- Fetal and neonatal neurogenetics. [Journal Article]
- HCHandb Clin Neurol 2019; 162:105-132
- Disorders of the developing nervous system may be of genetic origin, comprising congenital malformations of spine and brain as well as metabolic or vascular disorders that affect normal brain develop…
Disorders of the developing nervous system may be of genetic origin, comprising congenital malformations of spine and brain as well as metabolic or vascular disorders that affect normal brain development. Acquired causes include congenital infections, hypoxic-ischemic or traumatic brain injury, and a number of rare neoplasms. This chapter focuses on the clinical presentation and workup of neurogenetic disorders presenting in the fetal or neonatal period. After a summary of the most frequent clinical presentations, clues from history taking and clinical examination are illustrated with short case reports. This is followed by a discussion of the different tools available for the workup of neurogenetic disorders, including the various genetic techniques with their advantages and disadvantages. The implications of a molecular genetic diagnosis for the patient and family are addressed in the section on counseling. The chapter concludes with a proposed workflow that may help the clinician when confronted with a potential neurogenetic disorder in the fetal or neonatal period.
- Muenke syndrome: long-term outcome of a syndrome-specific treatment protocol. [Journal Article]
- JNJ Neurosurg Pediatr 2019 Jul 19; :1-8
- CONCLUSIONS: Patients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.
- Feasibility of fast brain diffusion MRI to quantify white matter injury in pediatric hydrocephalus. [Journal Article]
- JNJ Neurosurg Pediatr 2019 Jul 19; :1-8
- CONCLUSIONS: The utility of fast brain MRI can be extended beyond anatomical assessments to obtain dMRI measures. A reduction in PLIC and gCC MD to levels similar to those of controls was observed within 14 months following shunt surgery for hydrocephalus in PHH and CH infants. Further studies are required to assess the role of fast brain dMRI for assessing clinical outcomes in pediatric hydrocephalus patients.
- Human Cord Blood-Derived Unrestricted Somatic Stem Cell Infusion Improves Neurobehavioral Outcome in a Rabbit Model of Intraventricular Hemorrhage. [Journal Article]
- SCStem Cells Transl Med 2019 Jul 19
- Intraventricular hemorrhage (IVH) is a severe complication of preterm birth, which leads to hydrocephalus, cerebral palsy, and mental retardation. There are no available therapies to cure IVH and sta…
Intraventricular hemorrhage (IVH) is a severe complication of preterm birth, which leads to hydrocephalus, cerebral palsy, and mental retardation. There are no available therapies to cure IVH and standard treatment is supportive care. Unrestricted somatic stem cells (USSCs) from human cord blood have reparative effects in animal models of brain and spinal cord injuries. USSCs were administered to premature rabbit pups with IVH and their effects on white matter integrity and neurobehavioral performance were evaluated. USSCs were injected either via intracerebroventricular (ICV) or via intravenous (IV) routes in 3 days premature (term 32d) rabbit pups, 24 hours after glycerol-induced IVH. The pups were sacrificed at postnatal days 3, 7, and 14 and effects were compared to glycerol-treated but unaffected or nontreated control. Using in vivo live bioluminescence imaging and immunohistochemical analysis, injected cells were found in the injured parenchyma on day 3 when using the IV route compared to ICV where cells were found adjacent to the ventricle wall forming aggregates; we did not observe any adverse events from either route of administration. The injected USSCs were functionally associated with attenuated microglial infiltration, less apoptotic cell death, fewer reactive astrocytes, and diminished levels of key inflammatory cytokines (TNFα and IL1β). In addition, we observed better preservation of myelin fibers, increased myelin gene expression, and altered reactive astrocyte distribution in treated animals, and this was associated with improved locomotor function. Overall, our findings support the possibility that USSCs exert anti-inflammatory effects in the injured brain mitigating many detrimental consequences associated with IVH. Stem Cells Translational Medicine 2019.
- Diagnostic Performance of Glymphatic System Evaluation Using Diffusion Tensor Imaging in Idiopathic Normal Pressure Hydrocephalus and Mimickers. [Journal Article]
- CGCurr Gerontol Geriatr Res 2019; 2019:5675014
- CONCLUSIONS: Atlas-based ALPS index using the DTI method differentiated among iNPH, piNPH, and controls clearly.
- Development and evaluation of a craniocerebral model with tactile-realistic feature and intracranial pressure for neurosurgical training. [Journal Article]
- JNJ Neurointerv Surg 2019 Jul 18
- CONCLUSIONS: The neurosurgical training model can provide trainee surgeons with realistic experience in both tactile feedbacks and craniocerebral anatomy, improving their surgical skills.
- Comparison of CSF and MRI Findings among Neonates and Infants with E coli or Group B Streptococcal Meningitis. [Journal Article]
- AAAJNR Am J Neuroradiol 2019 Jul 18
- 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.
- Pineal region tumors: Long-term results of endoscopic third ventriculostomy and concurrent tumor biopsy with a single entry approach in a series of 64 cases. [Journal Article]
- CNClin Neurol Neurosurg 2019 Jul 08; 184:105418
- CONCLUSIONS: The results of this study support the safety and efficacy of endoscopic third ventriculostomy and concurrent biopsy of the pineal region tumors as a less invasive surgical method associated with low morbidity and mortality rate. Our data demonstrated how simultaneous endoscopic third ventriculostomy and biopsy of the pineal region tumors with a single entry approach can produce favorable results.
- [Mothers' emotional states and characteristics of early interaction with their prematurely born babies]. [Journal Article]
- ZNZh Nevrol Psikhiatr Im S S Korsakova 2019; 119(5):148-153
- Advanced technologies of neonatal reanimation and intensive care have led to the increase in the number of children with the extremely low and low birth weight. These children constitute a risk group…
Advanced technologies of neonatal reanimation and intensive care have led to the increase in the number of children with the extremely low and low birth weight. These children constitute a risk group often having severe development complications and disabilities. This article provides a review of the existing research literature on emotional states of mothers of prematurely born infants' and characteristics of their early interaction. The data on the influence of the mother's emotional state on her interaction with her prematurely born infant and its impact on the child's long-term development are presented. The authors present directions of studying development of prematurely born infants, which are not fully reflected in the literature, regarding infants with structural intracranial changes (intraventricular hemorrhages) and associated hydrocephalus and posthemorrhagic hydrocephaly. New targets of practical work have been developed to improve the family-centered interdisciplinary support for children at infancy and early age.
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- Spinal paraganglioma presenting as normal pressure hydrocephalus: a rare occurrence. [Journal Article]
- BJBr J Neurosurg 2019 Jul 18; :1-3
- CONCLUSIONS: The presence of an intraspinal tumor must be kept in mind as a possible cause of the hydrocephalus.