Purpose Fluid‐attenuated inversion recovery (FLAIR) nulls the CSF signal and is widely used in neuro MRI exams. A 3D scan can provide high SNR, contiguous coverage, and reduced sensitivity to through‐plane CSF flow. In this work, a 3D spiral FLAIR technique is proposed to improve the image quality of conventional 3D Cartesian FLAIR. Methods The 3D spiral FLAIR sequence incorporated a spiral‐in/out readout to preserve higher scan efficiency and eliminate off resonance‐induced artifacts observed with a commonly implemented spiral‐out readout, a compensation approach to minimize phase errors due to the concomitant fields accompanying the spiral gradient, and an adapted variable flip angle scheme to preserve scan efficiency and maintain a long and stable echo train. 3D Cartesian and spiral FLAIR (~6 min each) were acquired on a 3 Tesla scanner from 6 subjects (age range: 31‐64 years; mean: 39.5). Two neuroradiologists rated the images in a blinded fashion on a 5‐point scale. The noise performance was assessed quantitatively. Results Compared to 3D Cartesian FLAIR, 3D spiral FLAIR exhibits greater reduction of artifacts from CSF, especially anterior to the brain stem (rated better in 4 cases), artifacts attributed to blood/flow in the deep brain (better or much better in all 6 cases), and superior overall image quality (much better in 5 cases) despite residual susceptibility artifacts near the nasal cavity. Quantitative assessment demonstrates ~1.5× higher average SNR than Cartesian data. Conclusion 3D spiral FLAIR achieves higher SNR, reduced CSF, and blood/flow artifacts, providing an alternative to 3D Cartesian FLAIR for neurological exams.
Ankyrin repeat domain 17 (ANKRD17) is postulated to play a role in the integrity of blood vessels and has been reported to be associated with developmental delays, epilepsy, and growth restriction. Whereas ANKRD17-deficient mice have been demonstrated to experience catastrophic hemorrhages, vascular malformations have not been reported in human patients with pathogenic variants to ANKRD17. We report a term male neonate with a heterozygous de novo variant to ANKRD17 (ANKRD17; c6988 C > G, P.[P2330a]) who experienced subarachnoid hemorrhage from a ruptured aneurysm involving the left middle cerebral artery. He experienced acute symptomatic seizures and required clipping of his aneurysm at 35 days of life, later progressing to developing multifocal drug-resistant epilepsy. To our knowledge, this case represents the first report of a cerebrovascular malformation from a patient with ANKRD17. Further work is needed to investigate whether pathogenic variants to ANKRD17 can lead to cerebral aneurysms or other cerebrovascular malformations in children.
All work was performed at the Barrow Neurological Institute at Phoenix Children's Hospital.Objective: Investigate injury severity, neuroimaging, physiology, and outcomes with bolus hyperosmolar therapy (HT) of 3% hypertonic saline or mannitol.Methods: Retrospective cohort analysis was performed. Physiologic variables included intracranial pressure (ICP), arterial blood pressure (ABP), and heart rate (HR). Volume-pressure compensation (PVC) indices included ICP pulse amplitude (AMP) and correlation of AMP and ICP (RAP). Cerebrovascular pressure reactivity (CVPR) indices included pressure reactivity index (PRx), pulse amplitude index (PAx), wavelet PRx (wPRx), and correlation of AMP and cerebral perfusion pressure (RAC). Heart rate variability (HRV) indices included heart rate standard deviation (HRsd), heart rate root mean square of successive differences (HRrmssd) and low-high frequency ratio (LHF). Outcome was assessed using Glasgow Outcomes Scale Extended Pediatrics, 12-months post-injury. Generalized estimating equations was applied to investigate associations of physiologic changes and pre-treatment indices with HT efficacy. Repeated measures analysis of variance was applied to investigate changes after HT without intracranial hypertension (ICH). Wilcoxon rank-sum was applied to investigate HT responsiveness with age, injury severity, neuroimaging, and outcomes.Results: Thirty children received bolus HT. ICH reduction after HT was associated with reduced ICP (p = 0.0064), ABP (p = 0.0126), PRx (p = 0.0063), increased HRsd (p = 0.0408), and decreased pretreatment RAC (p = 0.0115) and wPRx (p = 0.0072). HT-responsive patients were older and had improved outcomes (p = 0.0394). HT without ICH was associated with increased ICP (P < 0.0001) and ABP (P < 0.0001), increases in all HRV indices and decreases in all PVC indices.Conclusion: After pediatric TBI, efficacious HT is associated with decreased ICP and ABP, pre-treatment indices suggesting efficient CVPR, and potentially improved outcomes.
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