Object The authors conducted a study to correlate histopathological features, MRI findings, and postsurgical outcomes in children with cortical dysplasia (CD) by performing a novel resection site–specific evaluation. Methods The study cohort comprised 43 children with intractable epilepsy and CD. The MR image review was blinded to pathology but with knowledge of the resection location. An MRI score (range 0–7) was calculated for each resection region based on the number of imaging features of CD and was classified as “lesional” or “nonlesional” according to all imaging features. Outcome was determined using the International League Against Epilepsy (ILAE) scale. The determination of pathological CD type was based on the ILAE 2011 consensus classification system, and the cortical gliosis pattern was assessed on GFAP staining. Results There were 89 resection regions (50 ILAE Type I, 29 Type IIa, and 10 Type IIb). Eleven (25.6%) of 43 children had more than one type of CD. The authors observed MRI abnormalities in 63% of patients, characteristic enough to direct resection (lesional) in 42%. Most MRI features, MRI score ≥ 3, and lesional abnormalities were more common in patients with Type II CD. Increased cortical signal was more common in those with Type IIb (70%) rather than Type IIa (17.2%) CD (p = 0.004). A good outcome was demonstrated in 39% of children with Type I CD and 72% of those with Type II CD (61% in Type IIa and 100% in Type IIb) (p = 0.03). A lesional MRI abnormality and an MRI score greater than 3 correlated with good outcome in 78% and 90% of patients, respectively (p < 0.03). Diffuse cortical gliosis was more prevalent in Type II CD and in resection regions exhibiting MRI abnormalities. Complete surgical exclusion of the MRI abnormality was associated with a better postoperative outcome. Conclusions This study provides a detailed correlation of MRI findings, neuropathological features, and outcomes in children with intractable epilepsy by using a novel resection site–specific evaluation. Because 25% of the patients had multiple CD subtypes, a regional analysis approach was mandated. Those children with lesional MRI abnormalities, Type II CD, and surgical exclusion of the MRI abnormality had better outcomes. Type II CD is more detectable by MRI than other types, partly because of the greater extent of associated gliosis in Type II. Although MRI findings were correlated with the pathological CD type and outcome in this study, the majority of patients (58%) did not have MRI findings that could direct surgical therapy, underscoring the need for improved MRI techniques for detection and for the continued use of multimodal evaluation methods in patient selection.

BACKGROUND AND PURPOSE: Group B Streptococcus and Escherichia coli (E coli) are the 2 most common causes of bacterial meningitis in neonates. The purpose of this study was to determine whether CSF and/or MR imaging findings differ between infants with group B streptococcal or E coli meningitis. MATERIALS AND METHODS: A retrospective review was performed among neonates (younger than 28 days) and infants (younger than 120 days) with proved group B streptococcal (n ϭ 57) or E coli meningitis (n ϭ 50). A CSF or blood culture positive for Streptococcus or E coli and an elevated CSF white blood cell count were used as the criterion standard. Independent, blinded review of brain MRIs obtained within 21 days of presentation were performed by 2 board-certified neuroradiologists. CSF laboratory values and MR imaging findings were compared between the groups. RESULTS: There was no statistically significant difference between the mean age at presentation for patients with group B streptococcal (40 days; range, 2-111 days) versus patients with E coli meningitis (31 days; range, 12-115 days) (P ϭ .18). There was no statistically significant difference in the CSF white blood cell count, glucose, or protein. There was a significant difference between group B streptococcal and E coli meningitis in the frequency of hydrocephalus (0% versus 22%, P ϭ .001) and infarct (40% versus 14%; P ϭ .038), respectively. There was no statistically significant difference in leptomeningeal enhancement, cerebritis, ventriculitis, abscess/granuloma, subdural effusion, extra-axial purulent material, intraventricular purulent material, hemorrhage, and sinus thrombosis. CONCLUSIONS: Although neonates and infants with group B streptococcal or E coli meningitis had similar age and CSF laboratory values, patients with group B streptococcal meningitis more frequently demonstrated infarcts, while those with E coli meningitis more frequently had early onset of hydrocephalus.

