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BACKGROUND: Resting-state functional MRI holds substantial potential for clinical application, but limitations exist in current understanding of how tumors exert local effects on resting-state functional MRI readings. PURPOSE: To investigate the association between tumors, tumor characteristics, and changes in resting-state connectivity, to explore neurovascular uncoupling as a mechanism underlying these changes, and to evaluate seeding methodologies as a clinical tool. MATERIALS AND METHODS: Institutional review board approval was obtained for this HIPAA-compliant observational retrospective study of patients with glioma who underwent MRI and resting-state functional MRI between January 2016 and July 2017. Interhemispheric symmetry of connectivity was assessed in the hand motor region, incorporating tumor position, perfusion, grade, and connectivity generated from seed-based correlation. Statistical analysis was performed by using one-tailed t tests, Wilcoxon rank sum tests, one-way analysis of variance, Pearson correlation, and Spearman rank correlation, with significance at P < .05. RESULTS: Data in a total of 45 patients with glioma (mean age, 51.3 years ± 14.3 [standard deviation]) were compared with those in 10 healthy control subjects (mean age, 50.3 years ± 17.2). Patients showed loss of symmetry in measures of hand motor resting-state connectivity compared with control subjects (P < .05). Tumor distance from the ipsilateral hand motor (IHM) region correlated with the degree (R = 0.38, P = .01) and strength (R = 0.33, P = .03) of resting-state connectivity. In patients with World Health Organization grade IV glioblastomas 40 mm or less from the IHM region, loss of symmetry in strength of resting-state connectivity was correlated with tumor perfusion (R = 0.74, P < .01). In patients with gliomas 40 mm or less from the IHM region, seeding the nontumor hemisphere yielded less asymmetric hand motor resting-state connectivity than seeding the tumor hemisphere (connectivity seeded:contralateral = 1.34 nontumor vs 1.38 tumor hemisphere seeded; P = .03, false discovery rate threshold = 0.01). CONCLUSION: Hand motor resting-state connectivity was less symmetrical in a tumor distance–dependent manner in patients with glioma. Differences in resting-state connectivity may be false-negative results driven by a neurovascular uncoupling mechanism. Seeding from the nontumor hemisphere may attenuate asymmetry in patients with tumors near ipsilateral hand motor cortices. © RSNA, 2020 Online supplemental material is available for this article.

Resting state functional connectivity magnetic resonance imaging (rsfcMRI) has become a key component of investigations of neurocognitive and psychiatric behaviors. Over the past two decades, several methods and paradigms have been adopted to utilize and interpret data from resting-state fluctuations in the brain. These findings have increased our understanding of changes in many disease states. As the amount of resting state data available for research increases with big datasets and data-sharing projects, it is important to review the established traditional analysis methods and recognize areas where research methodology can be adapted to better accommodate the scale and complexity of rsfcMRI analysis. In this paper, we review established methods of analysis as well as areas that have been receiving increasing attention such as dynamic rsfcMRI, independent vector analysis, multiband rsfcMRI and network of networks.

CD19-specific chimeric antigen receptor (CAR) T-cell therapy is highly effective against relapsed or refractory acute lymphoblastic leukemia (ALL), but is hindered by neurotoxicity. In 53 adult patients with ALL, we found a significant association of severe neurotoxicity with high pretreatment disease burden, higher peak CAR T-cell expansion, and early and higher elevations of proinflammatory cytokines in blood. Patients with severe neurotoxicity had evidence of blood–cerebrospinal fluid (CSF) barrier disruption correlating with neurotoxicity grade without association with CSF white blood cell count or CAR T-cell quantity in CSF. Proinflammatory cytokines were enriched in CSF during severe neurotoxicity with disproportionately high levels of IL6, IL8, MCP1, and IP10, suggesting central nervous system–specific production. Seizures, seizure-like activity, myoclonus, and neuroimaging characteristics suggested excitatory neurotoxicity, and we found elevated levels of endogenous excitatory agonists in CSF during neurotoxicity.Significance: We detail the neurologic symptoms and blood, CSF, and neuroimaging correlates of neurotoxicity associated with CD19 CAR T cells and identify neurotoxicity risk factors. Our findings implicate cellular components other than T cells and suggest novel links between systemic inflammation and characteristic neurotoxicity symptoms. Cancer Discov; 8(8); 958–71. ©2018 AACR.This article is highlighted in the In This Issue feature, p. 899

