Timeline of diagnosed pain causes in children with severe neurological impairment.

Objective: Pain's causes in children with severe cognitive impairment may be challenging to diagnose. This study aimed to investigate if there is a relationship between pain causes and the age of children. Methods: We conducted a multicenter retrospective study in three Italian Pediatric Units. Eligible subjects were patients from 1 to 18 years with severe neurological impairment. We collected data regarding diagnoses, pain causes and medical or surgical procedures. The timing of pain episodes was categorized into age-related periods: infants and toddlers (0-24 months), preschool children (3-5 years), schoolchildren (6-12 years), and adolescents (13-17 years). Results: Eighty children with severe neurological impairment were enrolled. The mean age was 11 years (±5.8). Gastroenterological pain was most common in the first years of life (p = 0.004), while orthopaedic and tooth pain was the most typical in schoolchildren and adolescents (p = 0.001 and p = 0.02). Concerning surgical procedures, PEG placement and gastric fundoplication were significantly more common in the first 5 years of age (p = 0.03), and heart surgery was typical of infants (p = 0.04). Orthopaedic surgery was more commonly reported in older children and adolescents (p < 0.001). Conclusions: Some causes of pain are more frequent in children with severe neurological impairment in defined age-related periods. Specific age-related pain frequencies may help physicians in the diagnostic approach.

Cat Scratch Endocarditis.

We reported a case of blood culture-negative infective endocarditis on a native valve, where the clinical presentation was exclusively related to extensive cerebral ischemia secondary to multiple systemic septic cardioembolic events. The cause was ascribed to subacute Bartonella henselae infection, presumably transmitted by cat scratch, documented by positive serologic findings.

Association between intermittent hypoxemia and neurodevelopmental outcomes in extremely premature infants: A single-center experience.

OBJECTIVE: To describe the association between intermittent hypoxemic events (IHEs) and severe neurodevelopmental impairment (SNDI) or death in extremely premature infants. STUDY DESIGN: Retrospective study of extremely premature infants 230/7-276/7 weeks gestational age (GA) and birthweight (BW) ≤1250 grams (g) admitted to a level IV neonatal intensive care unit (NICU) from 2013 to 2017. IHEs, defined as events with SpO2 ≤ 80 % lasting 10 s to 5 min, were algorithmically identified using data extracted from bedside monitors at 2 s intervals (0.5 Hz). The primary outcome was SNDI at 18-24 months corrected age (CA), defined as a Bayley-III motor, language or cognitive composite score ≤69, or death before discharge while the secondary outcome was SNDI alone. We used mixed-effects regression models to evaluate the relationship between mean daily IHE rate per postnatal week of life for the first 12 weeks and the outcomes, and logistic regression models to assess the association between outcomes and summary measures of hypoxic burden for the entire NICU hospitalization. RESULTS: The mortality rate was 7 % (18/249) during NICU hospitalization. Of 249 infants born during this time period, IHE and neurodevelopmental outcome data were fully available for 65 infants (mean GA 26 ± 1.4 weeks, mean birth weight (BW) 738 ± 199 g. The outcome of SNDI alone occurred in 34 % (22/65) with a majority demonstrating motor or language delay on the Bayley-III. Although mean daily IHE rate/week was not associated with SNDI or death, total IHE duration was associated with increased odds of SNDI (OR (95 % CI) 1.03 (1.01, 1.05), p = 0.008) in models adjusted for GA. CONCLUSIONS: In a cohort of extremely premature infants 23-27 weeks GA, each hour of total IHE duration (SpO2 ≤ 80 %) was associated with a 2.7 % (0.7 %, 4.8 %) increase in the odds of SNDI at 18-24 months CA.

Paediatric Palliative Care in a Reference Centre of Inherited Metabolic Diseases.

