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Child's Nervous System : ChNS :... Jul 2024Cervicothoracic ventral-dorsal rhizotomy (VDR) is a potential treatment of medically refractory hypertonia in patients who are not candidates for intrathecal baclofen,...
PURPOSE
Cervicothoracic ventral-dorsal rhizotomy (VDR) is a potential treatment of medically refractory hypertonia in patients who are not candidates for intrathecal baclofen, particularly in cases of severe upper limb hypertonia with limited to no function. A longitudinal cohort was identified to highlight our institutional safety and efficacy using cervicothoracic VDR for the treatment of hypertonia.
METHODS
Retrospective data analysis was performed for patients that underwent non-selective cervicothoracic VDR between 2022 and 2023. Non-modifiable risk factors, clinical variables, and operative characteristics were collected.
RESULTS
Six patients (three female) were included. Four patients underwent a bilateral C6-T1 VDR, one patient underwent a left C7-T1 VDR, and another underwent a left C6-T1 VDR. Three patients had quadriplegic mixed hypertonia, one patient had quadriplegic spasticity, one patient had triplegic mixed hypertonia, and one patient had mixed hemiplegic hypertonia. The mean difference of proximal upper extremity modified Ashworth scale (mAS) was - 1.4 ± 0.55 (p = 0.002), and - 2.2 ± 0.45 (p < 0.001) for the distal upper extremity. Both patients with independence noted quality of life improvements as well as increased ease with dressing and orthotics fits. Caregivers for the remaining four patients noted improvements in caregiving provision, mainly in dressing, orthotics fit, and ease when transferring.
CONCLUSION
Cervicothoracic VDR is safe and provides tone control and quality of life improvements in short-term follow-up. It can be considered for the treatment of refractory hypertonia. Larger multicenter studies with longer follow-up are necessary to further determine safety along with long-term functional benefits in these patients.
PubMed: 38958730
DOI: 10.1007/s00381-024-06479-5 -
The Journal of Bone and Joint Surgery.... Jul 2024
Expanding Approaches to Improve Orthopaedic Care Through the Application of Artificial Intelligence: Commentary on an article by Neil P. Sheth, MD, et al.: "Effects of Abaloparatide on Bone Mineral Density in Proximal Femoral Regions Corresponding to Arthroplasty Gruen Zones. A Study of...
Topics: Humans; Female; Bone Density; Artificial Intelligence; Bone Density Conservation Agents; Osteoporosis, Postmenopausal; Femur; Parathyroid Hormone-Related Protein
PubMed: 38958661
DOI: 10.2106/JBJS.24.00416 -
The International Journal of Eating... Jul 2024Understanding the emotional context of feeding behavior may help identify causal mechanisms of food avoidance among individuals with anorexia nervosa. Although...
BACKGROUND
Understanding the emotional context of feeding behavior may help identify causal mechanisms of food avoidance among individuals with anorexia nervosa. Although predominant food avoidance models assume fear of fat drives feeding behavior, disgust may be more theoretically and proximally relevant to moment-to-moment experiences of feeding. This study, therefore, aimed to examine affect and food avoidance using automated affect analysis from facial response by measuring time-specific transitions in disgust during a laboratory eating paradigm. We hypothesized that phase transitions in disgust would distinguish temporally self-initiated eating from food avoidance.
METHODS
Sixty-three adolescents with anorexia nervosa or another low-weight eating disorder (LWED) and 27 age- and sex-matched controls were recruited as part of a larger study; 45 patients and 22 controls provided data on autonomous eating and facial affect during a laboratory meal. Dynamic structural equation models quantified moment-to-moment relationships between disgust and feeding behavior.
RESULTS
Self-initiated eating was associated with greater increases in disgust, but not fear, intensity among those with LWED relative to control participants and greater disgust intensity predicted lower likelihood of self-initiated eating.
DISCUSSION
Phasic transitions in disgust provide moment-to-moment evidence of affective influence on self-initiated eating and lend credibility to the hypothesis that disgust contributes to food avoidance and initiation in individuals with LWED.