Objective FSE sequences play key roles in neck MRI despite the susceptibility issues in neck region. Iterative decomposition of asymmetric echoes (IDEAL, GE) is a promising method that separates fat and water images resulting in high SNR and improved fat suppression. We tested how neck tissue contrasts, image artifacts and fat separation as opposed to fat suppression in terms of image quality compare between routine and IDEAL FSE. Methods IDEAL based and routine T1 and T2-weighted FSE sequences were applied for neck MRI at 1.5T and 3T. Overall image quality including fat suppression, tissue contrast, image artifacts and lesion conspicuity were subjectively assessed for 20 patients clinically indicated for neck MRI. Quantitative tissue contrast estimates from parotid area were compared between IDEAL and routine FSE for 7 patients. Four patients with oncocytoma were also reviewed to assess benefits of separately reconstructed fat specific image sets. Results Subjective tissue contrast and overall image quality including image sharpness, fat suppression and image artifacts were superior for IDEAL sequences. For oncocytoma fat specific IDEAL images provided additional information. Objective CNR estimates from a central slice were equivalent for IDEAL and routine FSE at both field strengths. Conclusions We demonstrated that high SNR inherent in IDEAL FSE consistently translates into high tissue contrast with image quality advantages in neck anatomy where large susceptibility variation and physiological motions reduce image quality for conventional FSE T1 and T2. However, the objective contrast estimates for parotid gland at isocenter were statistically equivalent for IDEAL and conventional FSE perhaps because at or near isocenter routine FSE works well. Additionally, fat specific IDEAL image sets add to diagnostic specificity for fat deficient lesions.

INTRODUCTION: Functional MR imaging is increasingly being used for presurgical language assessment in the treatment of patients with brain tumors, epilepsy, vascular malformations, and other conditions. The inherent complexity of fMRI, which includes numerous processing steps and selective analyses, is compounded by institution-unique approaches to patient training, paradigm choice, and an eclectic array of postprocessing options from various vendors. Consequently, institutions perform fMRI in such markedly different manners that data sharing, comparison, and generalization of results are difficult. The American Society of Functional Neuroradiology proposes widespread adoption of common fMRI language paradigms as the first step in countering this lost opportunity to advance our knowledge and improve patient care. LANGUAGE PARADIGM REVIEW PROCESS: A taskforce of American Society of Functional Neuroradiology members from multiple institutions used a broad literature review, member polls, and expert opinion to converge on 2 sets of standard language paradigms that strike a balance between ease of application and clinical usefulness. ASFNR RECOMMENDATIONS: The taskforce generated an adult language paradigm algorithm for presurgical language assessment including the following tasks: Sentence Completion, Silent Word Generation, Rhyming, Object Naming, and/or Passive Story Listening. The pediatric algorithm includes the following tasks: Sentence Completion, Rhyming, Antonym Generation, or Passive Story Listening. DISCUSSION: Convergence of fMRI language paradigms across institutions offers the first step in providing a “Rosetta Stone” that provides a common reference point with which to compare and contrast the usefulness and reliability of fMRI data. From this common language task battery, future refinements and improvements are anticipated, particularly as objective measures of reliability become available. Some commonality of practice is a necessary first step to develop a foundation on which to improve the clinical utility of this field.