INTRODUCTION: Paediatric palliative care (PPC) has a significant role in improving the quality of life of children with life-limiting or life-threatening illnesses, diminishing symptom burden, and providing holistic support to patients and families. Inherited metabolic diseases (IMD) are a group of heterogeneous diseases that often present with severe neurologic impairment, needing lifelong care and challenging symptom management. OBJECTIVE: Our aim was to characterize the cohort of patients with IMD followed by the paediatric palliative care team (PPCT) and to describe the provision of care provided. METHODS: The descriptive analysis of demographic, clinical, and care delivery data of a cohort of paediatric patients was carried out with a confirmed diagnosis of IMD, followed in a Reference Centre, in the care of PPCT between 2018 and 2023. RESULTS: Thirteen (10%) of a total of 134 patients in the care of PPCT had a confirmed diagnosis of an IMD: 6 mitochondrial, 3 peroxisomal, 3 lysosomal, and 1 pterin metabolism disorder. The median age at referral was 9 years (0-18), the median duration of care was 2 years [2-4], median number of home visits in the last year was 2 [1-4], and median number of outpatient consults was 4 [2 -8]. Twelve patients (92%) had no autonomy in their activities of daily living. Neurologic (100%), gastrointestinal (92%), and respiratory (69%) symptoms were the main focus of care. All patients were polymedicated (5 or more different drugs). Nine (69%) had percutaneous gastrostomy and 2 (15%) had noninvasive ventilation. Median hospital admissions before and after starting care by PPCT were 4 and 1. Moreover, three patients died and one was at home. CONCLUSION: Mitochondrial, lysosomal, and peroxisomal disorders are complex multisystemic diseases that very often have no treatment intended to cure. These patients have a heavy symptom burden and frequent intercurrences. Addressing these symptoms is challenging, but PPC has proven to reduce hospital admissions with consequent improvement in quality of life. In the future, PPC should be available for all children and families with life-threatening conditions.

Open Surgical Treatments of Osteoporotic Vertebral Compression Fractures.

With an aging population, the osteoporotic vertebral compression fracture (OVCF) has become a constant concern for its physical and neurological complications, such as spinal kyphosis and refractory pains. Compared with traditional conservative treatments, the open surgery is more superior in some ways because of its direct decompression and correction. Various operation methods applying to different indications have been developed to deal with different fracture situations, including anterior, posterior, and combined surgery. In this review, we have concluded the latest developments of the surgery treating OVCF and the internal fixation as references for spinal surgeons of the choice of suitable treatments.

Study Confirms Safety and Effectiveness of Intra-Articular Glucocorticoids for Painful Hip Dislocation in Children and Young Adults with Neurologic Impairment.

BACKGROUND: Hip dislocation is a common source of pain in children with neurologic impairment. When medical interventions fail, orthopedic surgery does not guarantee a definitive result as the displacement may continue postoperatively and a second operation is often required. METHODS: Retrospective analysis of data regarding the safety and effectiveness of an intra-articular corticosteroid injection (IACI) in 11 patients, aged 15 ± 5 years old, collected through a telephonic questionnaire administered to parents. RESULTS: 21 IACIs were performed, a mean number of 1.9 ± 1.5 times for each patient, at a mean age (of the first IACI) of 13.5 ± 5 years. According to the parents, the IACI significantly lowered the number of participants experiencing pain (82% reduction) and using analgesics (60% reduction). There was also a significant improvement in the children's hip mobility (63% reduction in patients experiencing stiffness), decubitus (90% reduction in obligated positioning), behavior (80% reduction in lamenting or crying patients), sleep quality (87.5% reduction in patients awakening every night), and caregivers' quality of life (91% reduction in worried parents). The mean reported duration of the IACIs' benefit was 5.4 ± 2.4 months (range 1-9), with a positive correlation with the number of IACIs (r = 0.48; p-value = 0.04) and a negative correlation with the age at the first injection (r = -0.71; p-value = 0.02). The only reported adverse event was mild local swelling in one child. CONCLUSIONS: the IACI could represent a safe and effective intervention for painful hip dislocation, both before and after surgery, with a long-lasting benefit which seems to increase as multiple IACIs are performed.

Prophylactic antireflux procedures are not necessary in neurologically impaired children undergoing gastrostomy placement.

