Benign paroxysmal torticollis, benign paroxysmal vertigo, and benign tonic upward gaze are not benign disorders.

AIM: Benign paroxysmal torticollis (BPT), benign paroxysmal vertigo (BPV), and benign tonic upward gaze (BTU) are characterized by transient and recurrent episodes of neurological manifestations. The purpose of this study was to analyse the clinical relationships between these syndromes, associated comorbidities, and genetic bases. METHOD: In this cross-sectional study, clinical data of patients with BPT, BPV, or BTU were collected with a focus on developmental achievements, learning abilities, and rehabilitation. Neuropsychological assessment and genetic testing were performed. RESULTS: Fifty patients (median age at inclusion 6y) were enrolled. Psychomotor delay, abnormal neurological examination, and low or borderline IQ were found in 19%, 32%, and 26% of the patients respectively. Cognitive dysfunction was present in 27% of the patients. CACNA1A gene mutation was identified in eight families, and KCNA1 and FGF14 mutation in one family respectively. The identification of a CACNA1A mutation was significantly associated with BTU (p=0.03) and with cognitive dysfunction (p=0.01). Patients with BPV were less likely to have cognitive dysfunction. INTERPRETATION: Children with BPT, BPV, or BTU are at high risk of impaired psychomotor and cognitive development. These syndromes should not be regarded as benign and should be considered as part of the spectrum of a neurodevelopmental disorder. WHAT THIS PAPER ADDS OK: Patients with benign paroxysmal torticollis (BPT), benign paroxysmal vertigo (BPV), and benign tonic upward gaze (BTU) have an increased risk of psychomotor delay. These patients also have an increased risk of abnormal neurological examination and cognitive dysfunction. Gene mutations, especially in CACNA1A, were identified in 21% of the families. BPT, BTU, and BPV should not be regarded as benign. BPT, BTU, and BPV should be considered as part of the spectrum of a neurodevelopmental disorder.

Efficacy and Tolerance of Lidocaine 5% Patches in Neuropathic Pain and Pain Related to Vaso-occlusive Sickle Cell Crises in Children: A Prospective Multicenter Clinical Study.

BACKGROUND: The management of neuropathic pain and pain related to bone vaso-occlusive crises in sickle cell disease remains challenging in children. Lidocaine 5% patches are recommended in adults for neuropathic pain treatment, but they are not recommended in children. The purpose of this study was to assess the efficacy and tolerance of lidocaine 5% patches in pediatric inpatients. METHODS: This prospective, multicenter, single-arm, phase II study aimed to assess the use of lidocaine 5% patches in 6- to 21-year-old pediatric patients suffering from neuropathic pain or superficial bone vaso-occlusive crises. Patches were applied on the painful area for 12 hours a day. The primary endpoint was the proportion of inpatients with significant pain relief defined as a decrease of at least 2 points on the visual analog pain scale (VAS) measured at 12 hours after patch placement over at least 2 consecutive days. RESULTS: The 12-hour VAS score decreased by at least 2 points over 2 consecutive days in 48.6% of patients 95% unilateral confidence interval (33.8%). Only 7.7% of patients experienced grade 1 or grade 2 toxicities. CONCLUSION: Although lidocaine 5% patches decreased the pain's intensity in nearly half of the enrolled patients with an excellent tolerance, the efficacy endpoint was not reached. Further studies should consider a more refined selection of the experimental population to assess the efficacy of lidocaine 5% patches in the pediatric population.

Longitudinal study shows that depression in childhood is associated with a worse evolution of headaches in adolescence.

AIM: The aim of this study was to examine the course of headache diagnosis, headache frequency, anxiety, comorbid depressive symptoms and school absenteeism in adolescents with migraine and tension-type headaches five years after baseline. METHODS: We followed a group of 122 children with a mean age of 10.1 (±1.3) years, with headache from a paediatric migraine centre in Paris who had taken part in a previous study from September 2007 to June 2008. This five-year longitudinal study took place in January to June 2012. The measures that were used included demographic variables, headache diagnosis, headache data and a psychological assessment. RESULTS: At the five-year point, about 22% of the children had become headache free, 34% had little to no disability, and 36% had a changed diagnosis. Moreover, a longer history of headache at baseline was associated with a worse evolution of headache at follow-up. Lastly, high depression scores, but not anxiety, were a predictor of more headache disability at follow-up. CONCLUSION: High depression scores in childhood were a risk factor that was associated with persistence and worsening of headaches in adolescence. This suggests that mental health assessments should be carried out in paediatric headache pain clinics.

[Measuring the intensity of restraint during care in children: Validation of the Pric scale]. / Mesurer l'intensité de la contention lors des soins chez les enfants : validation de l'échelle Pric.