BACKGROUND: Imaging criteria to evaluate the response of brain metastases to stereotactic radiosurgery (SRS) in the early posttreatment period remains a crucial unmet need. The aim of this study is to correlate early (within 12 wk) posttreatment perfusion MRI changes with long-term outcomes after treatment of lung cancer brain metastases with SRS. METHODS: Pre- and posttreatment perfusion MRI scans were obtained in patients treated with SRS for intact non–small cell lung cancer brain metastases. Time-dependent leakage (K(trans)), blood plasma volume (Vp), and extracellular extravascular volume (Ve) were calculated for each lesion. Patients were followed longitudinally with serial MRI until death, progression, or intervention (whole brain radiation or surgery). RESULTS: We included 53 lesions treated with SRS from 41 total patients. Median follow-up after treatment was 11 months. Actuarial local control at one year was 85%. Univariate analysis demonstrated a significant difference (P = 0.032) in posttreatment K(trans) SD between patients with progressive disease (mean = 0.0317) and without progressive disease (mean = 0.0219). A posttreatment K(trans) SD cutoff value of 0.017 was highly sensitive (89%) for predicting progressive disease and no progressive disease. Early posttreatment volume change was not associated with outcome (P = 0.941). CONCLUSION: Posttreatment K(trans) SD may be used as an early posttreatment imaging biomarker to help predict long-term response of lung cancer brain metastases to SRS. This can help identify patients who will ultimately fail SRS and allow for timelier adjustment in treatment approach. These data should be prospectively validated in larger patient cohorts and other histologies.

BACKGROUND AND PURPOSE: A recent study using task-based fMRI demonstrated that the middle frontal gyrus is comparable with Broca9s area in its ability to determine language laterality using a measure of verbal fluency. This study investigated whether the middle frontal gyrus can be used as an indicator for language-hemispheric dominance in patients with brain tumors using task-free resting-state fMRI. We hypothesized that no significant difference in language lateralization would occur between the middle frontal gyrus and Broca area and that the middle frontal gyrus can serve as a simple and reliable means of measuring language laterality. MATERIALS AND METHODS: Using resting-state fMRI, we compared the middle frontal gyrus with the Broca area in 51 patients with glial neoplasms for voxel activation, the language laterality index, and the effect of tumor grade on the laterality index. The laterality index derived by resting-state fMRI and task-based fMRI was compared in a subset of 40 patients. RESULTS: Voxel activations in the left middle frontal gyrus and left Broca area were positively correlated ( r = 0.47, P r = 0.56, P CONCLUSIONS: The middle frontal gyrus is comparable with the Broca area in its ability to determine hemispheric dominance for language using resting-state fMRI. Our results suggest the addition of resting-state fMRI of the middle frontal gyrus to the list of noninvasive modalities that could be used in patients with gliomas to evaluate hemispheric dominance of language before tumor resection. In patients who cannot participate in traditional task-based fMRI, resting-state fMRI offers a task-free alternate to presurgically map the eloquent cortex.

PURPOSE: Prediction of clinical outcomes in patients with primary central nervous system lymphoma (PCNSL) is important for optimization of treatment planning. Quantitative imaging biomarkers for PCNSL have not yet been established. This study evaluated the prognostic value of pretreatment dynamic contrast-enhanced MRI and diffusion-weighted imaging for progression-free survival (PFS) in patients with PCNSL. METHODS: Pretreatment dynamic contrast-enhanced MRI and diffusion-weighted imaging were retrospectively analyzed in 18 immunocompetent patients with PCNSL. Volumes of interest encompassing the tumors were assessed for measurements of blood plasma volume (Vp), volume transfer constant (K(trans)), and apparent diffusion coefficient. Patients were divided into short and long PFS groups based on median PFS. Imaging and clinical variables were correlated with PFS. RESULTS: Median PFS was 19.6 months. Lower Vp(mean) and K(trans)(mean) values increased risk for rapid progression (< 19.6 months). Receiver operating characteristic curve analysis demonstrated an optimal Vp(mean) cutoff value of 2.29 (area under the curve [AUC] = 0.74, sensitivity and specificity = 0.78, p = 0.023) for separating patients with short and long PFS. The optimal K(trans)(mean) cutoff was 0.08 (AUC = 0.74, sensitivity = 0.67, specificity = 0.78, p = 0.025). Kaplan–Meier survival analysis with log-rank test demonstrated significantly (p = 0.015) increased risk of rapid progression for patients with Vp(mean) < 2.29. Vp(mean) was significantly (p = 0.03) associated with PFS on univariate Cox analysis. Apparent diffusion coefficient values and clinical factors did not influence PFS. CONCLUSIONS: Pretreatment Vp and K(trans) derived from dynamic contrast-enhanced MRI may be novel prognostic quantitative imaging biomarkers of progression-free survival in patients with PCNSL. These data should be prospectively validated in larger patient cohorts.

