Maria Chiara Paolino

Melatonin to prevent migraine or tension-type headache in children

We designed a 3-month open label trial of melatonin prophylaxis in children with primary headache. After a one month baseline period without receiving preventive drugs, all children received a 3-month course of melatonin, 3 mg, administered orally, at bedtime. A total of 22 children were enrolled (10 boys, mean age 12.2±2.6 years, age range 6–16 years), 13 had recurrent migraine without aura, 1 with aura and 8 had chronic tension-type headache. When the trial ended, 14 of the 21 subjects reported that the headache attacks had decreased by more than 50% in respect to baseline and 4 of them reported having no headache attacks. After receiving melatonin for one month one subject dropped out because of excessive daytime sleepiness. Our promising results warrant randomized placebo-controlled trials in children to assess the real effectiveness of melatonin in preventing primary headache.

Neurocognitive assessment and sleep analysis in children with sleep-disordered breathing

OBJECTIVE To assess possible correlations between intelligence quotient (IQ) and attention deficit hyperactive disorder (ADHD) rating scale values and sleep (including cyclic alternating patterns analysis) and respiratory parameters in children with sleep-disordered breathing (SDB). METHODS Thirteen children who satisfied the criteria for primary snoring and 31 children for obstructive sleep apnea syndrome (OSAS) underwent polysomnography in a standard laboratory setting and a neurocognitive assessment. Sixty normal controls recruited from two schools underwent the neurocognitive assessment. RESULTS The IQ estimates of controls were higher and the ADHD rating scale scores lower than those of children with SDB. Children with OSAS had a higher REM sleep latency and arousal index as well as a lower N3 and A mean duration than children who snored. In our sample of children with SDB, the percentage of wakefulness after sleep onset, of N1, of A2, of arousal and A2 index correlated positively with global intelligence. Total and hyperactivity scores correlated positively with the A2 index. Regression analysis mostly confirmed the correlations between neurocognitive measures and sleep parameters and further demonstrated a negative correlation between the hyperactivity rating score and oxygen saturation during the night. CONCLUSIONS Our results support the hypothesis that arousal is a defensive mechanism that may preserve cognitive function by counteracting the respiratory events, at the expense of sleep maintenance and NREM sleep instability. SIGNIFICANCE We believe that our study makes an interesting contribution to research on the relationship between sleep fragmentation and cognitive function.

Coexistence of epilepsy and Brugada syndrome in a family with SCN5A mutation

Cardiac arrhythmias are associated with abnormal channel function due to mutations in ion channel genes. Epilepsy is a disorder of neuronal function also involving abnormal channel function. It is increasingly demonstrated that the etiologies of long QT syndrome and epilepsy may partly overlap. However, only a few genetic studies have addressed a possible link between cardiac and neural channelopathies. We describe a family showing the association between Brugada syndrome and epilepsy in which a known mutation in the SCN5A gene (p.W1095X, c.3284G>A) was identified. We suggest that this mutation can be responsible for cardiac and brain involvement, probably at different developmental age in the same individual. This observation confirms the possibility that SCN5A mutations may confer susceptibility for recurrent seizure activity, supporting the emerging concept of a genetically determined cardiocerebral channelopathy.

Sleep clinical record: an aid to rapid and accurate diagnosis of paediatric sleep disordered breathing

Overnight polysomnography (PSG) is an expensive procedure which can only be used in a minority of cases, although it remains the gold standard for the diagnosis of sleep disordered breathing (SDB). The objective of this study was to develop a simple, PSG-validated tool to screen SDB, thus reducing the use of PSG. For every participant we performed PSG and a sleep clinical record was completed. The sleep clinical record consists of three items: physical examination, subjective symptoms and clinical history. The clinical history analyses behavioural and cognitive problems. All three items were used to create a sleep clinical score (SCS). We studied 279 children, mean±sd age 6.1±3.1 years, 63.8% male; 27.2% with primary snoring and 72.8% with obstructive sleep apnoea (OSA) syndrome. The SCS was higher in the OSA syndrome group compared to the primary snoring group (8.1±9.6 versus 0.4±0.3, p<0.005), correlated with apnoea/hypopnoea index (p=0.001) and had a sensitivity of 96.05%. Positive and negative likelihood ratios were 2.91 and 0.06, respectively. SCS may effectively be used to screen patients as candidates for PSG study for suspected OSA syndrome, and to enable those with a mild form of SDB to receive early treatment.

Cognitive profile, parental education and BMI in children: reflections on common neuroendrocrinobiological roots.

ABSTRACT Overweight and obesity may be associated with cognitive problems and both may share “neuroendocrinobiological roots” in common cerebral areas. We investigated intellectual performances and a possible “specific cognitive profile” in overweight/obese children. A cross-sectional study was conducted on 898 school children (6 to 13 years) attending primary schools. Wechsler Intelligence Scale for Children-revised (WISC-R) revealed significant differences in performance intelligence quotient (PIQ) scores between body mass index (BMI) subgroups (p<0.01). Regression analysis identified BMI as the only variable significantly related to PIQ (p<0.05). Gender (p<0.05) and parental educational score (p<0.001) were significantly related to verbal intelligence quotient (VIQ). Parental educational score was the only factor significantly related to total intelligence quotient (TIQ) (p<0.05). Parental education seems to play a major role in TIQ and VIQ; a lower PIQ score is clearly related to a higher BMI. A routine neurocognitive assessment in overweight/ obese children is recommended. Finally, we have added some reflections on common neuroendocrinobiological roots.

