P300 auditory event related potential in children with attention deficit hyperactivity disorder [ADHD]

The overall purpose of this research was to evaluate children with attention deficit hyperactivity disorder [ADHD], investigate P300 auditory evoked potentials in those children and to compare between medicated and non-medicated children. The study included twenty children aged between 7 and 12 years, with primary diagnosis of ADHD, who participated in this study. All subjects had normal hearing, and they were divided into two groups: non medicated group [10 subjects], and medicated group [10 subjects] under methylphenidate treatment [10mg/day]. They underwent detailed history, psychometric assessment, Otoscopic examination, and basic audiological evaluation and P300 evaluation. The twenty children who participated in the study were free from any gross neurological signs. Majority of the children [14 children - 70%] have had normal age appropriate psychomotor development Sixty per cent came from consanguineous families. Seventy five per cent have had smooth past medical history. Half of the included children have had normal scholastic achievement. The mean general intelligence quotient [IQ] among the studied children was 85.35 [S. 0. 9.65]. The mean percentage score of ADHD was 90.95 [5. D. 10.74]. Among 40 ears [20 right and 20 left] 60% of the subjects did not have any P300, 20% from the medicated group and 40% from non-medicated group. From meditated group 8 ears out of 20 ears [40%] did not show P300, right ears [37.5%] and left ear [62.5%]. For non medicated group 16 ears out of 20 ears [80%] did not showed P300, right ears [44%] and left ear [56%]. There were significantly reduced in P300 latency and increased amplitude in medicated group compared with non-medicated. Multivariate analysis between different variables revealed the following statistically significant correlations: verbal reasoning and P300 latency in medicated group, quantitative reasoning and P300 latency in medicated group, general IQ and P300 latency in non-medicated group and quantitative reasoning and P300 latency in non-medicated group. Children with ADHD need to have thorough and comprehensive psychometric assessment because on general psychological examinations, they may function normally. Also, the medicated subjects have more persistence of P300, which showed reduced latency and increased amplitude than non - medicated group. So, auditory cognitive evoked potential [P300] topography may be able to predict robust response to the stimulant medications in children with ADHD

Acute Guillain Barre Syndrome in childhood: clinical, electrophysiological and immunological study

Acute Guillain Barre Syndrome [acute GBS] is one of the leading causes of acute flaccid paralysis [AFP] in children. The syndrome has been recognized as a heterogeneous disorder with different subtypes, by clinical, electrophysiological and pathologic criteria. The disease may present with a wide variety of clinical presentations as well as prognostic outcome. The study was conducted on all children with acute GBS attending Alexandria University Children's Hospital during the period from September 2000 to September 2001 for the purpose of characterization of acute GBS in Egyptian Children and its relation to presumed infection with Cytomegalovirus [CMV] or Epstien-Barr virus [EBV]. All clinical, laboratory and electrophysiological data of the children with acute GBS who are admitted to the hospital during the period of the study were evaluated. Serum IgM titer against CMV and EPV were assessed and correlated with other patients'data. Thirty five children were admitted to the hospital during that period. Sixty age and sex matched healthy children were included as controls for studying IgM titre of EBV and CMV. Affected children were 22 boys and 13 girls. Their ages ranged between one and 10 years. Eight cases did not have preceding illness before the disease. The initial symptom was motor in 11 cases, sensory in 2 cases and mixed in 22 cases. Ascending paralysis was dominant in all cases except three who had descending paralysis. Only five cases did not exhibit severe pediatric GBS [grade 4 or 5] according to the functional grading system. Twenty nine cases exhibited the major electrophysiological type of acute inflammatory demyelinating polyneuropathy [AIDP]. Albuminocytologic dissociation was encountered in 25 cases. Two cases died in the acute phase, one was left with residual neurological deficit while the rest recovered completely. There was a statistically significant difference between cases and controls as regards the IgM titre against CMV virus [P < 0.05] while IgM titres against EBV were not statistically different from the control group. In addition children with presumed Prior CMV infection tended to be younger in their age significantly more than those with presumed EBV infection [r = 0.382, P = 0.003]. The only significant association was the relation between the type of initial symptoms versus the IgM titre against CMV and EBV where sensory symptoms were prominent more significantly among cases with presumed CMV infection than those with presumed EBV infection [P<0.005]. Acute GBS demonstrates diverse clinical, electrophsyiolgical and laboratory dilemma. Clinical and electrophysiological criteria remain the most important prognostic factors. Despite its initial grave presentation, early institution of therapy using intra-venous immunoglobulin [IVIG] or plasma exchange ensures dramatic recovery in most of cases. The underlying etio-pathologic mechanisms are still not properly understood. Their clarification might have its impact on better delineation of clinical presentations, outcome and prevention

Validity of clinical versus genetic study for identification of the fragile X syndrome

