Discrete white matter abnormalities at age 8-11 years in children born extremely preterm are not associated with adverse cognitive or motor outcomes.

AIM: Little is known about the prevalence of discrete white matter abnormalities (WMA) beyond the first years in children born extremely preterm (EPT) and the relation to neurodevelopmental outcomes. Our aim was to investigate the prevalence of discrete WMA in children born EPT and the relationship to neonatal white matter injuries (WMI), white matter (WM) volume, WM diffusivity and neurodevelopment. METHODS: The study was a part of a longitudinal follow-up study of EPT neonates. All children were scanned at Karolinska University hospital 2004-2007 (neonates) and 2014-2015 (children at 8-11 years). WMA was qualitatively assessed by visual inspection. Developmental assessment was conducted at 12 years. RESULTS: In total, 112 children (median age 10.3 years, 56 girls) underwent MRI of the brain (68 EPT, 45 controls). In the EPT group, a subset had MRI around term equivalent age (n = 61). In the EPT group, the prevalence of discrete WMA at 8-11 years was 52%. There was a positive association between WMI at TEA and 8-11 years. There was no association between WMI and WM volumes or diffusivity at 8-11 years. Discrete WMA was not related to neurodevelopmental outcomes. CONCLUSION: Discrete WMA was prevalent in children born EPT at 8-11 years but were not related to neurodevelopmental outcomes.

Glioneuronal tumors in childhood - Before and after surgery. A long-term follow-up study.

AIM: To give a detailed description of the long-term outcome of a cohort of children with glioneuronal tumors regarding pre- and postsurgical factors, including "dual" and "double" pathology, seizure freedom, and psychosocial outcome. METHODS: During a fifteen-year period (1995-2009), all patients (age 0-17.99years) with a glioneuronal brain tumor diagnosed and treated at Uppsala University Children's Hospital were identified from the National Brain Tumor Registry and the National Epilepsy Surgery Registry. Hospital medical records were reviewed and neuroradiological and neuropathological findings were re-evaluated. A cross-sectional long-term follow-up prospective evaluation, including an interview, neurologic examination, and electroencephalogram, was accomplished in patients accepting participants in the study. RESULTS: A total of 25 out of 28 (89%) eligible patients were included. The M:F ratio was 1.5:1. Mean follow-up time after surgery was 12.1years (range 5.0-19.3). Twenty patients were adults (>18years) at follow-up. Seizure freedom was achieved in 64%. Gross total resection (GTR) was the only preoperative factor significantly correlating to seizure freedom (p=0.027). Thirty-eight percent were at some time postoperatively admitted for a psychiatric evaluation. There was a trend towards both higher educational level and employment status in adults who became seizure free. CONCLUSION: Long-term outcome is good regarding seizure freedom if GTR can be achieved, but late seizure recurrence can occur. "Dual" and "double" pathology is uncommon and does not influence seizure outcome. Obtaining seizure freedom seems to be important for psychosocial outcome, but there is a risk for psychiatric comorbidities and long-term follow-up by a multi-professional team is advisable.

Visualization of the fetal lip and palate: is brain-targeted MRI reliable?

Objective : To evaluate the ability of brain-targeted magnetic resonance imaging (MRI) to assess the anatomy of the fetal upper lip and palate. Design : Two independent readers made a blind retrospective review of 60 brain-targeted MRIs of fetuses from 20 to 38 gestational weeks (GW). Fifty-five MRIs were normal and five had orofacial anomalies, including one isolated cleft lip and four cleft lip and palate. Both normal and cleft MRIs had postnatal confirmation. The upper lip, primary palate, secondary palate, and nasal septum were scored into four levels, from evidently normal to evidently abnormal. In case of a suspected pathology, the readers attempted a diagnosis. Setting : Collaboration between a university hospital and a large private practice MRI center. Results : Interobserver agreement (weighted kappa) was 0.79 for the upper lip, 0.70 for the primary palate, 0.86 for the secondary palate, and 0.90 for the nasal septum. The scoring levels of the readers did not change significantly across gestational age. Normality was correctly scored in 96% to 100% of the normal lips and primary palates and in 93% to 97% of the normal secondary palates depending on the reader. A deviated septum was only scored in two fetuses with unilateral cleft palates. The readers identified all pathological cases. Conclusion : Brain-targeted fetal MRI in experienced hands seems to be highly accurate for the evaluation of the lip and palate in fetuses above 20 GW, regardless of gestational age. The assessment of the secondary palate may be slightly more limited than the lip or primary palate.

