Iranian Journal of Child Neurology

Objective

Neurodevelopmental disability is one of the most common problems of children referred to  Pediatric Neurology Clinics. These children may suffer from speech delay, intellectual deficiency and behavioral disorder.

Some patients with neurodevelopomental delay, especially those with intellectual disability and speech problems, have abnormal electroencephalograms, without clinical seizure. It seems that treating these patients with antiepileptic drugs normalizes the EEG, thereby preventing the electrical paroxysmal discharges that could be harmful for the developing brain. Several studies reported the use of Valproate, Lamotrigine and Corticosteroid in suppression of subclinical  epileptiform discharges and improvement of developmental behavioral conditions.

condition of children with neurodevelopmental delay and elimination of subclinical EEG discharges after 18 months of treatment.

Also we used high dose intravenous methyl - prednisolone in a group of children with neurodevelopmental delay and electrical status epilepticus during slow - wave sleep without clinical seizure. In these children results of the appropriate neuro-metabolic tests and magnetic resonance imaging of the brain revealed no abnormality. Because no underlying etiology could be determined, isolated non convulsive status epilepticus was established. After treatment no

significant response was observed in these group of children.

In another study we used Lamotrigine in children with neurodevelopmental delay, abnormal epileptiform discharges but without clinical seizures. Our results revealed Lamotrigine provides effective control of both subclinical epileptiform discharges and behavioral disorder, without improvement in their cognition. Further studies are needed to investigate and confirm the cognitive and behavioral effects of Lamotrigine in children with psychomotor retardation.

Objectives

Juvenile Myoclonic Epilepsy(J.M.E.) needs life-long anti-epileptic drug (AED) treatment. Of various drugs tried in this condition, valproate effectively treats all types of seizures seen in J.M.E.

Among valproate side effects, neural tube defects (NTD) in the offspring, is a deterring factor in its use in childbearing period. To avoid NTD, most authorities advise on switching to AED drug before conception.As well, the effects of valproate on male fertility is feared of.

Clonazepam controls only the myoclonic jerks, leaving the patients unprotected and susceptible to generalized seizures, which mostly occur in the morning after sleep deprivation. Sleep deprivation is the most prevalent precipitating factor for generalized seizures in these patients.

Materials &Methods

Between Jan 2003 & April 2008, 15 newly diagnosed JME patients (9 girls, 6 boys), were given clonazepam (1.0-4.0 mg) at bedtime, and were compared to 16 patients on valproate treatment. All were advised to avoid sleep deprivation.

Results

This study showed, all patients in the clonazepam group, (100 %) had full control of their myoclonic jerks; 4(26.6%) had breakthrough episodes of generalized seizures, provoked by sleep deprivation; the rest (11,73.4 %), were in full remission of their myoclonic and generalized seizures. In control group, 2(12.5 %), had episodes of breakthrough, sleep-deprived, generalized seizures;the rest(14, 87.5%) were in remission. Statistically, there was no significance between

the results in both groups.

Clonazepam side effects were limited to mild to moderate drowsiness in the morning, eliminated by giving the dose earlier at night.

Conclusion

The study suggests that if J.M.E. patients avoid sleep deprivation, they can be reated with clonazepam alone to avoid side effects of valproate.

Objectives The cause of rheumatoid arthritis (RA) as a chronic inflammatory autoimmune disease is still unknown. It appears that both genetic and environmental factors play a role in its pathogenesis. Recent studies reveal that in addition to the CNS, immune cells synthesis neurotransmitters so that these catecholamines can regulate immune functions. The aim of this study is to evaluate the dopamine receptor gene expression profiles on peripheral blood mononuclear cells of rheumatoid arthritis patients in comparison with normal individuals. Material & Methods In the present study, we investigated dopamine receptor gene expression in PBMCs of 40 RA patients and 40 healthy individuals using Real Time-PCR.The specificities of the obtained Real time PCR products for the respective dopamine receptors fragments were confirmed by sequenced analysis capillary system Results We found that DRD1-DRD5 types of dopamine receptors genes expression profiles of rheumatoid arthritis patients differ compared to healthy individuals. Moreover, a significant difference of DR2 and DR4 gene expression was seen in rheumatoid arthritis patients. Conclusion This study showed that some types of dopamine receptors genes expression profiles alter in rheumatoid arthritis patients with comparison to healthy individuals Moreover, this alteration possibly could result in dysfunction of dopaminergic system in immune cells and finally lead to rheumatoid arthritis.

Objectives

The cause of rheumatoid arthritis (RA) as a chronic inflammatory autoimmune disease is still unknown. It appears that both genetic and environmental factors play a role in its pathogenesis. Recent studies reveal that in addition to the CNS, immune cells synthesis neurotransmitters so that these catecholamines can regulate immune functions. The aim of this study is to evaluate the dopamine receptor gene expression profiles on peripheral blood mononuclear cells of rheumatoid arthritis patients in comparison with normal individuals.