PURPOSE: Fundoplication is frequently used in children with neurologic impairment even in the absence of reflux due to concerns for future gastric feeding intolerance, but supporting data are lacking. We aimed to determine the incidence of secondary antireflux procedures (fundoplication or gastrojejunostomy (GJ)) post gastrostomy tube (GT) placement in children with and without neurologic impairment. METHODS: Children under 18 undergoing a GT placement without fundoplication between 2010 and 2020 were identified utilizing the PearlDiver Mariner national patient claims database. Children with a diagnosis of cerebral palsy or a degenerative neurologic disease were identified and compared to children without these diagnoses. The incidence of delayed fundoplication or conversion to GJ were compared utilizing Kaplan-Meier and Cox proportional hazards regression analyses. RESULTS: A total of 14,965 children underwent GT placement, of which 3712 (24.8%) had a diagnosis of neurologic impairment. The rate of concomitant fundoplication was significantly higher among children with a diagnosis of neurologic impairment as compared to those without (9.3% vs 6.4%, p < 0.001). While children with neurologic impairment had a significantly higher rate of fundoplication or GJ conversion at 5 years compared to children without (12.6% [95% confidence interval (CI): 11.4%-13.8%] vs 8.6% [95% CI 8.0%-9.2%], p < 0.001), the overall incidence remained low. CONCLUSION: Although children with neurologic impairment have a higher rate of requiring an antireflux procedure or GJ conversion than other children, the overall rate remains less than 15%. Fundoplication should not be utilized in children without clinical reflux on the basis of neurologic impairment alone.

Pediatric neuropalliative care.

Pediatric palliative care seeks to support quality of life for children and families affected by serious illness. Children with neurological disease are among the most frequent recipients of pediatric palliative care. Several important elements distinguish pediatric palliative care from adult practice, including a longer illness duration, longitudinal relationships over the span of years, diseases characterized by chronic fragility rather than progressive pathology, and the reliance on parents as proxy decision makers. This chapter will provide an overview of pediatric neuropalliative care, with emphasis on the types of disease trajectories, symptom management, and communication principles for supporting shared decision making with families. The role of neurology expertise is highlighted throughout, with special attention toward incorporating palliative care into pediatric neurology practice.

Role of gabapentin in reducing the need for high-risk medications in patients with stable severe neurological impairment.

Objective: The study was aimed at assessing the prevalence of pain behaviors in children with severe neurological impairment (SNI), as well as the use of prescribed pain behavior medications, and the effects of gabapentin initiation on behaviors and use. Methods: A pre-post study was conducted on data from 11 patients with SNI who received gabapentin at a children's hospital in Canada. Symptoms and the use of high-risk pain behavior medications were assessed before and after gabapentin initiation and titration. Results: Pain was identified as a primary concern in most patients (8/11 [73%]) before gabapentin initiation. Dystonia was the most prevalent pain behavior (6/11 [55%]). Of the 11 patients, eight (73%) were taking benzodiazepines for symptom management, four (36%) were taking opioids, and one was taking a hypnotic sedative. Symptom improvement was observed in 10/11 (91%) patients after gabapentin initiation and titration. The use of benzodiazepine decreased in 6/8 (75%) patients, opioid use decreased in 3/4 patients, and hypnotic sedative use decreased in 1/1 patient. Successful discontinuation occurred for benzodiazepines in 5/8 (62.5%) patients, opioids in 1/4 (25%) patients, and hypnotic sedatives in 1/1 patient. Conclusions: Prescription medications with substantive risks, including benzodiazepines, opioids, and hypnotic sedatives, were used with high prevalence for pain behaviors in children with SNI. This study revealed an association between gabapentin initiation, and improved symptom burden and decreased use of the three medications.

Establishment of a nonshockable rhythm cardiac arrest model caused by asphyxia.

