Pure motor guillain barre among Egyptian infants and children

Guillain Barre syndrome [GBS] is an acute polyneuropathy with a relatively symmetrical weakness and a wide range of severity. It is a heterogeneous disorder which encompasses clinical and electrophysiological subtypes. Variability in the spectrum may be the result of different pathogenic mechanisms. Pure motor GB is one of these variants. If is an acute motor neuropathy with motor weakness without paranesthesia or sensory loss. We studied 9 patients with pure motor GB out of 40 patients with GB to assess the distinctive clinical, electrophysiological, and laboratory findings in this variant in Egyptian children. The results showed that pure motor GB was detected in 22.5% of GB patients during the period from April 2001 till the end of October 2002. The clinical course was preceded by illness during the 4 weeks prior to onset of GB in all patients with pure motor GB [100%] compared with 83.8% in the other GB patients. The cranial nerves were less frequently involved in pure motor GB [11.1%] than in other group [45.2%]. The descending pattern was observed in 33.3% patients with pure motor GB, compared with only 3.2% in other GB patients. However, the mean duration of clinical course until nadir of weakness was not statistically different between the two groups as well as the mean duration of improvement [P: 0.786, P: 0.113 respectively]. As regards the electrophysiological examination, 66.7% of acute motor axonal neuropathy [4 out of 6] had pure motor GB which was statistically significant [X[2]7.897, P: 0.005]. Campylobacter infection was detected in 4 patients of pure motor GB [44.4%] compared with only [12.9%] in other patients [P: 0.05] and Odd's ratio for risk estimate was 5.4 which was statistically significant

Conclusion: pure motor GB in Egyptian children has distinctive clinical, electrophysiological pattern. Ifs association with Campylobacter jejuni infection may explain the increased incidence of this variant in Egyptian children

Study of profile of guillain-barre syndrome among Egyptian children admitted to the Alexandria university children's hospital

Guillain-Barre syndrome [GBS] has become the most common cause of acute flaccid paralysis with virtual elimination of poliomyelitis. It has become apparent that the clinical spectrum of GBS comprises a group of pathological entities, each with distinctive features. The present study was conducted on 40 GBS children who were admitted to Alexandria University Children's Hospital from the start of April 2001 till the end of October 2002. The study finally concluded that: 1. Abnormalities of nerve conduction were the most sensitive and specific finding in GBS. 2. The electrophysiological type which was recorded in majority of the studied cases was acute inflammatory demy elina ting polyradiculo-neuropathy [AIDP], 30 cases [75%]. 3. The most reliable indicators for poor prognosis were respiratory muscle paralysis with the need for ventilation and/or electro diagnostic evidence of markedly reduced amplitude of motor action potentials of less than 20% of the lower limit of normal. 4. General supportive measures still remain important in the management. 5. Specific immunotherapy like I. V. immunoglobulin was found to be effective, particularly if started early within 2 weeks of onset of symptoms. 6. GBS has a good prognosis and mortality rate was about 11% which is similar to that in other studies

Study of Somatosensory Evoked Potentials, Nerve Conduction, Electromyography and Some Serum Trace Elements in Infants and Young Children with Protein Energy Malnutrition

Protein energy malnutrition [PEM] leads to impaired myelination with segmental demyelination or even arrested myelination in severe long-standing malnutrition. While many studies have focused on the morphological and biochemical changes in the nervous system associated with malnutrition, few studies have examined the physiological sequelae of these changes. Moreover, the effects of trace elements deficiencies in PEM on the function of the peripheral nerves and the long myelinated central tracts need to be elucidated in these children. In this study, a group of 15 children with nun edematous malnutrition [marasmus] and a group of 15 children with edematous malnutrition [kwashiorkor and marasmus kwashiorkor] aged 8-36 months were compared to a control group of 15 age and sex matched children. All children under the study were evaluated electro-physiologically using somatosensory evoked potentials [SEPs], motor and sensory nerves conduction and needle electromyography [EMG] studies. Serum levels of zinc, copper, selenium and manganese were assessed. The results showed that both ncm edematous and edematous groups had significant reduction of sensory and motor nerves velocities, amplitude and distal latencies with significant delay in the mean cortical SEP and the mean central conduction time compared to the control group. Neuropathic electromyography findings were present in both groups of PEM in the form of fibrillation potentials, positive sharp waves and large polyphonic motor unit action potentials. Serum levels of copper and selenium were significantly related to the motor and sensory conduction deficits, whereas serum levels of zinc and manganese were significantly related to sensory nerve conduction changes only. Copper and zinc levels were significantly lower in patients represented with neuropathic electromyography changes. There was no significant correlation between the serum levels of any of the studied trace elements and the changes in SEPs

Conclusion: the study demonstrated SEPs ,nerve conduction deficits and neuropathic EMG changes in children with PEM. These deficits may be due to more than one factor including protein deficiency, and trace elements deficiencies. The evidence of the latter is documented in the present study and it seems to contribute to these deficits especially in the peripheral nerves