Material & Methods

In the present study, we investigated dopamine receptor gene expression in PBMCs of 40 RA patients and 40 healthy individuals using Real Time-PCR.The specificities of the obtained Real time PCR products for the respective dopamine receptors fragments were confirmed by sequenced analysis capillary system

Results

We found that DRD1-DRD5 types of dopamine receptors genes expression profiles of rheumatoid arthritis patients differ compared to healthy individuals. Moreover, a significant difference of DR2 and DR4 gene expression was seen in rheumatoid arthritis patients.

Conclusion

This study showed that some types of dopamine receptors genes expression profiles alter in rheumatoid arthritis patients with comparison to healthy individuals Moreover, this alteration possibly could result in dysfunction of dopaminergic system in immune cells and finally lead to rheumatoid arthritis.

Objective

The Lennox-Gastaut syndrome (LGS), one of the most difficult epilepsy syndromes to treat, is characterized by a triad of intractable seizures of various types, a slow (< 2.5-hertz) spike-wave pattern in EEG and mental retardation. The aim of this study was to evaluate the efficacy and safety of lamotrigine as add-on therapy in intractable epilepsy of children with LGS.

Materials & Methods

In a quasi- experimental study, 40 children with LGS referred to the pediatric neurology clinic of Shaheed Sadoughi Hospital in Yazd, between August 2007 and to November 2008, were evaluated.

Results

Twenty-two boys and 18 girls with a mean age of 4.12 ±1.8 years were evaluated. At the end of three months of treatment with lamotrigine, 12 % were seizure free, 52% had> 50% reduction in seizure frequency and 12% had increase in seizures. Means of seizure frequency/per week, before and after treatment were 70 (range 1-180) and 18.6 (range 0-60) respectively, indicating effectiveness of the drug in seizure reduction (P value = 0.003). The drug was effective in 72 % of mixed type seizures, 40 % of generalized tonic-clonic and 33% of drop attack and tonic seizures. Transient side effects were seen in 12.5 % (drowsiness in 3 and ataxia in 2 children). No serious side effects were seen.

Conclusion

Lamotrigine should be considered as an add-on therapy in management of intractable epilepsy in LGS.

Objective

Data on the relationship between iron deficiency anemia and febrile convulsions are controversial. The aim of this study was to determine the association between iron deficiency anemia and febrile convulsions among children.

Materials & Methods

This case-control study was conducted during 2006-2007, on 90 children with febrile seizures (case) and 90 febrile children without seizures (control) referred to the Amirkola children hospital (a referral hospital in the north of Iran). Two groups were matched for age and sex. In all children hemoglobin (Hb) level, hematocrit (Hct), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH) and plasma ferritin (PF) were determined and the data collected were analyzed statistically using the t-test.

Results

The mean PF and TIBC levels were not significantly different in the febrile seizure compared to the reference group; neither were differences in Hb levels statistically significant between two groups. However MCV and MCH were significantly higher in the febrile seizure group (p

Conclusion

Plasma ferritin levels were not significantly lower in children with febrile seizures in comparison with the children in control group. It seems that iron insufficiency does not play a role in pediatric febrile seizures.

Objective

The purpose of this study was to determine the prevalence and the most common risk factors of motor developmental delay in infants.

Materials & Methods

Following ethical approval, a study was carried out on the prevalence and risk factors of infants with motor developmental delay. The first stage was conducted through a cross-sectional study to determine the prevalence of motor developmental delay on 7500 infants and the second stage was an analytic case - control survey to identify the most common risk factors on 140 infants, aged one month to three years with motor developmental delay. Data was collected using a demographic questionnaire, the Parents Evaluation of Developmental Status questionnaire, the Denver Developmental Screening Test II, a neurological assessment form, and the movement and tone assessment form.

Results

The prevalence of motor developmental delay in 7500 infants was 18.7/1000. The most common risk factors in infants with motor developmental delay were prematurity (25.6%), low birth weight (19.2%), neonatal seizures (7.5%), hyaline membrane disease (6.7%), systemic infections of mothers during pregnancy (5.9%), severe neonatal hyperbilirubinemia (5%) in sequence. Motor developmental delay was significantly correlated with consanguinity of parents (p=0.001), prematurity (p=0.046), abnormal head circumference at birth (p=0.038), and low birth weight (p=0.026).

Conclusion

The prevalence of motor developmental delay appears to be high and further studies should focus on different preventive strategies, controlling the most common risk factors and emphasizing on early detection and treatment of high risk infants.

Objective

The Freeman-Sheldon syndrome is a rare congenital myopathy and dysplasia, in which fibrotic contractures of the facial muscles result in the characteristic "whistling face". Difficulties with intubation may be attributed in part to microstomia and micrognathia. In addition to other deformities, limb myopathy results in ulnar flexion contractures of the hand and equinovarus/valgus deformities of the feet. Intravenous access may be difficult because of limb deformities and thickened subcutaneous tissues. Limbs may be encased in plaster casts or splints limiting the available sites for venepuncture. The authors report with a review of literature the case of an infant with Freeman-Sheldon syndrome, which his characteristics was mentioned above.