. RESULTS: MSI spike sources were obtained in 13 of 16 patients. MSI spike sources localized the cortical and subcortical discharges before intraoperative electrocorticography in nine patients and before extraoperative subdural grid electroencephalographic monitoring in four patients. The localization of MSI spikes sources was characterized by clustered spike sources in 10 patients. By use of neuronavigation, the clustered spike sources were correlated to the interictal zone indicated by intraoperative electrocorticography in six patients and to the ictal onset zone shown on extraoperative subdural grid electroencephalography in three patients. Cortical excision of the spike cluster focus was then performed in these six patients. The technique used here to resect MSI spike source clusters that correlate with the ictal onset zone by invasive subdural grid monitoring is illustrated in one patient who underwent cortical resection for epilepsy surgery. CONCLUSION: Three-dimensional reconstruction of MSI data linked to neuronavigation is a promising technique to facilitate resections around eloquent cortex in children with epilepsy.

Slow-flow spinal epidural AVF with venous ectasias: two pediatric case reports.

We report the clinical and imaging findings in the cases of two children who initially presented with back pain related to epidural AVF in the cervicothoracic spine. Both lesions were of particular interest because of their exclusive epidural and paraspinal venous drainage and the presence of the prominent venous pouches in the epidural space. Angiography revealed that one was multifocal and of relatively slow flow. We think that these unusual features have important implications for treatment.

Safety and feasibility of a CT protocol for acute stroke: combined CT, CT angiography, and CT perfusion imaging in 53 consecutive patients.

By combining non-contrast-enhanced CT imaging, CT perfusion imaging, and cranial-to-chest CT angiography (CTA), the entire cerebrovascular axis can be imaged during acute stroke. To our knowledge, the safety and feasibility of this technique have not been previously reported. In a consecutive series of 53 patients with suspected acute stroke, renal failure was not observed. Median imaging time was 27 minutes (range, 9-67 minutes). Image quality was degraded by motion in 1.3% of vessels studied. Dynamic CT perfusion data were successfully obtained in 52 patients (98% of patients). High-speed, multisection, helical CT scanners allow rapid, safe imaging of the entire neurovascular axis in patients with acute stroke by use of combined CT imaging, CT perfusion imaging, and CTA.

Predictors of 30-month outcome after perinatal depression: role of proton MRS and socioeconomic factors.

The objective was to determine in infants with perinatal depression whether the relative concentrations of N-acetylaspartate and lactate in the neonatal period are associated with (1) neurodevelopmental outcome at 30 mo of age or (2) deterioration in outcome from age 12 to 30 mo; and to determine whether socioeconomic factors are associated with deterioration in outcome. Thirty-seven term neonates were prospectively studied with single-voxel proton magnetic resonance spectroscopy of the basal nuclei and intervascular boundary zones. Thirty-month outcomes were classified as normal [if Mental Development Index of the Bayley Scales of Infant Development (MDI) >85 and neuromotor scores (NMS) <3; n = 15], abnormal [if MDI or=3 at 12 and 30 mo; n = 11], or deteriorated [if normal at 12 mo and abnormal at 30 mo (MDI or=3); n = 11]. Thirty percent (11/37) of our cohort deteriorated between 12 and 30 mo. N-acetylaspartate/choline decreased across the groups ordered as normal, deteriorated, and abnormal [in basal nuclei (p