The primary hand motor region is classically believed to be in the “hand knob” area in the precentral gyrus (PCG). However, hand motor task-based activation is often localized outside this area. The purpose of this study is to investigate the structural and functional connectivity driven by different seed locations corresponding to the little, index, and thumb in the PCG using probabilistic diffusion tractography (PDT) and resting-state functional magnetic resonance imaging (rfMRI). Twelve healthy subjects had three regions of interest (ROIs) placed in the left PCG: lateral to the hand knob (thumb area), within the hand knob (index finger area), and medial to the hand knob (little finger area). Connectivity maps were generated using PDT and rfMRI. Individual and group level analyses were performed. Results show that the greatest hand motor connectivity between both hemispheres was obtained using the ROI positioned just lateral to the hand knob in the PCG (the thumb area). The number of connected voxels in the PCG between the two hemispheres was greatest in the lateral-most ROI (the thumb area): 279 compared with 13 for the medial-most ROI and 9 for the central hand knob ROI. Similarly, the highest white matter connectivity between the two hemispheres resulted from the ROI placed in the lateral portion of PCG (p

Altered communication (hoarseness, dysphonia, and breathy voice) that can result from vocal fold paralysis, secondary to numerous etiologies, may be amenable to surgical restoration. In this article, both traditional and cutting-edge phonosurgical procedures targeting the symptoms resulting from vocal fold paralysis are reviewed, with emphasis on the characteristic imaging appearances of various injectable materials, implants, and augmentation procedures used in the treatment of vocal fold paralysis. In addition, complications of injection laryngoplasty and medialization laryngoplasty are illustrated. Familiarity with the expected imaging changes following treatment of vocal fold paralysis may prevent the misinterpretation of posttreatment changes as pathology. Identifying common complications related to injection laryngoplasty and localization of displaced implants is crucial in determining specific management in patients who have undergone phonosurgical procedures for the management of vocal fold paralysis.

Introduction This study examines the effect of family environment on language performance in children with myelomeningocele compared with age- and education-matched controls selected from the same geographic region. Methods Seventy-five monolingual (English) speaking children with myelomeningocele [males: 30; ages: 7–16 years; mean age: 10 years 1 month, standard deviation (SD) 2 years 7 months] and 35 typically developing children (males: 16; ages 7–16 years; mean age: 10 years 9 months, SD 2 years 6 months) participated in the study. The Comprehensive Assessment of Spoken Language (CASL) and the Wechsler tests of intelligence were administered individually to all participants. The CASL measures four subsystems: lexical, syntactic, supralinguistic and pragmatic. Parents completed the Family Environment Scale (FES) questionnaire and provided background demographic information. Standard independent sample t-tests, chi-squared and Fisher's exact tests were used to make simple comparisons between groups for age, socio-economic status, gender and ethnicity. Spearman correlation coefficients were used to detect associations between language and FES data. Group differences for the language and FES scores were analysed with a multivariate analysis of variance at a P-value of 0.05. Results For the myelomeningocele group, both Spearman correlation and partial correlation analyses revealed statistically significant positive relationships for the FES ‘intellectual–cultural orientation’ (ICO) variable and language performance in all subsystems (P < 0.01). For controls, positive associations were seen between: (1) ICO and lexical/semantic and syntactic subsystems; and (2) FES ‘independence’ and lexical/semantic and supralinguistic tasks. Conclusions The relationship between language performance and family environment appears statistically and intuitively sound. As in our previous study, the positive link between family focus on intellectually and culturally enhancing activities and language performance among children with myelomeningocele and shunted hydrocephalus remains robust. Knowledge of this relationship should assist parents and professionals in supporting language development through activities within the natural learning environment.