Visual scoring of sleep: A comparison between the Rechtschaffen and Kales criteria and the American Academy of Sleep Medicine criteria in a pediatric population with obstructive sleep apnea syndrome

OBJECTIVE To compare the new American Academy of Sleep Medicine (AASM) criteria for scoring sleep with the previous Rechtschaffen and Kales (R&K) criteria in a cohort of children with primary snoring, obstructive sleep apnea syndrome (OSAS) and normal controls. METHODS Polysomnography was performed in 26 consecutive children with primary snoring (13 males, mean age 6.2 years, SD 3.2), in 39 with OSAS (24 males, mean age 6.1 years, SD 3.0), and in 10 age-matched normal controls. RESULTS Compared to the other groups, OSAS children showed a lower percentage of slow-wave sleep, using both R&K and AASM criteria; they also showed a higher percentage of stage shifts, and N1, using the AASM criteria. Children with primary snoring showed a higher percentage of N1, compared to controls. CONCLUSIONS These results indicate that the use of the new AASM criteria seem to disclose more differences in sleep parameters than the R&K rules in children with OSAS. SIGNIFICANCE The AASM criteria seem to disclose a high degree of sleep fragmentation in children with OSAS, mostly related to the repeated occurrence of N1.

Migraine treatment in developmental age: guidelines update

There is a serious lack of controlled studies on the pharmacological treatment of primary migraine in the developmental age; there is, consequently, an urgent need for new, evidence-based approaches to this long-neglected field of research. Moreover, previous studies have stated that the placebo response is greater in pediatric patients than in adults and that a reduction in the attack frequency in the absence of any pharmacological treatment is observed more frequently in pediatric migraine patients than in adults. Besides these preliminary considerations, the shorter duration of migraine attacks and other characteristic semeiological features of the clinical picture in children are such that the design of randomized controlled trial (RCT) is more problematic in the developmental age than in the adult. Bearing in mind all these weak points, the aim of this review was to summarize and update recent guidelines for the treatment of primary migraine in children and adolescents. The most recent guidelines are those published by the Italian Society for the study of Headache, the French Society for the study of Migraine and Headache, and the American Academy of Neurology. We have incorporated into these guidelines the results from the few, recent RCTs, clinical controlled trials, open-label studies, meta-analyses and reviews that have been published since 2004; owing to the lack of strong evidence in this field of research, we have sometimes even mentioned pilot non-controlled studies, case series and expert opinions. Lastly, evidence was classified and the recommendations were categorized according to different levels.

Metabolic epilepsy: an update.

Inborn errors of metabolism comprise a large class of genetic diseases involving disorders of metabolism. Presentation is usually in the neonatal period or infancy but can occur at any time, even in adulthood. Seizures are frequent symptom in inborn errors of metabolism, with no specific seizure types or EEG signatures. The diagnosis of a genetic defect or an inborn error of metabolism often results in requests for a vast array of biochemical and molecular tests leading to an expensive workup. However a specific diagnosis of metabolic disorders in epileptic patients may provide the possibility of specific treatments that can improve seizures. In a few metabolic diseases, epilepsy responds to specific treatments based on diet or supplementation of cofactors (vitamin-responsive epilepsies), but for most of them specific treatment is unfortunately not available, and conventional antiepileptic drugs must be used, often with no satisfactory success. In this review we present an overview of metabolic epilepsies based on various criteria such as treatability, age of onset, seizure type, and pathogenetic background.

Headache and attention deficit and hyperactivity disorder in children: Common condition with complex relation and disabling consequences

The aim of this review was to analyze literature data on the complex association between headache and attention deficit and hyperactivity disorder (ADHD) in children, in order to explore its possible consequences on child neurological development. Headache and ADHD are two common conditions in the pediatric population. They both are disabling diseases that impact the childs quality of life and are associated with severe cognitive, emotional, and behavioral impairments. To assess and analyze literature data about the association of ADHD and headache in children and possible physiopathogenesis relationships, we searched for the following terms: headache, migraine, tension-type headache, ADHD, and children (MESH or text words). We found different studies that assess the clinical, epidemiological, and physiopathogenetic overlap between these two diseases, with contrasting results and unresolved questions. Structural and functional abnormalities in brain networks have been found to be central in both headache and ADHD pathophysiology. It will be crucial to gain a better understanding of how subcortical-cortical and corticocortical network development is altered during the onset of the disorders.

Sleep cyclic alternating pattern analysis in infants with apparent life-threatening events: A daytime polysomnographic study

OBJECTIVE Non-REM sleep is characterized by a physiologic oscillating pattern that exhibits different levels of arousal, coded as cyclic alternating pattern. The aim of this study was to analyze the development of cyclic alternating pattern parameters in a group of infants with apparent life-threatening events. METHODS A total of 26 infants with apparent life-threatening events (14 females, mean age 3.4 months, 2.37 S.D., age range 0.5-9 months) were studied while they slept in the morning between feedings, by means of a 3-h video-electroencephalographic-polygraphic recording. Sleep was visually scored using standard criteria. The control group was composed of 36 healthy infants (16 females, mean age 3.2 months, 2.17 S.D., age range 0.5-9 months). RESULTS Children with apparent life-threatening events showed an increased frequency of periodic breathing, gastroesofageal reflux and of other risk conditions. They presented also an increased obstructive apnoea/hypopnea index. A full NREM sleep development was found in a significantly smaller percentage of patients, and they showed a significant reduction of the percentage of REM sleep, of cyclic alternating pattern A1 subtypes, an increased percentage of A2 and A3 subtypes and increased index of A2, A3 subtypes and arousal, compared to normal controls. Cyclic alternating pattern rate showed a significant positive correlation with age, only in controls. CONCLUSIONS Our results show a higher level of arousal and an increased non-REM sleep discontinuity in babies with apparent life-threatening events, compared to controls. SIGNIFICANCE The enhanced mechanism of arousal might counteract life-threatening events and represent an important neurophysiologic distinction from future victims of sudden infant death syndrome who also experience similar events.