PubMed: 38958505
DOI: 10.1002/eat.24254 -
Muscle & Nerve Jul 2024Not all patients with chronic inflammatory demyelinating polyneuropathy (CIDP) have evidence of demyelination on nerve conduction studies (NCS). Patients with...
Treatment response in patients with clinical and supportive laboratory features of chronic inflammatory demyelinating polyneuropathy without demyelinative findings on nerve conduction studies: A retrospective study.
INTRODUCTION/AIMS
Not all patients with chronic inflammatory demyelinating polyneuropathy (CIDP) have evidence of demyelination on nerve conduction studies (NCS). Patients with "supportive" evidence of CIDP on cerebrospinal fluid (CSF), magnetic resonance imaging (MRI), ultrasound (US), or nerve biopsy but not on NCS, often receive immunomodulating therapy. We evaluated the treatment response of patients with clinical and supportive features of CIDP lacking NCS evidence of demyelination.
METHODS
Retrospective chart review was conducted on 232 patients who met CIDP clinical criteria and were treated with disease-modifying therapy. Patients included did not have NCS criteria of demyelination, but did have supportive CSF, MRI, or US findings consistent with CIDP. A positive treatment response was defined as at least a one-point improvement in the modified Rankin scale (mRS), or a four-point increase in the Medical Research Council sum score (MRCSS).
RESULTS
Twenty patients met criteria: 17 of the 18 (94%) patients with CSF protein >45 mg/dL, 6 of the 14 (43%) with MRI lumbosacral root or plexus enhancement, and 4 of the 6 (67%) with enlarged proximal nerves on US. Eighteen patients received intravenous immunoglobulin, 10 corticosteroids, one plasma exchange, and six other immunomodulatory therapies. Twelve patients had a positive treatment response on the MRCSS or mRS. The presence of MRI lumbosacral root or plexus enhancement was associated with a positive treatment response.
DISCUSSION
A trial of immunomodulating treatment should be considered for patients with clinical features of CIDP in the absence of NCS evidence of demyelination, particularly when there is MRI lumbosacral root or plexus enhancement.
PubMed: 38958279
DOI: 10.1002/mus.28198 -
American Journal of Physical Medicine &... Jul 2024This feasibility study tested the capability of high frequency stimulation (HFS) to block muscle contractions elicited by electrical stimulation of the same nerve...
This feasibility study tested the capability of high frequency stimulation (HFS) to block muscle contractions elicited by electrical stimulation of the same nerve proximally. During a tendon lengthening surgery in the forearm, the anterior interosseous nerve (AIN) was exposed. A specialized nerve cuff electrode was placed around the nerve, and a stimulating probe held on the nerve 1 cm proximal to the cuff electrode delivered pulses of current causing the pronator quadratus muscle to contract. Through the cuff electrode, 20 kHz HFS was delivered to the nerve for 10 seconds during proximal stimulation. HFS amplitudes between 5 and 10 mA peak-to-peak were tested to determine which produced complete and partial block of the electrically induced contractions. The minimum HFS amplitude that produced complete block was 8 mA, with lower amplitudes producing partial block. In all trials, muscle contractions resumed immediately after HFS was turned off. This demonstration of high frequency electrical nerve block is a milestone in the road to clinical implementation of HFS mediated motor block for spasticity.
PubMed: 38958190
DOI: 10.1097/PHM.0000000000002577 -
Journal of Orthopaedics Nov 2024Genu recurvatum deformity after treatment of leg-length discrepancy (LLD) with tension-band plating is a recognized, but poorly described phenomenon in medical...
AIMS AND OBJECTIVES
Genu recurvatum deformity after treatment of leg-length discrepancy (LLD) with tension-band plating is a recognized, but poorly described phenomenon in medical literature. The aim of this study was to evaluate clinical and radiological features of patients treated with tension-band plating for LLD assessing the development of a recurvatum deformity and its relationship to plate and screw disposition in a transversal plane, thus attempting to establish optimal plate positioning.