To prospectively determine anomalies of limbic tracts and to describe the relationship between these anomalies, seen on diffusion-tensor magnetic resonance (MR) and fiber tract (FT) reconstruction images, and learning and memory in children with myelomeningocele (MM) and Chiari II malformation.The investigation was HIPAA compliant and approved by institutional review boards; informed consent was obtained. In seven male and six female patients (aged 6 months to 16 years) with MM and Chiari II malformation, diffusion-tensor imaging and FT reconstruction were performed. FT reconstruction was generated with fractional anisotropy continuous tracking algorithm and manually drawn regions of interest. Limbic tract abnormalities were assessed on FT reconstruction images by an experienced pediatric neuroradiologist blinded to results of cognitive testing. Nine patients met criteria for memory and learning testing by a trained cognitive neuroscientist blinded to MR results. Exact Wilcoxon rank sum test was used to compare performance with learning and memory tasks in two groups.Eleven of 13 patients had defects within fornices and/or cingulum; three patients had aberrant fibers of cingulum. In nine patients, six had deficits in general memory; four, in learning; and four, in both. Atresia or hypoplasia of crura and body of fornices was noted in six patients with memory deficits and four patients with learning deficits. Five of six patients with memory deficits and three of four with learning deficits had hypoplasia or atresia of cingulum. Exact Wilcoxon rank sum test demonstrated significantly poorer performance for nonverbal immediate recall tasks in patients with anomalies of the fornix compared with those without (P = .04, exact two-tailed test).Diffusion-tensor and FT reconstruction images revealed that limbic fiber abnormalities were common in patients with MM and Chiari II malformation. Nonverbal immediate recall task performance appeared to be related to abnormalities of the fornix.

Background Previously, our studies demonstrated language differences impacting academic performance among children with myelomeningocele and shunted hydrocephalus (MMSH). This follow-up study considers the environmental facilitators within families (achievement orientation, intellectual–cultural orientation, active recreational orientation, independence) among a cohort of children with MMSH and their relationship to language performance. Methods Fifty-eight monolingual, English-speaking children (36 females; mean age: 10.1 years; age range: 7–16 years) with MMSH were evaluated. Exclusionary criteria were prior shunt infection; seizure or shunt malfunction within the previous 3 months; uncorrected visual or auditory impairments; prior diagnoses of mental retardation or attention deficit disorder. The Comprehensive Assessment of Spoken Language (CASL) and the Wechsler Abbreviated Scale of Intelligence (WASI) were administered individually to all participants. The CASL Measures four subsystems: lexical, syntactic, supralinguistic and pragmatic. Parents completed the Family Environment Scale (FES) questionnaire and provided background demographic information. Spearman correlation analyses and partial correlation analyses were performed. Results Mean intelligence scores for the MMSH group: full scale IQ 92.2 (SD = 11.9). The CASL revealed statistically significant difficulty for supralinguistic and pragmatic (or social) language tasks. FES scores fell within the average range for the group. Spearman correlation and partial correlation analyses revealed statistically significant positive relationships for the FES ‘intellectual–cultural orientation’ variable and performance within the four language subsystems. Socio-economic status (SES) characteristics were analyzed and did not discriminate language performance when the intellectual–cultural orientation factor was taken into account. Conclusion The role of family facilitators on language skills in children with MMSH has not previously been described. The relationship between language performance and the families’ value on intellectual/cultural activities seems both statistically and intuitively sound. Focused interest in the integration of family values and practices should assist developmental specialists in supporting families and children within their most natural environment.

Objective: This comparative cohort study examined language differences in young children (preschool to first grade) with myelomeningocele and shunted hydrocephalus (MM/SH). Method: A well-validated, standardized language test was administered to 17 children with MM/SH (age range 4–6 years) and 16 age-matched, nonaffected children. Results: Standard scores were obtained within lexical/semantic, syntactic, and pragmatic domains. An analysis of performance on individual subtests within the lexical/semantic domain was striking. Children with MM/SH performed significantly worse (p < 0.01) on a test measuring the comprehension of words representing important early learning concepts and on pragmatic tasks that measured their ability to use language functionally in social situations. Conclusions: Words representing basic precepts and concepts are commonly used in instructions to children in early childhood. Difficulty with functional pragmatic language results in suboptimal communication skills. Academic and community implications are discussed.