Restraint is used relatively often during pediatric care. However, no scale has yet been validated to assess its intensity. The study presented here did this for the Procedural Restraint Intensity in Children tool in metrological terms (with some limitations). In the absence of a reference scale in this area, the reliability of this tool was studied under experimental conditions. It is nevertheless the first scale with metrological validation, measuring the intensity of physical constraint. Other work is underway to validate it in real clinical situations.

A simple tool to measure procedural restraint intensity in children: validation of the PRIC (Procedural Restraint Intensity in Children) scale.

PURPOSE: Restraint is often used when administering procedures to children. However, no metrologically scale to measure the restraint intensity had yet been validated. This study validated the metrological criteria of a scale measuring the restraint intensity, Procedural Restraint Intensity in Children (PRIC), used during procedures in children. DESIGN AND METHODS: The PRIC scale performance was measured by a group of 7 health professionals working in a children's hospital, by watching 20 videos of health care procedures. This group included 2 physicians, 1 pediatric resident, and 4 nurses. The intra-class correlation coefficients were calculated to evaluate the inter-rater and test-retest reliability and the construct validity with the correlation between PRIC scale and a numerical rating scale. RESULTS: One hundred and forty measurements were made. Inter-rater and test-retest correlation coefficients were 0.98 and 0.98, respectively. The 2 scales were positively correlated with a Spearman coefficient of 0.93. CONCLUSIONS: This study validated the Procedural Restraint Intensity in Children (PRIC) scale in metrological terms with some limitation. However, there is not gold standard scale to precisely validate the reliability of this tool and this study has been conducted in "experimental" conditions. Nevertheless, this is the first scale measuring the intensity of physical restraint with a metrological validation. The next step will be to validate it in real clinical situations.

Early Diagnosis of Migraine Necessary in Children: 10-Year Follow-Up.

BACKGROUND: The prevalence of migraine in individuals younger than 20 years old is 8%, with a mean age at onset of younger than 8 years. However, the long-term prognosis of migraine attacks has not been clearly established. OBJECTIVE: Our main objective was to evaluate disease course 10 years after migraine diagnosis in a cohort of children and adolescents. METHODS: Migraine was diagnosed in 1999 in a pediatric headache reference center using International Headache Society criteria. In 2009, all patients were interviewed by telephone on the persistence and characteristics of any headaches. The main end point was prevalence of persistent migraine attacks in 2009. Variables associated with persistent attacks were analyzed. RESULTS: Overall, 142 children were diagnosed with migraine in 1999. Of these, 84 were interviewed by telephone in 2009. In 1999, the mean age was 11.6 ± 3.1 years, 54% were male, and 50% had migraine without aura. Migraine attacks were common (1-3 attacks/week in 38%). Mean age at onset was 7 years and 4 months (±3 years). In 2009, migraine prevalence was 39/84 (46% [95% confidence interval 36-56]), 12 patients (14%) were headache-free, and 62 patients (74%) had tension-type headaches that were isolated headaches in 33 (39%) patients. The rate of patients with at least one migraine attack per week fell from 37% to 8% (P = 0.001) over the 10-year period. Age at the first visit to the center was significantly higher in 2009 migraine sufferers than nonsufferers (12.5 ± 3.0 versus 10.9 ± 3.1 years (P = 0.02)). In multivariate analysis, age ≥12 years at the time of first presentation was the only significant factor associated with long-term chronic migraine (odds ratio = 3.0 [1.1-8.0]). CONCLUSIONS: Ten years after first presentation, 46% of patients had migraine but the frequency of attacks had diminished. The only factor associated with chronic migraine was age ≥12 years at first presentation.

The efficacy of premixed nitrous oxide and oxygen for fiberoptic bronchoscopy in pediatric patients: a randomized, double-blind, controlled study.

STUDY OBJECTIVE: s: The aim of the study was to evaluate the efficacy and safety of premixed 50% nitrous oxide and oxygen on the quality of sedation and pain control during fiberoptic bronchoscopy (FB) in children. DESIGN: A prospective, randomized, double-blind study. SETTING: Pediatric pulmonary department in a pediatric tertiary university hospital. PATIENTS: One hundred five children aged 1 month to 18 years. INTERVENTIONS: Patients inhaled after sedation and local anesthesia either premixed 50% nitrous oxide and oxygen (nitrous oxide group) or premixed 50% nitrogen and oxygen (control group) during FB. MEASUREMENT AND RESULTS: The rate of failure was significantly greater in the control group (62%) than in the nitrous oxide group (21%, p = 0.00003). The efficacy of premixed 50% nitrous oxide and oxygen was also demonstrated with higher satisfaction scores (p = 0.000001), lower Children's Hospital of Eastern Ontario Pain Scores (p = 0.002), better visual analog scale ratings (p = 0.03), and improved behavior scores. Side effects were minor and similar in both groups. CONCLUSIONS: This study demonstrates the improved efficacy of sedation, pain control, and safety of premixed 50% nitrous oxide and oxygen for FB in children.