MATERIALS AND METHODS
Retrospective study of children with LLD treated with tension-band plating. Primary endpoints were clinical and radiological knee recurvatum and anterior and posterior physeal areas measured drawing a line spanning from the lateral to the medial tension-band plates in the transverse plane using volumetric magnetic resonance imaging (vMRI). These findings were compared between patients with and without knee recurvatum.
RESULTS
Twelve children (mean age 11.7 years) were included. Average follow-up was 2.6 years (1.5-5.0). Tension-band plating led to a significant reduction in LLD (mean, 15 mm). Six patients (50 %) developed clinical genu recurvatum (mean, 22°). According to vMRI, patients with genu recurvatum had a larger posterior to anterior physeal area ratio in both distal femur (1.6 versus 0.9, < 0.05) and proximal tibial physes (2.2 versus 1.0, < 0.05).
CONCLUSION
The optimal position of the tension-band plates in distal femoral and proximal tibial physes should be in a point where a posterior to anterior physeal areas ratio is around 1.0, so as to achieve an even distribution of the physeal areas in the multidimensional physeal transverse plane. This point anatomically corresponds in the sagittal X-ray view to an imaginary line located just anterior to the posterior diaphyseal cortical bone on a true lateral radiograph for both femur and tibia.
PubMed: 38957798
DOI: 10.1016/j.jor.2024.06.004 -
Clinical Nephrology. Case Studies 2024We investigated the pathogenesis of a perihilar variant of focal segmental glomerulosclerosis detected by kidney biopsy in a 16-year-old male. The disease was refractory...
Renal and cardiac biopsy findings in an adolescent patient with the 3243A>G mitochondrial DNA mutation: Favorable renal prognosis post renal transplantation from the mother.
We investigated the pathogenesis of a perihilar variant of focal segmental glomerulosclerosis detected by kidney biopsy in a 16-year-old male. The disease was refractory to steroid therapy, and at the second kidney biopsy, abnormal mitochondrial proliferation was newly observed in the podocytes. The patient also developed late-onset hearing loss and had a family history of diabetes, and genetic testing confirmed the mitochondrial DNA mutation 3243A>G (48%). Eight months after hemodialysis was started, encephalopathy occurred presumably due to rapid dehydration. After changing dialysis into continuous ambulatory peritoneal dialysis, encephalopathy was resolved, but the patient developed myocardial hypertrophy, probably because of the myocardial overreaction to congestion. A myocardial biopsy showed mitochondrial proliferation in the myocardium. After renal transplantation from his mother with a heteroplasmy of 4%, the cardiomyopathy improved, and the renal function has remained stable for 4 years. We speculated that the abnormal mitochondrial morphology in the kidney and heart may be characteristic of mitochondrial genetic disease, and renal transplantation from the mother with a low heteroplasmy was considered desirable for mitochondrial nephropathy with poor prognosis.
PubMed: 38957708
DOI: 10.5414/CNCS111422 -
Annals of Gastroenterological Surgery Jul 2024Malnutrition, characterized by altered body composition and impaired function, is particularly prevalent among gastric cancer patients, affecting up to 60% of them.... (Review)
Review
Malnutrition, characterized by altered body composition and impaired function, is particularly prevalent among gastric cancer patients, affecting up to 60% of them. Malnutrition in these patients can manifest both before and after surgery, due to factors such as gastric outlet obstruction, cancer cachexia, and anatomical changes. Notably, total gastrectomy (TG) presents the most significant nutritional challenges. However, function-preserving gastrectomy, such as pylorus-preserving gastrectomy (PPG) and proximal gastrectomy (PG), have shown promise in improving nutritional outcomes. Effective nutritional risk screening and assessment are vital for identifying patients at risk. Nutritional support not only improves nutritional parameters but also reduces complications, enhances quality of life (QoL) and survival rates. Those unable to maintain more than 50% of the recommended intake for over 7 days are recommended for nutritional support. Common methods of nutritional support include oral nutrition supplements (ONS), enteral nutrition (EN), or parenteral nutrition (PN) depending on the patient's status. Effect of perioperative nutritional support remains controversial. Preoperative interventions including ONS and PN have shown mixed results, with selective benefits in patients with sarcopenia or hypoalbuminaemia, while impact of EN in gastric outlet obstruction patients have been positive. In contrast postoperative support appears to be consistent. Tube feeding after TG has shown improvements, and ONS have been effective in reducing weight loss and improving nutritional biomarkers. PN was also associated with benefits such as weight maintenance and QoL. This review explores the mechanisms, assessment, and clinical impact of malnutrition, emphasizing the importance of nutritional support in gastric cancer patients undergoing gastrectomy.