The ear in fetal MRI: what can we really see?

INTRODUCTION: The aim of this study was to investigate the ability to depict the components of the ear on brain-oriented fetal MRI studies. METHODS: Retrospective evaluation of the ear in MRI studies was performed post-mortem in 16 fetuses ranging from 15 to 22 gestation weeks (GW), and in 122 examinations in vivo of fetuses ranging from 20 to 38 GW. The cochlea, vestibular apparatus, middle ear, and external auditory canal were separately graded according to the components that were delineated. RESULTS: The components of the inner and middle ear were fully delineated in 100% of the post-mortem examinations, but the external auditory canals were only seen in only 25%. In the in vivo group, the imaging detail was much lower. Cochlear turns could be identified in 75% of the fetuses, the vestibule and the lateral semicircular canals in 72% andossicles in 70%. Before 25 GW, the ability to identify these individual parts was 50%, 30%, and 33%, respectively, and above it was 89%, 93%, and 90% . In most cases, the external auditory canals could only be seen after 29 GW. CONCLUSION: In fetal MRI studies in vivo, it is possible to depict the components of the ear in the majority of the fetuses, in such a manner as to exclude major malformations. However, MRI might not provide enough detail to rule out pathology of the ear before 25 GW, this being a critical age for pregnancy management in many countries.

Measurements of the normal fetal brain at gestation weeks 17 to 23: a MRI study.

INTRODUCTION: To obtain measurements of the normal fetal brain before 24 weeks of gestation (GW), a deadline for medical decisions on fetal viability in a large number of countries. METHODS: We retrospectively reviewed 70 normal MR examinations of fetuses aged GW 17 to 23. The fronto-occipital diameter, the cerebral bi-parietal diameter, the transverse cerebellar diameter, the vermian height, and antero-posterior diameter were measured. RESULTS: The median, maximum, and minimum values for each parameter were displayed for each individual GW. CONCLUSION: The recorded data might contribute to a better assessment of fetal health by providing normal boundaries for the brain growth.

Superficial siderosis: a case report.

Superficial siderosis of the central nervous system is the result of chronic recurrent hemorrhages (e.g., arteriovenous malformations, tumors, or trauma), which leads to the accumulation of cytotoxic hemosiderin and presents with hearing loss, cerebellar dysfunction, and myelopathy. This article presents a clinical case of an 11-year-old boy in whom the diagnosis of medulloblastoma was established. He underwent surgery, and after a few years, he began to complain of hearing loss. Magnetic resonance imaging revealed the cause of the hearing disturbance. The aim of this article is to review the recent literature related to the etiology, clinical and radiologic features of superficial siderosis, emphasizing the role of magnetic resonance imaging.

Accuracy and Reliability of X-ray Measurements in the Cervical Spine.