Anxiety, depression and school absenteeism in youth with chronic or episodic headache.

BACKGROUND: Chronic daily headache (CDH) in children has been documented in general and clinical populations. Comorbid psychological conditions, risk factors and functional outcomes of CDH in children are not well understood. OBJECTIVES: To examine anxiety and depression, associated risk factors and school outcomes in a clinical population of youth with CDH compared with youth with episodic headache (EH). METHODS: Data regarding headache characteristics, anxiety, depression and missed school days were collected from 368 consecutive patients eight to 17 years of age, who presented with primary headache at a specialized pediatric headache centre. RESULTS: A total of 297 patients (81%) were diagnosed with EH and 71 were diagnosed with CDH. Among those with CDH, 78.9% presented with chronic tension-type headache and 21.1% with chronic migraine (CM). Children with CDH had a higher depression score than the standardized reference population. No difference was observed for anxiety or depression scores between children with CDH and those with EH. However, children with CM were more anxious and more depressed than those with chronic tension-type headache. Youth experiencing migraine with aura were three times as likely to have clinically significant anxiety scores. Headache frequency and history were not associated with psychopathological symptoms. Children with CDH missed school more often and for longer periods of time. CONCLUSIONS: These findings document the prevalence of anxiety, depression and school absenteeism in youth with CDH or EH. The present research also extends recent studies examining the impact of aura on psychiatric comorbidity and the debate on CM criteria.

EVENDOL, a new behavioral pain scale for children ages 0 to 7 years in the emergency department: design and validation.

Numerous behavioral pain measures have been validated for young children, but none is appropriate to assess pain in emergency departments (EDs), where caregivers need a simple, easily completed scale. Our objective was to elaborate and validate a tool, relevant in any painful situation, with agitation or prostration, and for any age under 7 years. Five items (scored 0 to 3) were developed by pediatric pain and emergency caregivers. The new scale, called EVENDOL, was tested at children's arrival and after analgesics, at rest, and during mobilization. The validation study included 291 children from birth to 7 years old in 4 French EDs, and independent observations by the ED nurse and a researcher. The Cronbach coefficient was excellent (0.83 to 0.92). Construct validity was demonstrated by a decrease in scores after nalbuphine: 8.14 to 3.62 of 15 at rest (P<.0001), 11.87 to 6.65 at mobilization (P = .0011); by good correlations between EVENDOL and nurse or researcher numerical scores: 0.79 to 0.92 (P<.0001); by good correlations between children's self-assessment scores and EVENDOL in children ages 4 to 7 (0.64 to 0.93). Discriminant validity with tiredness, anxiety, and hunger was good. Interrater reliability was excellent between nurses and researcher (weighted kappa 0.7 to 0.9), and in a group of 6 nurses (simultaneous assessment of 122 videos). The treatment threshold was determined at 4 of 15. EVENDOL has excellent validity and can be used for all children under age 7 in EDs, for any age and any pain, acute as well as more prolonged.

Pain management in newborns: from prevention to treatment.

All neonates in the Neonatal Intensive Care Unit (NICU) or during the first days of life undergo painful and stressful procedures. Epidemiologic studies have shown that pain induced by these procedures is not effectively prevented or is inadequately treated. Pain experienced during the neonatal period may lead to negative outcomes, especially in preterm neonates. Prevention is the first step of pain management, and practical guidelines should be used in the NICU. Assessment must be done with adequate tools that take into account the infant's pathology and gestational age. Distinguishing between acute and prolonged pain is important for both assessment and treatment. The most common drugs that have been studied for the treatment of pain and stress are opioids, hypnosedatives, and NMDA receptor antagonists. Morphine and fentanyl are most frequently used for acute or prolonged pain in the NICU. They have potent analgesic effects and few immediate or long-term adverse effects. Midazolam is a commonly used hypnosedative, but its adverse effects limit its use. Drugs such as propofol and ketamine have been used for acute painful procedures; however, further research is needed to assess their long-term effects. Use of non-pharmacologic pain management techniques has increased in recent years. These methods are easy, inexpensive, and effective in helping newborns recover from painful procedures. Sweet solutions and non-nutritive sucking, breastfeeding, skin-to-skin mother care, swaddling, and facilitated tucking are the most commonly employed and evaluated non-pharmacologic methods. Hospitals should promote and improve parent involvement in pain management. In-service education and well organized hospital teams are crucial for successful implementation of pain protocols in newborns.