OBJECTIVE: Cardiac arrest (CA) is caused by a nonshockable rhythm with a low success rate of return of spontaneous circulation (ROSC) and a poor prognosis. This study intended to establish a nonshockable rhythm CA model caused by asphyxia. MATERIALS AND METHODS: Healthy adult male Wistar rats were injected with vecuronium bromide to induce CA. After the CA duration reached the target time point, cardiopulmonary resuscitation was performed. The survival status and neurological and cardiac function were evaluated after ROSC. Brain histopathology, including hematoxylin staining, Nissl staining and Terminal dUTP nick-end labeling (TUNEL) staining, was performed to evaluate the surviving cells and apoptotic cells. Apoptosis-related proteins after ROSC for 72 h were analyzed by western blot. RESULTS: CA was successfully induced in all animals. The time for the three groups of animals to PEA was 320 ± 22 s in the CA-8 group, 322 ± 28 s in the CA-12 group and 320 ± 18 s in the CA-15 group. The time to asystole was 436 ± 54 s in the CA-8 group, 438 ± 62 s in the CA-12 group and 433 ± 56 s in the CA-15 group. The NDS of rats in the CA group was significantly decreased after ROSC for 24 h. The NDS in the CA-15 group was 5-16 points, while it was 58-67 points and 15-43 points in the CA-8 and CA-12 groups, respectively. The cardiac function of animals in the CA group was impaired after ROSC, and the ejection fraction, fractional shortening, stroke volume and cardiac output, were all significantly decreased. Brain histopathology showed that the number of surviving neurons was decreased, and the number of apoptotic cells was increased in CA group, the longer the CA duration, the more apoptotic cells increased. The expression of the proapoptotic protein Bax and the apoptotic executive protein caspase3 in the hippocampus of CA rats was significantly increased, while the expression of the antiapoptotic protein Bcl-2 was significantly reduced. CONCLUSIONS: The use of vecuronium can successfully induce CA caused by nonshockable rhythm in rats, which will help to further study the pathophysiological changes after CA by nonshockable rhythm.

Quality of life of caregivers supporting neurologically impaired patients who underwent surgery.

BACKGROUND: A questionnaire survey was conducted to clarify whether surgical treatments performed by a pediatric surgeon improve the quality of life (QOL) of caregivers (medical doctors, nurses, physical therapists, and nursery teachers) supporting neurologically impaired (NI) patients who work at specialized institutions. METHODS: The questionnaire survey was conducted with caregivers who cared for NI patients who underwent an operation at the Department of Pediatric Surgery, Kurume University Hospital. Quality-of-life related responses concerning the change of care after surgical procedures in NI patients were collected in addition to the background data related to caregivers and the surgical procedures performed for them. Degrees of difficulty in caring for NI patients and of the caregivers' own QOL postoperatively were also compared. RESULTS: There were 192 valid responses. The majority of responses were from females and nurses aged in their 40s and 50s. The surgical procedure performed most often in the NI patients was gastrostomy. Of the respondents, 77% answered that the postoperative care became easier. The degrees of difficulty in caring for NI patients and the caregivers' own QOL were significantly higher and lower, respectively, among physical therapists compared with caregivers in other occupations. Moreover, the QOL in respondents with factors related to a longstanding relationship with NI patients was significantly higher compared with the respondents without them. CONCLUSION: This survey indicated that surgical procedures for NI patients might contribute to improved QOL for the caregiver by reducing the difficulty of caring for NI patients.

A Standardized Multimodal Neurological Monitoring Protocol-Guided Cerebral Protection Therapy for Venoarterial Extracorporeal Membrane Oxygenation Supported Patients.

Background: The main objective of this study was to investigate the role of a multimodal neurological monitoring (MNM)-guided protocol in the precision identification of neural impairment and long-term neurological outcomes in venoarterial extracorporeal membrane oxygenation (VA-ECMO) supported patients. Methods: We performed a cohort study that examined adult patients who underwent VA-ECMO support in our center between February 2010 and April 2021. These patients were retrospectively assigned to the "with MNM group" and the "without MNM group" based on the presence or absence of MNM-guided precision management. The differences in ECMO-related characteristics, evaluation indicators (precision, sensitivity, and specificity) of the MNM-guided protocol, and the long-term outcomes of the surviving patients were measured and compared between the two groups. Results: A total of 63 patients with VA-ECMO support were retrospectively assigned to the without MNM group (n = 35) and the with MNM group (n = 28). The incidence of neural impairment in the without MNM group was significantly higher than that in the with MNM group (82.1 vs. 54.3%, P = 0.020). The MNM group exhibited older median ages [52.5 (39.5, 65.3) vs. 31 (26.5, 48.0), P = 0.008], a higher success rate of ECMO weaning (92.8 vs. 71.4%, P = 0.047), and a lower median duration of building ECMO [40.0 (35.0, 52.0) vs. 58.0 (48.0, 76.0), P = 0.025] and median ECMO duration days [5.0 (4.0, 6.2) vs. 7.0 (5.0, 10.5), P = 0.018] than the group without MNM. The MNM-guided protocol exhibited a higher precision rate (82.1 vs. 60.0%), sensitivity (95.7 vs. 78.9%), and specificity (83.3 vs. 37.5%) in identifying neural impairment in VA-ECMO support patients. There were significant differences in the long-term outcomes of survivors at 1, 3 and 6 months after discharge between the two groups (P < 0.05). However, the results showed no significant differences in ICU length of stay (LOS), hospital LOS, survival to discharge, or 28-day mortality between the two groups (P > 0.05). Conclusion: The MNM-guided protocol is conducive to guiding intensivists in the improvement of cerebral protection therapy for ECMO-supported patients to detect and treat potential neurologic impairment promptly, and then improving long-term neurological outcomes after discharge.