The early detection and specific diagnosis of viral infections of the central nervous system are important because many of these diseases are potentially treatable. However, clinical symptoms and physical examination are often nonspecific, and rapid diagnostic tests are available for some, but not all, viruses. Neuroimaging, in conjunction with clinical history and laboratory tests, plays an important role in narrowing the differential diagnoses. In this article, we review the clinical features, imaging characteristics, diagnosis, and treatment of the more common viral infections and prions that involve the central nervous system.

To characterize the MRI features of the petromastoid canal in children with sensorineural hearing loss (SNHL) and in normal infants.High resolution MRI examinations of 564 children who were evaluated for SNHL and brain MRI examinations of 112 infants who had normal studies were studied independently by two reviewers.In SNHL group, visibility of the PMC decreased for right and left PMC (P0.001). The width of the right PMC significantly decreased as age increased (P0.0001). There was no relation between abnormalities of membranous labyrinth and cochlear nerve and PMC visibility in children with SNHL (p0.05). In the normal group, the PMC visibility decreased with increasing age (right P = 0.0001, left P = 0.001). In the normal group also, as age increased, the PMC width decreased for both PMCs (right, P = 0.0006; left, P = 0.03).The PMC is more frequently visualized in young children. Its visibility and width are not associated with abnormalities of membranous labyrinth and cochlear nerves.

Hypertrophy of the optic nerves and optic chiasm is described in a 5-month-old boy with infantile Krabbe disease. Optic nerve and optic chiasm hypertrophy is a rarely described feature of Krabbe disease. The areas of the prechiasmatic optic nerves and optic chiasm were measured and compared with those of 26 age-matched controls. The areas of the prechiasmatic optic nerves and optic chiasm were 132% and 53% greater than normal, respectively.

Purpose To compare objective and subjective image quality in neck CT images acquired at different tube current–time products (275 mA s and 340 mA s) and reconstructed with filtered-back-projection (FBP) and adaptive statistical iterative reconstruction (ASIR). Materials and methods HIPAA-compliant study with IRB approval and waiver of informed consent. 66 consecutive patients were randomly assigned to undergo contrast-enhanced neck CT at a standard tube-current–time-product (340 mA s; n = 33) or reduced tube-current–time-product (275 mA s, n = 33). Data sets were reconstructed with FBP and 2 levels (30%, 40%) of ASIR-FBP blending at 340 mA s and 275 mA s. Two neuroradiologists assessed subjective image quality in a blinded and randomized manner. Volume CT dose index (CTDIvol), dose-length-product (DLP), effective dose, and objective image noise were recorded. Signal-to-noise ratio (SNR) was computed as mean attenuation in a region of interest in the sternocleidomastoid muscle divided by image noise. Results Compared with FBP, ASIR resulted in a reduction of image noise at both 340 mA s and 275 mA s. Reduction of tube current from 340 mA s to 275 mA s resulted in an increase in mean objective image noise (p = 0.02) and a decrease in SNR (p = 0.03) when images were reconstructed with FBP. However, when the 275 mA s images were reconstructed using ASIR, the mean objective image noise and SNR were similar to those of the standard 340 mA s CT images reconstructed with FBP (p > 0.05). Subjective image noise was ranked by both raters as either average or less-than-average irrespective of the tube current and iterative reconstruction technique. Conclusion Adapting ASIR into neck CT protocols reduced effective dose by 17% without compromising image quality.

