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the gut microbiota composition in both genders, but the association with reductions in local and systemic inflammation, oxidative stress, and improvement of metabolic homeostasis only occurs in male mice.Neurofibromatosis type 1 (NF1) is a genetic disorder that affects a range of tissue systems, however the associated muscle weakness and fatigability can have a profound impact on quality of life. Prior studies using the limb-specific Nf1 knockout mouse (Nf1Prx1-/-) revealed an accumulation of intramyocellular lipid (IMCL) that could be rescued by a diet supplemented with L-carnitine and enriched for medium-chain fatty acids (MCFAs). In this study we used the Nf1Prx1-/- mouse to model a range of dietary interventions designed to reduce IMCL accumulation, and analyze using other modalities including in situ muscle physiology and lipid mass spectrometry. Histological IMCL accumulation was significantly reduced by a range of treatments including L-carnitine and high MCFAs alone. A low-fat diet did not affect IMCL, but did provide improvements to muscle strength. Empesertib Supplementation yielded rapid improvements in IMCL within 4 weeks, but were lost once treatment was discontinued. In situ muscle measurements were highly variable in Nf1Prx1-/- mice, attributable to the severe phenotype present in this model, with fusion of the hips and an overall small hind limb muscle size. Lipidome analysis enabled segregation of the normal and modified chow diets, and fatty acid data suggested increased muscle lipolysis with the intervention. Acylcarnitines were also affected, suggestive of a mitochondrial fatty acid oxidation disorder. These data support the theory that NF1 is a lipid storage disease that can be treated by dietary intervention, and encourages future human trials.

The aim of this retrospective study is to verify whether preoperative systemic inflammatory markers (serum C-reactive protein [CRP] and neutrophil-lymphocyte ratio [NLR]) can help in predicting the disease-specific survival (DSS) and local recurrence (LR) rate in adult patients affected by localized myxofibrosarcoma (MFS) of the extremities.

We reviewed 126 adult patients with primary, localized MFS of the limbs. We analyzed DSS and LR.

Median age at the time of surgery was 68 years (range 19-92). Median CRP was 0.4 mg/dL and median NLR was 2.8. A worse DSS was found in patients who had preoperative CRP >0.5 mg/dL (p = 0.002) and in those with NLR >3.5 (p < 0.001). In multivariate analysis, tumor size and grade as well as preoperative CRP values and NLR were confirmed to be prognostic factors in terms of DSS. An increased risk of LR was found in multivariate analysis in patients with a tail sign and with high gadolinium enhancement at preoperative MRI.

Patients with high preoperative CRP and NLR levels, as well as large and high-grade tumors, might be considered as candidates for additional, more aggressive treatment approaches or more stringent follow-up schedules.

Patients with high preoperative CRP and NLR levels, as well as large and high-grade tumors, might be considered as candidates for additional, more aggressive treatment approaches or more stringent follow-up schedules.In human preterm newborns, caffeine increases brain activity and improves neurodevelopmental outcomes. In animal models of hypoxic ischemic brain injury, caffeine pretreatment reduces infarct volume. We studied the relationship between tissue neuroprotection and brain activity after injury to further understand caffeine neuroprotection. Rat dams received caffeine prior to birth or on postnatal day 3 (P3) through P16. Caffeine-treated and -untreated pups underwent the Vannucci procedure (unilateral carotid ligation, global hypoxia) on P2. A subset had EEG recordings. Brain hemispheric infarct volume was measured on P16. P2 hypoxic ischemia (HI) results in histologic brain injury (mean ± standard deviation infarct volume 10.3 ± 4.6%) and transient suppression of EEG activity. Caffeine pretreatment reduces brain injury (mean ± standard deviation infarct volume 1.6 ± 4.5%, p less then 0.001) and improves amplitude-integrated EEG (aEEG) and EEG burst duration and amplitude. Caffeine treatment after HI does not reduce infarct volume (mean ± standard deviation 8.3 ± 4.1%, p = 1.0). However, caffeine posttreatment was equally effective at restoring aEEG amplitude and EEG burst duration and amplitude. Thus, caffeine supports brain background electrical activity independent of tissue neuroprotection.

Glossopharyngeal neuralgia (GPN) is a rare facial pain syndrome with debilitating symptoms. For medication-resistant GPN, stereotactic radiosurgery (SRS) is an emerging treatment option with a promising role; however, recurrence rates after SRS are fairly high. We present a patient who underwent repeat SRS for recurrent GPN and subsequently maintained over 3 years of complete pain relief. For the first time, we present a systematic review of repeat SRS for recurrent GPN.

Twelve cases of repeat SRS for GPN have previously been reported in the literature (13 studies including ours). Among patients with follow-up, initial pain relief was achieved in 83% (n = 10) of cases a median of 5 weeks after repeat SRS; 2 patients failed to obtain any pain relief. A favorable pain response (BNI I-IIIb) was achieved in 67 and 58% of cases at 6 and 12 months, respectively. All 13 were targeted to the glossopharyngeal meatus. Three patients (23%) experienced adverse radiation effects. Five patients (50%) experienced recurrence a median of 14 months after repeat SRS. Two patients (17%) required additional surgical intervention. At the final follow-up, 75% (n = 9) of the patients had a favorable pain outcome. Key Messages Repeat SRS may be a viable alternative to open surgery for the treatment of recurrent GPN, albeit with an increased risk of adverse radiation effects. Though limited by a small cohort of patients, the best predictors of an effective second treatment may be a response to initial SRS for >5 months, a maximum dose >75 Gy, and a target at the glossopharyngeal meatus. Larger prospective studies are needed to better define its role.

75 Gy, and a target at the glossopharyngeal meatus. Larger prospective studies are needed to better define its role.

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