Acute Dystonic Reaction After Propofol Administration: A Pediatric Case Report.

We present a case of a 12-year-old female with a history of infantile spasms who developed a propofol-associated acute dystonic reaction after emergence from general anesthesia for foot surgery. Uniquely, the patient's postoperative symptoms of an acute dystonic reaction were refractory to standard treatment with anticholinergics but were successfully treated with corticosteroids. The absence of any dystonic symptoms following subsequent foot surgery under general anesthesia without propofol supported a propofol-associated etiology.

Retrospective analysis of neurological findings in esophageal atresia: Allostatic load of disease complexity, cumulative sedation, and anesthesia exposure.

Background: There is limited knowledge regarding the impact of perioperative critical care on frequency of neurological imaging findings following esophageal atresia (EA) repair.

Methods: This is a retrospective study of infants (n = 70) following EA repair at a single institution (2009-2020). Sex, gestational age at birth, type of surgical repair, underlying disease severity, and frequency of neurologic imaging findings were obtained.

Infant Perioperative Risk Factors and Adverse Brain Findings Following Long-Gap Esophageal Atresia Repair.

Recent findings implicate brain vulnerability following long-gap esophageal atresia (LGEA) repair. We explored the relationship between easily quantifiable clinical measures and previously reported brain findings in a pilot cohort of infants following LGEA repair. MRI measures (number of qualitative brain findings; normalized brain and corpus callosum volumes) were previously reported in term-born and early-to-late premature infants (n = 13/group) <1 year following LGEA repair with the Foker process.

Infant Brain Structural MRI Analysis in the Context of Thoracic Non-cardiac Surgery and Critical Care.

To determine brain magnetic resonance imaging (MRI) measures of cerebrospinal fluid (CSF) and whole brain volume of full-term and premature infants following surgical treatment for thoracic non-cardiac congenital anomalies requiring critical care. Full-term ( = 13) and pre-term ( = 13) patients with long-gap esophageal atresia, and full-term naïve controls ( = 19) < 1 year corrected age, underwent non-sedated brain MRI following completion of thoracic non-cardiac surgery and critical care treatment. Qualitative MRI findings were reviewed and reported by a pediatric neuroradiologist and neurologist.

Clinical and genetic characterization of AP4B1-associated SPG47.

The hereditary spastic paraplegias (HSPs) are a heterogeneous group of disorders characterized by degeneration of the corticospinal and spinocerebellar tracts leading to progressive spasticity. One subtype, spastic paraplegia type 47 (SPG47 or HSP-AP4B1), is due to bi-allelic loss-of-function mutations in the AP4B1 gene. AP4B1 is a subunit of the adapter protein complex 4 (AP-4), a heterotetrameric protein complex that regulates the transport of membrane proteins.

3D Super-Resolution Motion-Corrected MRI: Validation of Fetal Posterior Fossa Measurements.

Purpose: Current diagnosis of fetal posterior fossa anomalies by sonography and conventional MRI is limited by fetal position, motion, and by two-dimensional (2D), rather than three-dimensional (3D), representation. In this study, we aimed to validate the use of a novel magnetic resonance imaging (MRI) technique, 3D super-resolution motion-corrected MRI, to image the fetal posterior fossa.

Methods: From a database of pregnant women who received fetal MRIs at our institution, images of 49 normal fetal brains were reconstructed.

Increased pediatric functional neurological symptom disorders after the Boston marathon bombings: a case series.

Background: Functional neurological symptom disorders are frequently the basis for acute neurological consultation. In children, they are often precipitated by high-frequency everyday stressors. The extent to which a severe traumatic experience may also precipitate functional neurological abnormalities is unknown.

Turner syndrome and meningioma: support for a possible increased risk of neoplasia in Turner syndrome.

Neoplasia is uncommon in Turner syndrome, although there is some evidence that brain tumors are more common in Turner syndrome patients than in the general population. We describe a woman with Turner syndrome (45,X) with a meningioma, in whom a second neoplasia, basal cell carcinomas of the scalp and nose, developed five years later in the absence of therapeutic radiation. Together with 7 cases of Turner syndrome with meningioma from a population-based survey in the United Kingdom, and 3 other isolated cases in the literature, we review this small number of patients for evidence of risk factors related to Turner syndrome, such as associated structural anomalies or prior treatment.

Magnetic resonance volumetric assessments of brains in fetuses with ventriculomegaly correlated to outcomes.

Objectives: The purpose of this study was to correlate 2-dimensional magnetic resonance (MR) measurements of lateral ventricular width and 3-dimensional measurements of lateral ventricular and supratentorial parenchymal volumes to postnatal outcomes in fetuses with ventriculomegaly.

Methods: A total of 307 fetuses (mean gestational age, 26.0 weeks; range, 15.

Guillain-Barré syndrome in a child with pain: lessons learned from a late diagnosis.

Unlabelled: Children with Guillain-Barré Syndrome (GBS) often do not present like adults with an ascending paralysis and sensory abnormalities, but typically have pain and gait difficulties as predominant symptoms. We present a case of paediatric GBS that was not diagnosed until late in the course because of limited neurological examination, erroneous interpretation of newly acquired data and insufficient familiarity with the disorder in children. Through this case, essentials of paediatric GBS are reviewed.

Molecular basis for preferential protective efficacy of antibodies directed to the poorly acetylated form of staphylococcal poly-N-acetyl-beta-(1-6)-glucosamine.

Poly-N-acetyl-glucosamine (PNAG) is a staphylococcal surface polysaccharide influencing biofilm formation that is also under investigation for its vaccine potential. Antibodies that bind to PNAG with either low (<15%) or high (>90%) levels of acetate are superior at opsonic and protective activity compared with antibodies that bind to PNAG with only high levels (>70%) of acetate. PNAG is synthesized by four proteins encoded within the intercellular adhesin (ica) locus icaADBC.

The teicoplanin-associated locus regulator (TcaR) and the intercellular adhesin locus regulator (IcaR) are transcriptional inhibitors of the ica locus in Staphylococcus aureus.

Infections involving Staphylococcus aureus are often more severe and difficult to treat when the organism assumes a biofilm mode of growth. The polysaccharide poly-N-acetylglucosamine (PNAG), also known as polysaccharide intercellular adhesin, is synthesized by the products of the intercellular adhesin (ica) locus and plays a key role in biofilm formation. Numerous conditions and exogenous factors influence ica transcription and PNAG synthesis, but the regulatory factors and pathways through which these environmental stimuli act have been only partially characterized.