Pancreatic beta-cells function and anti-insulin antibodies in children with down syndrome

Down syndrome has been linked with many autoimmune disorders. Type 1 diabetes mellitus is more prevalent in Down syndrome. The autoimmune etiology of type 1 diabetes mellitus has been well documented after the discovery of insulin autoantibodies in newly diagnosed diabetics and in individuals at high risk for type 1 diabetes mellitus. The aim of this work was to assess pancreatic beta-cells function and presence of anti-insulin antibodies in children with Down syndrome for possibility of occurrence of pancreatic dysfunction before development of overt diabetes mellitus. The study was carried out on twenty Down syndrome children aged from 4 to 15 years, none of them had history of diabetes mellitus or other autoimmune disease. Ten age and sex matched apparently healthy children with normal karyotyping served as controls. All of them were subjected to full history taking and thorough clinical examination followed by assessment of anti-insulin antibodies with blood glucose and C-peptide levels at fasting state, 2 minutes, 30 minutes and an hour after intravenous infusion of glucose 10% [5 ml/kg body weight] as a challenge test. The results showed that two girls with Down's syndrome had anti-insulin antibodies levels above the cut- off point of anti-insulin antibodies 1> 10.09 u/ml]. The first one, aged 4 years, had fasting blood glucose level 124mg/dl then 140mg/dl one hour after infusion. Her fasting C- peptide level was 0.8ng/ml then 1.ing/ml after one hour of the intravenous glucose tolerance test. The second girl, aged 14 years, had fasting blood glucose 118mg/dl then 130mg/dl one hour after glucose infusion. Her fasting C-peptide was 0.9ng/ml then 1.7 after one hour of the intravenous glucose infusion. Female children had significantly higher blood glucose level at fasting state and one hour after glucose infusion. There was significant proportional correlation between anti-insulin antibodies and blood glucose level at lasting state, two minutes, half an hour and one hour after intravenous glucose infusion and significant inverse correlation were found between it and C-peptide levels at fasting state , two minutes and half an hour after the challenge test

Conclusions and Recommendations: tests to assess anti insulin antibodies should be included in Down syndrome children care and Follow up of Down syndrome children with positive anti insulin antibodies may be of value particularly when therapies become available to preserve beta cell mass. Early detection and consequently early treatment might reduce the burden and complications of diabetes in those children

Neurodevelopmental assessment of children with idiopathic generalized tonic-clonic epilepsy: a short-term prospective study

The respective role of seizures, cerebral lesion, and antiepileptic drugs [AED] on development, cognition and behavior is difficult to identify and despite numerous clinical investigations, this remains questionable. The aim of this research was to assess neurodevelopment in children with newly diagnosed idiopathic generalized tonic-clonic epilepsy before and 6 months after treatment using the most commonly used AED in pediatric population as monotherapy [sodium valproate and carbamazepine] to determine the possibility of cognitive and behavioral effects of these AED. A randomized parallel group study design has been conducted on 40 children with newly diagnosed generalized tonic clonic epilepsy. Their age ranged from 4 to 9 years. All the included children received baseline neurodevelopmental assessment before administration of any AED. Then they were randomly assigned to two groups [20 patients each]. The first group received sodium valproate and the second group received carbamazepine as monotherapy, with follow-up and re-evaluation 6-month after initiation of treatment. Neurodevelopmental assessment of studied children included global developmental assessment using Denver II test, cognitive assessment using Wechsler Intelligence Scales, visual motor assessment using Bender-Gestalt test and behavior assessment using the Pediatric Symptom Checklist. The results showed that there were no significant differences between the valproate and carbamazepine groups on any neurodevelopmental measures neither prior to administration of the study drug nor at 6- month reassessment, except for picture completion score of performance IQ. Within group comparisons, using t-test for related samples determined that valproate group had a significant lowering of full scale IQ on reassessment. Performance IQ scores decreased significantly in both groups at the 6-month reassessment compared to pre-treatment scores in spite of complete control of seizures within the therapeutic dose. The same was observed with Denver I1 test, as the proportion of suspect cases to normal were significantly higher in both groups at 6-month reassessment. Analysis of Pediatric Symptom Checklist results showed that 35% of all patients [5 patients in valproate group and 9 patients in carbamazepine group] had score 2 [problems] before start of any treatment and this was significantly different after 6 months in valproate group only. There was no significant change in Bender-Gestalt score in both groups at 6-month reassessment. Significant relation was observed between lowering in IQ score after treatment and frequent seizures, cases with slow wave and spikes, and generalized epileptiform activity in EEG

Conclusions and Recommendations: the two studied drugs were associated with absolute cognitive and behavioral side effects, although these effects are significantly evident in valproate group. Therefore, the cognitive and behavioral effects of therapies for epilepsy must be an important consideration in the evaluation of risk/benefit ratios directing treatment decisions. Physicians prescribing anticonvulsants should monitor the child behavior, academic progress, cognitive function, and mood, during follow-up visits. Diagnosis of such selective changes early in life is of critical importance, both to provide the affected children with appropriate remedial help in school and in daily life and to protect against the adverse socio-emotional consequences that can impose on the developing child