PubMed: 38957563
DOI: 10.1002/ags3.12788 -
ARP Rheumatology 2024Transient osteoporosis of the hip (TOH) is an important but often neglected cause of hip pain, which can gradually lead to debilitating mobility and carries risks such...
Transient osteoporosis of the hip (TOH) is an important but often neglected cause of hip pain, which can gradually lead to debilitating mobility and carries risks such as fracture or avascular necrosis. A 39-year-old woman presented to the Rheumatology department two weeks post-cesarean delivery, reporting the onset of left mechanical hip pain since the 33rd week of pregnancy. After delivery, similar complaints emerged on the right side. Hip X-ray showed a decrease in bone density in the left hip. Later, Magnetic Resonance Imaging revealed bilateral bone marrow edema in both proximal femurs. The diagnosis of TOH was established, and the patient was treated with conservative measures. Seven months later, she was asymptomatic. Pregnancy is a recognized risk factor for TOH, especially in the last trimester. It is an important differential diagnosis to consider in cases of hip pain in pregnant or newly breastfeeding women.
Topics: Humans; Female; Pregnancy; Adult; Pregnancy Complications; Osteoporosis; Hip Joint; Arthralgia; Magnetic Resonance Imaging
PubMed: 38956998
DOI: No ID Found -
Scientific Reports Jul 2024Rapamycin slows cystogenesis in murine models of polycystic kidney disease (PKD) but failed in clinical trials, potentially due to insufficient drug dosing. To improve...
Rapamycin slows cystogenesis in murine models of polycystic kidney disease (PKD) but failed in clinical trials, potentially due to insufficient drug dosing. To improve drug efficiency without increasing dose, kidney-specific drug delivery may be used. Mesoscale nanoparticles (MNP) selectively target the proximal tubules in rodents. We explored whether MNPs can target cystic kidney tubules and whether rapamycin-encapsulated-MNPs (RapaMNPs) can slow cyst growth in Pkd1 knockout (KO) mice. MNP was intravenously administered in adult Pkd1KO mice. Serum and organs were harvested after 8, 24, 48 or 72 h to measure MNP localization, mTOR levels, and rapamycin concentration. Pkd1KO mice were then injected bi-weekly for 6 weeks with RapaMNP, rapamycin, or vehicle to determine drug efficacy on kidney cyst growth. Single MNP injections lead to kidney-preferential accumulation over other organs, specifically in tubules and cysts. Likewise, one RapaMNP injection resulted in higher drug delivery to the kidney compared to the liver, and displayed sustained mTOR inhibition. Bi-weekly injections with RapaMNP, rapamycin or vehicle for 6 weeks resulted in inconsistent mTOR inhibition and little change in cyst index, however. MNPs serve as an effective short-term, kidney-specific delivery system, but long-term RapaMNP failed to slow cyst progression in Pkd1KO mice.
Topics: Animals; Sirolimus; Mice; Polycystic Kidney Diseases; Mice, Knockout; Nanoparticles; Disease Models, Animal; TOR Serine-Threonine Kinases; TRPP Cation Channels; Kidney; Drug Delivery Systems; Male
PubMed: 38956234
DOI: 10.1038/s41598-024-65830-7