We report on the conventional and diffusion tensor imaging (DTI) findings of a 2-year-old child with clinical presentation of Joubert's Syndrome (JS) and brainstem structural abnormalities as depicted by neuroimaging.Conventional magnetic resonance imaging (MRI) showed a “molar tooth” configuration of the brainstem. A band-like formation coursing in an apparent axial plane anterior to the interpeduncular fossa was noted and appeared to partially cover the interpeduncular fossa.DTI maps and three-dimensional (3D) tractography demonstrated a prominent red-encoded white matter bundle anterior to the midbrain. Probable aberrant course of the bilateral corticospinal tracts (CST) was also depicted. Absence of the decussation of the superior cerebellar peduncles and elongated thickened, horizontal superior cerebellar peduncle (SCP) reflecting the molar tooth sign were also shown.Our report and the review of the published cases suggest that DTI and tractography may be very helpful to differentiate between interpeduncular heterotopias and similarly located white matter bundles corroborating the underlying etiology of axonal guidance disorders in the complex group of ciliopathies including JS. Our case represents an important additional puzzle piece to explore the variability of these ciliopathies.

Liver transplantation treats the hepatic affectation of urea cycle defects (UCDs), however irreversible neurologic damage pre-transplant is difficult to assess providing transplant teams with ethical dilemmas for liver transplantation. The purpose of our study was to determine whether pre-transplant neuroimaging can predict developmental outcomes post-liver transplant in children with UCDs. Methods Patients undergoing liver transplantation for UCD’s at Cincinnati Children’s between 2002 & 2012 were identified. Neurologic assessments prior to and after transplantation were categorized into mild, moderate, or severe disability. Neuroimaging data were categorized into mild, moderate, or severe by a single pediatric neuro-radiologist. Results 15 patients were identified of whom 8 had neuroimaging prior to transplantation. Of the 8 patients that had neuroimaging, 4 were categorized as severe, 1 moderate, and 3 had no to mild delay. All 4 patients whose imaging was severe were found to have moderate to severe neurologic delay. Of the 3 patients with no to mild changes on neuroimaging 2 out of 3 were found to have no to mild delay on developmental assessments after transplantation. Conclusion Neuroimaging may be a helpful tool in determining developmental prognosis and outcomes post-liver transplantation for UCDs. Further studies maybe needed to validate our preliminary findings.

BACKGROUND AND PURPOSE: Bacterial meningitis most commonly affects young children and can result in critical adverse outcomes, including sensorineural hearing loss (SNHL). The purpose of this study is to determine the diagnostic accuracy of MR imaging for predicting the development of SNHL among infants with bacterial meningitis. MATERIALS AND METHODS:A retrospective review was performed among infants (age ,365 days) with bacterial meningitis (n ¼ 115). Independent and consensus blinded review of brain MRIs (n ¼ 239) performed less than 90 days from presentation were conducted. Abnormal appearance of the inner ear was defined as enhancement on postcontrast T1-weighted (T1-weighted1C) sequence and FLAIR hyperintensity. The consensus MR imaging appearance of the inner ear on FLAIR, T1-weighted1C, and combined evaluation was compared with criterion standard audiometric testing to determine the sensitivity and specificity of MR imaging for detecting SNHL. RESULTS:The mean age at diagnosis of bacterial meningitis was 50.6 days (range, 0-338 days) and 24.3% had SNHL. Sensitivity and specificity was 0.61/0.96, 0.50/0.94, and 0.61/0.94 for T1-weighted1C, FLAIR hyperintensity, and combined evaluation, respectively, for prediction of SNHL. There was excellent interobserver agreement for both the T1-weighted1C and FLAIR sequences and combined evaluation for presence of abnormal enhancement and hyperintense signal, respectively. Factors associated with abnormal MR imaging findings on T1-weighted1C and/or FLAIR in patients with SNHL included low CSF glucose (P ¼ .04, .02) and high CSF protein (P ¼ .04, .03). CONCLUSIONS:Abnormal enhancement and/or FLAIR hyperintensity of the inner ear demonstrate high specificity and average sensitivity for prediction of SNHL among infants with bacterial meningitis.