Serum aluminum level and its relation to serum trace elements in children with attention deficit hyperactivity disorders

Attention deficit hyperactivity disorder [ADHD] is the most common neurobehavioral disorder of childhood. Despite this disorder is increasing in prevalence, it is still controversial and its etiology is not fully known. There is increasing awareness of the role that metals1 minerals play in health and disease. Aluminum is one of the most abundant metals in the environment. It contributes very significantly to nervous diseases by affecting diverse functions, so a clear need exists for active work on the environmental causes of ADHD. This motivated us to carry out this research to find the possible role of aluminum in ADHD and its effect on the level of essential trace elements known to affect behavior and attention in children [iron, zinc and copper]. This study was conducted on twenty children [12 boys and 8 girls] aged 4-8 years diagnosed with attention deficit hyperactivity disorder according to DSM-IV criteria. Twenty age and sex matched normal children were included in the study as control group. All were subjected to psychometric assessment using Conner's rating scale and standard cognitive assessment intelligence scale. Serum aluminum, calcium, iron, zinc, and copper were measured using spectrophotometric techniques. The results proved that patients with ADHD showed statistically highly significant increase in serum levels of aluminum [p= 0.002] and significant lowering of serum calcium [p= <0.001], iron [p= <0.001], and zinc [p=0.01] as compared to controls. Serum levels of copper were lower in ADHD children than controls but the difference was not statistically significant [p=0, 29]. Correlation between studied variables revealed significant proportional correlation between Conner's rating score and serum levels of aluminum and significant inverse correlation between it and serum levels of calcium, iron and zinc. As regards IQ, there was inverse correlation between serum aluminum and total, verbal and performance IQ score and there was significant direct correlation between serum zinc levels and total, verbal and performance IQ. Serum levels of iron were proportionally correlated to both verbal and performance score while calcium was correlated to performance score only. When correlating serum levels of aluminum with calcium and studied trace elements levels, serum aluminum levels were inversely correlated to serum levels of iron, calcium, and zinc. There was no significant correlation between serum levels of aluminum and serum levels of copper. Mean serum level of aluminum was significantly higher in those patients living in industrial areas as compared to those living in non-industrial areas

Conclusions and Recommendations: in the light of present study, we can postulate the significance of increased levels of aluminum in etiopathogenesis of ADHD. The significance of these findings for children health requires better understanding of the amount and bioavailability of aluminum in food, air, drinking water, and medications and from sources unique to infants and children such as vaccination. Exposure to aluminum should be monitored and limited as far as possible and this needs multidisciplinary collaborative research efforts

Effect of erythropoietin, interleukins 3 and 6, and macrophage colony stimulating factor on hemoglobin synthesis in sickle cell anemia

Sickle cell anemia is characterized by significant morbidity and early mortality. The determination of physiologic factors that interact to regulate fetal hemoglobin synthesis is important for the design of strategies to alter globin chain production. This study was conducted to assess serum erythropoietin level, interleukins IL-3, IL-6 and granulocyte macrophage colony stimulating factor [GM-CSF] in children with sickle cell anemia [SCA] during steady state and their relation to fetal hemoglobin in them. The study was also aimed to assess the hypothesis that different set of regulatory might be required to maintain the level of increased hematopoiesis in HFSS and LFSS patients. Thirty children with homozygous SCA in the steady state were involved in the study. Their age ranged from 2-10 years [mean 5.35 +/- 2.55 yr.]. They were divided according to their level of HbF into two groups: 15 patients with HbF >9gm/dl [HFSS] and 15 pateints with HbF <9 gm/dl [LFSS]. Fifteen apparently healthy age and sex matched children served as a control group. All children were subjected to a complete blood count, measurement of fetal hemoglobin and serum level of erythropoietin, IL-3, IL-6, GM-CSF.The results proved that SCA patients had significantly increased levels of EPO, IL-3, IL-6 and GM-CSF than controls. HFSS group had significantly higher levels of IL-3 [69.3 +/- 16.1 pg/ml vs 10.4 +/- 11.3pg/ml] and LFSS group had significantly higher levels of GM-CSF [28.1 +/- 21.3 pg/ml vs 4.73 +/- 6.58 pg/ml] and significantly higher erythropoietin levels [91 +/- 40.8 iu/I vs 24.9 +/- 6.08iu/I]. There was no statistical difference in the level of IL-6 between the two groups

Conclusion: SCA children exhibit differences in the capacity of their mononuclear and endothelial cells to produce cytokines and hematopoietic growth factors according to their peripheral HbF level and GMCSF and iL-3 are differentially involved in the control of SCA depending on the level of hematopoietic stress. We conclude also that elevated levels of cytokines is a characteristic of SCA even in the steady state and it is indicative of ongoing micro-vascular adhesion and severity of the degree of anemia