Background:On the basis of clinical experience, it seems that sleep disturbances are common in children with cerebral palsy (CP); however, there is a lack of research and objective data to support this observation.Aim of Work:Our aim was to assess sleep of children with cerebral palsy, using validated sleep questionnaire.Subjects and Methods:one hundred children with diagnosis of CP were investigated via sleep questionnaires, with their ages from 2-12 years. The 100 children with CP were divided into two groups, pre-school group (52 children had a mean age 2.35 ± 1.04 years) and school ages group (48 children had a mean age 10.21 ± 3.75 years).Results:We found high incidence of sleep problem in both pre-school and school age groups. We found that pre-school children have more prevalence of early insomnia (46.2%, P value 0.028) and sleep bruxism (50%, P value 0.000), while school group suffer more sleep disordered breathing (SDB) (50%, P value 0.001), more nightmares (50%, P value 0.001), more sleep talking (12.5% P value 0.049), and more excessive daytime sleepiness (EDS) (62.5%, P value 0.001).Conclusion:Results of our study indicate that CP children have high incidence of sleep problem in both pre-school and school age groups.

Objective: To evaluate the impact of iron chelating drugs and serum ferritin on the neurocognitive functions of patients with β thalassemia major (β-TM), using psychometric, neurophysiologic and radiologic tests. Methods: Eighty children with β-TM were enrolled into the study and were compared to 40 healthy controls. All participants were evaluated by measuring serum ferritin, neurocognitive assessment by Benton Visual Retention Test, Wechsler Intelligence Scale for Children, Wisconsin Card Sort Test, P300 and magnetic resonance spectroscopy (MRS). Results: WISC in our study showed that 40% of cases were borderline mental function as regards total IQ. Neurophysiologic tests were significantly impaired in patients compared to control group, with significant impairment in those receiving desferrioxamine (DFO). P300 amplitude was significantly lower in cases compared to controls (2.24 and 4.66 uv, respectively), recording the shortest amplitude in patients receiving DFO. Altered metabolic markers in the brain were detected by MRS in the form of reduced N-acetylaspartate to creatine ratio in 78.3% of our cases. There were significant correlations between psychometric tests and both neurophysiologic (P300) and radiologic (MRS) tests. Conclusion: β-TM is associated with neurocognitive impairment that can be assessed by psychometric, neurophysiologic and radiologic tests. The role of hemosiderosis and iron chelation therapy on cognitive functioning still need more research.

ObjectiveOur study aimed to estimate the frequency of inborn errors of metabolism (IEMs) in patients presenting with acute encephalopathy-like picture at an emergency department (ED).Subjects and methodsOur study was a prospective observational study conducted on 30 patients admitted to the pediatric ED with unexplained acute encephalopathy. The study included 30 children with an age ranging from 1 month to 5 years. All patients were subjected to full history taking, thorough clinical examination, and laboratory investigations including serum ammonia, serum lactate, arterial blood gases, tandem mass spectroscopy, organic acid of urine, cerebrospinal fluid examination to exclude central nervous system infection plus the routine laboratory tests (kidney functions, liver functions, random blood glucose, complete blood picture), and brain imaging computed tomography and/or magnetic resonance imaging brain.ResultsThirty children presented with acute encephalopathy at the ED. All were screened for suspected IEMs. Ten (33.3%) of them was positive in the initial screening test. There were four (13.3%) patients with possible mitochondrial diseases, four (13.3%) patients with possible organic acidemia, one (3.3%) patient with possible urea cycle defect, and one (3.3%) patient with possible nonketotic hyperglycinemia.ConclusionAny case of unexplained acute encephalopathy presenting to the ED should be investigated for suspected IEM, especially in high-risk families, as early interventions will lead to improved outcome.