STUDY DESIGN: This study is a post hoc analysis of a multicenter prospective randomized controlled trial which compared artificial disc replacement and anterior cervical discectomy and fusion. PURPOSE: Useful radiographic parameters for assessing cervical alignment include the Cobb angles, T1 slope (T1S), occipitocervical inclination (OCI), K-line tilt (KLT), and cervical sagittal vertical axis (cSVA). This study aimed to determine measurement accuracy and reliability for these parameters. OVERVIEW OF LITERATURE: Various authors have assessed repeatability by comparing different methods of measurement, but knowledge of measurement error and minimal detectable change is scarce. METHODS: We evaluated 758 lateral cervical radiographs. One medical student and one spine surgeon (i.e., measured ×2 within 4 weeks) independently measured the parameters obtaining 5,850 values. Standard error of measurement (SEm) and minimum detectable change (MDC) were calculated for each parameter. The accuracy and reliability of the Cobb angle measurements were calculated for the different types of angles: cervical lordosis, prosthesis angle, segmental angle with two bone surfaces (SABB), and segmental angle with one bone and one metal surface. Reliability was determined with intraclass correlation coefficient (ICC). RESULTS: SEm was 1.8° and MDC was 5.0° for the Cobb angle, with an intraobserver/interobserver ICC of 0.958/0.886. All the different subtypes of Cobb angles had an ICC higher than 0.950, except SABB (intraobserver/interobserver ICC of 0.922/0.716). The most accurate and reliable measurement was for KLT. CONCLUSIONS: This study provides normative data on SEm and MDC for Cobb angles, T1S, KLT, OCI, and cSVA in cervical lateral radiographs. Reliability was excellent for all parameters except SABB (e.g., good).

Prediction of clinical outcome in subacute subarachnoid hemorrhage using diffusion tensor imaging.

OBJECTIVEClinical outcome in nontraumatic subarachnoid hemorrhage (SAH) is multifactorial and difficult to predict. Diffusion tensor imaging (DTI) findings are a prognostic marker in some diseases such as traumatic brain injury. The authors hypothesized that DTI parameters measured in the subacute phase of SAH can be associated with a poor clinical outcome.METHODSDiffusion tensor imaging was prospectively performed in 54 patients at 8-10 days after nontraumatic SAH. Logistic regression analysis was performed to evaluate the association of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values with a poor clinical outcome (modified Rankin Scale score ≥ 3) at 3 months.RESULTSAt 8-10 days post-SAH, after adjusting for other variables associated with a poor outcome, an increased ADC at the frontal centrum semiovale was associated with a poor prognosis (OR estimate 1.29, 95% CI 1.04-1.60, p = 0.020). Moreover, an increase of 0.1 in the FA value at the corpus callosum at 8-10 days after SAH corresponded to 66% lower odds of having a poor outcome (p = 0.002).CONCLUSIONSDecreased FA and increased ADC values in specific brain regions were independently associated with a poor clinical outcome after SAH. This preliminary exploratory study supports a potential role for DTI in predicting the outcome of SAH.

[Merosin-positive congenital muscular dystrophy, white matter abnormalities, and bilateral posterior occipital cortical dysplasia]. / Distrofia muscular congénita merosina positiva, anormalidades da substância branca e displasia cortical occipital posterior bilateral.

Congenital muscular dystrophy (CMD) is one of the most frequent dystrophies of childhood, which is commonly characterized by neonatal muscle impairment with or without clinical evidence of central nervous system involvement. CMDs were classified into five clinically distinct forms: the two classical CMDs with and without deficit of the a2 laminin chain (merosin) caused by mutations on chromosome 6q2, the Fukuyama CMD (severe form, initially described in Japanese patients and recently linked to the chromosome 9q31-33), Walker-Warburg syndrome and the muscle-eye-brain disease described in Finnish patients. The majority of these forms have severe clinical and imagiological involvement of SNC. This aspect is rarely observed on classical CMD, particularly in the merosin-positive form. We describe a case of a 28 year-old woman, with clinical and histopathological signs of classical CMD merosin-positive (no deficient), without mental retardation, but with epilepsy. MRI T2 weighted images, revealed diffuse and symetrical high signal white matter of both cerebral hemispheres, affecting corpos calosum, posterior arms of internal capsules and the piramidal tract to mesencephalon. It also disclosed diffuse and symetrical high signal of basal ganglia, specially, the head of caudate nuclei. These were associated with bilateral occipital posterior cortical dysplasia. The observed imagiological pattern could represent a new subtype of CMD, hybrid between classical CMD and the severe forms, however it is not clear where it fits in the spectrum. This case denotes the possible envolvement of SNC in patients merosin-positives. Based on this findings we suggest doing MRI scans to all patients with CMD no deficient in merosin.