Nutrition and preparation of blenderized tube feeding in children and adolescents with neurological impairment: A scoping review.

BACKGROUND: The use of homemade tube feeding formula has become increasingly popular for children requiring enteral nutrition. This project aimed to investigate nutrition and preparation of blenderized tube feeding in the field of children and adolescents with neurological impairment. METHODS: A scoping review was performed using established methodologies. In January 2021, we searched PubMed, Embase, CINAHL Complete, the Cochrane Central Register of Controlled Trials, and gray literature to identify relevant articles. MAJOR FINDINGS: Twenty-two papers were included describing the composition of food items, preparation procedures, and food safety. No randomized controlled trials and only a few prospective studies were included. A broad variety of food items from all food groups and many examples of recipes were presented. Most recipes provided 1.0 kcal/ml but tended to contain less energy and nutrients than expected, which could be due to preparation issues, such as sieving and the high viscosity of the blend. Preparation requires a commercial-grade household blender and diligence to ensure thorough household hygiene for adequate food safety. CONCLUSIONS: This review revealed practical experience in the nutrition and preparation aspects of blenderized tube feeding but minimal empirical evidence. Multiple examples of the composition of food items and preparation procedures for blenderized tube feeding were found, but uncertainty regarding the ideal composition or preparation was also exposed. The future of blenderized tube feeding would benefit from clinically tested recipes that include an evaluation of nutrients, viscosity, and microbial contamination, as well as the effect of the food's appearance and scent on the target group.

A Pragmatic Approach to Assessment of Chronic and Recurrent Pain in Children with Severe Neurologic Impairment.

The term "severe neurologic impairment" (SNI) is used to describe a group of disorders of the central nervous system which arise in childhood, resulting in motor impairment, cognitive impairment and medical complexity. As a result, much assistance is required with activities of daily living. Since these patients are often unable to self-report pain, or they may exhibit uncommon behaviors when suffering, pain manifestations may go unrecognized. In this article, the basic principles of how to approach pain in children with SNI are discussed.

Validation of Neurologic Impairment Diagnosis Codes as Signifying Documented Functional Impairment in Hospitalized Children.

OBJECTIVE: To assess the performance of previously published high-intensity neurologic impairment (NI) diagnosis codes in identification of hospitalized children with clinical NI. METHODS: Retrospective study of 500 randomly selected discharges in 2019 from a freestanding children's hospital. All charts were reviewed for 1) NI discharge diagnosis codes and 2) documentation of clinical NI (a neurologic diagnosis and indication of functional impairment like medical technology). Test statistics of clinical NI were calculated for discharges with and without an NI diagnosis code. A sensitivity analysis varied the threshold for "substantial functional impairment." Secondary analyses evaluated misclassified discharges and a more stringent definition for NI. RESULTS: Diagnosis codes identified clinically documented NI with 88.1% (95% confidence interval [CI]: 84.7, 91) specificity, and 79.4% (95% CI: 67.3, 88.5) sensitivity; negative predictive value (NPV) was 96.7% (95% CI: 94.8, 98.0), and positive predictive value (PPV) was 49% (95% CI: 42, 56.1). Including children with milder functional impairment (lower threshold) resulted in NPV of 95.7% and PPV of 77.5%. Restricting to children with more severe functional impairment (higher threshold) resulted in NPV of 98.2% and PPV of 44.1%. Misclassification was primarily due to inclusion of children without functional impairments. A more stringent NI definition including diagnosis codes for NI and feeding tubes had a specificity of 98.4% (95% CI: 96.7-99.3) and sensitivity of 28.6% (19.4-41.3). CONCLUSIONS: All scenarios evaluated demonstrated high NPV and low-to-moderate PPV of the diagnostic code list. To maximize clinical utility, NI diagnosis codes should be used with strategies to mitigate the risk of misclassification.