To determine whether there are detectable differences in tensor metrics between children who read normally and children with simple developmental dyslexia and/or differences between the right and left hemispheres in these groups by using 3.0-T diffusion-tensor (DT) magnetic resonance (MR) imaging focused on the superior longitudinal fasciculus (SLF), inferior fronto-occipital and inferior longitudinal fasciculi (IFO-ILF), and posterior limb of the internal capsule (PLIC).This was a prospective, HIPAA-compliant institutional review board-approved investigation with written informed parental consent. Nineteen English-speaking, right-handed children with a normal IQ and developmental dyslexia (16 male, three female; age range, 6-16 years; mean age, 9.9 years) and 18 normal-reading, age-matched pediatric control subjects (13 male, five female; age range, 6-15 years; mean age, 10.0 years) underwent DT imaging (30 directions, three signals acquired, voxel size of 2 mm). Regions of interest were placed on the SLF, IFO-ILF, and PLIC, and tensor metrics were calculated. Statistical analyses of differences in cognitive function between the dyslexic and control groups were performed by using the two-sample t test. Differences in tensor metrics were examined by using analysis of covariance models.In the control subjects, the fractional anisotropy (FA) of all tracts studied increased with age. In the dyslexic subjects, the age-related increases in FA in the SLF were most similar to those in the control subjects (P = .504), while mean FA values for the IFO-ILF (P = .009) and PLIC (P.0001) were higher than those in the control subjects up to around 11 years of age, after which they were lower. Apparent diffusion coefficients consistently decreased in both groups. There was a nonsignificant increase in mean axial diffusivity in the IFO-ILF in the control group but not in the dyslexia group. Increases in axial diffusivity seen in the PLIC in the control group were not seen in the dyslexia group. There were no marked differences in tensor metrics between the left and right hemispheres within or between the two groups.Findings at 3.0-T DT imaging suggest that white matter differences in dyslexic children are not limited to the portion of the brain traditionally considered to be integral to word recognition and processing.

Objective. Description of temperament profiles of children has largely been reported in typically developing populations. Children undergo individual- ized assessments of achievement (developmental/aca- demic) and receive individualized interventions. In con- trast, their individual behavioral styles are not evaluated as completely, if at all. For children with developmental disabilities, description of temperament characteristics can provide better understanding of the already complex child. This study describes temperament characteristics in a group of children with myelomeningocele and shunted hydrocephalus (MM/SH). Methods. A consecutive cohort study with historical control group measuring temperament characteristics was conducted at a tertiary-level, university affiliated, interdisciplinary spina bifida program. Analysis in- cludes group comparisons. Primary caregivers of 46 chil- dren (age range: 5-12 years) with MM/SH completed age-appropriate Carey Temperament Scales question- naires as a component of a larger developmental study. The Carey Temperament Scales comprise a series of be- havioral rating instruments that assess 9 temperament characteristics: activity, adaptability, approach-with- drawal, mood, intensity, attention/persistence, distracti- bility, sensory threshold, and rhythmicity/predictability. The Carey questionnaires used in this study were (1) the Behavioral Style Questionnaire for children aged 5 to 7 years and (2) the Middle Childhood Temperament Ques- tionnaire for those aged 8 to 12 years. Both question- naires assess the same temperament characteristics; the contexts within which items were rated were designed to better reflect developmental levels. Children were ex- cluded when there were comorbid diagnoses of atten- tion-deficit/hyperactivity disorder or mental retardation. Results. One-sample normal tests (Bonferroni cor- rected) revealed that children within the MM/SH group differed significantly from the standardized population in 5 dimensions: (1) adaptability (less adaptable), (2) ap- proach/withdrawal (poor first approach/greater with- drawal), (3) distractibility (more distractible), (4) atten- tion/persistence (less attentive/persistent), and (5) predictability/rhythm (less predictable). Caregiver per- ceptions of having a difficult-to-manage child were sig- nificantly correlated with negative mood, more intensity of response, and less adaptability. Conclusions. Temperament profiles previously de- scribed in typically developing populations (eg, "easy" or "difficult child") were not prominent profiles in this group of children with MM/SH. A constellation of tem- perament characteristics not commonly recognized may place these children at risk for being contributors to and recipients of misunderstandings of social cues and have implications for successful learning within academic, home/community, and medical settings. Pediatrics 2005; 115:e58-e63. URL: www.pediatrics.org/cgi/doi/10.1542/ peds.2004-0797; hydrocephalus, myelomeningocele, tem- perament, parental perceptions, early intervention.