Defining Persistent Total Parenteral Nutrition Use in Patients with Neurologic Impairment.

Background: Patients with neurologic impairment (NI) experience gastrointestinal symptoms as one of three common problems associated with NI, including occasional persistent total parenteral nutrition (TPN) use. Objective: To describe the incidence of persistent TPN use in patients with NI. Design: Retrospective chart review on patients 0-38 years old enrolled in the Complex Health Care Program from January 2011 to October 2015. Setting/Subjects: This study occurred in a United States pediatric tertiary care hospital. Two hundred and eight participants were included based on NI, utilizing a surgical feeding tube, and having encounters with a dietitian. Measurements: The primary outcome was incidence of persistent TPN use in patients with NI. Secondary outcomes included mortality rate, hospitalization frequency, time-to-TPN initiation, and describing symptoms preceding persistent TPN use. Results: Median number of admissions was 4 for 168 hospitalized patients (59% male, 58% White). One hundred twenty-five patients required admission for unplanned bowel rest with average length-of-stay of 7.3 days. Twenty-six patients required TPN initiation. Average time-to-TPN was two years since enrollment. Mortality rate was 14% (n = 28). TPN initiation (odds ratio [OR]: 3.99; 95% confidence interval [CI]: 1.16-13.8) was significantly associated with increased OR of mortality. Conclusions: Our study demonstrates a substantial population of patients with NI and surgical feeding tube are affected by persistent feeding intolerance. We propose that persistent TPN use may be a risk factor for mortality. Additional research is needed to delineate relationships between persistent TPN use, hospitalizations, and mortality.

Severity and Outcome of Neurologic Deficits in Patients with Pyogenic Spondylodiscitis: A Systematic Review.

Patients with advanced pyogenic spondylodiscitis can present with neurologic deficits. However, the prevalence, severity, and outcome of the neurologic deficits are not well known. A systematic review was performed to improve the knowledge of this commonly encountered clinical scenario. The considerable number of severe neurologic deficits in addition to their poor recovery, even after surgical intervention, demonstrates that the early diagnosis of pyogenic spondylodiscitis is crucial. Prompt surgical intervention is likely associated with a greater chance of improvement of neurologic status than nonsurgical treatment.

Progressive Multifocal Leukoencephalopathy in a Chemotherapy-Naive Patient With Chronic Lymphocytic Leukemia: A Case Report.

Progressive multifocal leukoencephalopathy is a rare, progressive demyelinating disease of the central nervous system caused by reactivation and replication of the John Cunningham (JC) virus in cerebral oligodendrocytes. The JC virus is a small ubiquitous polyomavirus that can be detected in up to 50% of the adult population. It affects almost exclusively immunocompromised patients and is generally observed in patients with acquired immunodeficiency syndrome and patients with hematologic malignancies and autoimmune or chronic inflammatory diseases medicated with immunosuppressive and immunomodulatory drugs. However, it is rarely described in patients with hematologic malignancies, not undergoing chemotherapy or immunosuppressive therapy. It has a poor prognosis, and the treatment is based on restoring the immune system, given that no specific antiviral treatment is available. We present a case of a chemotherapy-naive patient with chronic lymphocytic leukemia associated with progressive multifocal leukoencephalopathy.

Severe neurologic impairment and respiratory failure after fall and hip fracture in an elderly woman.

Fat embolism syndrome is a rare complication of long bone fractures and orthopedic surgery. We report a case of a 90-year-old woman who developed severe neurologic impairment and respiratory failure few hours after an accidental fall complicated by fracture of the left femur neck and left humerus.