Fragile X syndrome is the most common cause of idiopathic X linked mental retardation among boys. It presents several diagnostic problems due to variable clinical features. Inconsistencies also exist in both cytogenetic and molecular markers. This study was conducted on 100 males referred for mental retardation of unknown cause at the Alexandria University Children's Hospital with an age three years and older. The study population was screened for clinical detection of Fragile X syndrome using Ten-item checklist. Patients with positive checklist were subjected to cytogenetic analysis to detect the fragile site on X chromosome and molecular analysis for detection of the altered DNA sequence using polymerize chain reaction [PCR].The patients' ages ranged 3-14 years with a mean of 5.99 +/- 2.68 years. Their IQs ranged 33-85 with a mean of 60.22 +/- 14.05. Out of the 100 males, 22 cases scored positive on the checklist. The most frequent clinical characteristic was hyperactivity [50%] and the least frequent was macro-orchids [2%]. Cytogenic analysis was positive for the fragile site at Xq 27.3 for four cases [18.2%] and negative for 18 cases [81.8%].PCR was positive [more than 200 CGG repeats] for 7 cases [31.8%] and negative for 15 cases [68.2%]. Also, there was a highly significant correlation between the score of the checklist and the positivist of both tests [P=0.01 for each test]. In conclusion, pre-test selection based on clinical criteria is useful to identify high-risk groups and minimizes the cost-effectiveness of both cytogenesis and molecular laboratory testing of fragile X syndrome. Combination of behavioral and physical characteristics is valuable especially in pre-pubertal cases

Giant and absent somatosensory evoked potentials in children with cerebral palsy with and without epilepsy

Many studies have reported the relationship between giant somato-sensory evoked potentials [SEP] and myoclonic epilepsy in adults and children. However there are few reports about giant SEP and absent SEP in children with cerebral palsy. To assess the diagnostic significance of giant SEP and absent SEP in children with cerebral palsy with and without epilepsy. The study included 25 children [10 boys and 15 girls] with cerebral palsy, 10 of them with a history of epilepsy. The age of all the CP children was 5.2 +/- 2.3 years. Ten healthy children of matched age, sex and height were included in the study as a control group. SEP to median [N20] and posterior tibial [P40] nerves stimulation was recorded. Giant SEP was defined as that bigger than 3 SD of the mean amplitude in normal children. Inter-ictal EEG recordings were done for all the patient population as well as for the controls. The peak latency of N20 in normal children was: 16.2 ms +/- 1.1 ms while its amplitude was: 7.5 micro.V +/- 2.6 micro V. The peak latency of P40 in normal children was: 31.3 ms +/- 3.2 ms while its amplitude was: 4.8 micro V +/- 1.8 micro V. Giant SEP was considered if the amplitude was more than 15.3 micro V for N20 and 10.2 micro V for P40. SEP was absent in 5 CP children without epilepsy [33.3%] and a patient [10%] with epilepsy. All of them were severely handicapped. On the other hand 4 CP children with epilepsy [myoclonic seizures] [40%] showed giant SEP while none of the CP children without epilepsy showed giant SEP. EEG recordings were unremarkable in 10 patients [40%]. Abnormal EEG findings were: focal paroxysmal discharges in 5 children [20%], polyspike and slow wave complexes In 4 children [16%] and abnormal background activity in 6 children [24%]. Both groups of CP children [with and without epilepsy] showed significant increase of the SEP latency and significant decrease of the SEP amplitude of the median and posterior tibial nerves compared to the control group. Giant SEP being present in CP children only with epilepsy, suggests that it is related to a state of cortical hyper-excitability. Absent SEP is present in CP children with and without epilepsy, but more in severely handicapped children. So absent SEP is attributed to the severity of neurological disorder

Clinico-epidemiological study of epilepsy in the first three years of life among children attending the Alexandria university Children's hospital during one year

The study is aimed at defining the patterns and possible risk factors of childhood epilepsy in the first three years of life. All children with epilepsy aged 29 days to three years attending Epilepsy Follow up Outpatient Clinic at Alexandria University Children's hospital [Alexandria. Egypt] from January 1st to December 31st 2000 were included. The eligible population was 182. Controls included an equal number of age and sex matched children. The records of children were retrieved and analyzed with a specially designed software program. The study revealed a significant difference between cases and controls regarding family socioeconomic profile, parental consanguinity, and family history of epilepsy, pre-natal and neonatal risk factors and home deliveries. Regarding post-natal factors, meningitis, encephalitis, significant head trauma, status epileptics [febrile or febrile], post-pertussis problems and febrile convulsions were significant. With regard to the epilepsy profile, generalized seizures were more common [80.2%]. Most had a single seizure type [85.7%]. Abnormal neurological findings occurred in 132 children [72.5%]. Abnormal inter-ictal EEG findings were found in 166 cases [91.2%]. Abnormal CT findings were found in 175 cases [87%]. Symptomatic epilepsy accounted for the majority of cases [106 children-58,2%], Epileptic syndromes were identified in 29 children [15.6%]. The most common one was West syndrome. With regard to treatment, the most commonly used drugs were carbamazepine and valproate. Monotherapy was the rule in 86.8%of cases. Regarding partial seizures, secondarily generalized seizures were the most responsive to treatment Absence seizures were the most responsive type of generalized seizures. There are well-defined significant risk factors in the first three years of life. Socioeconomic factors play an important role for the frequency of epilepsy especially in developing countries. Peculiar seizure types, epilepsy types and syndromes are more frequent in this age group

Morphology of cerebral lesions by computed tomography in children with hemiplegic cerebral palsy: correlation with the development of memory and intelligence

The study was initiated to evaluate CT findings and their relation with the development of intelligence and memory in children with hemiplegic cerebral palsy. The study included 25 children: 15 boys and 10 girls. Their ages ranged between 2.7 and 9.6 years. Fifteen cases had right hemiplegia and 10 cases had left sided affection. Congenital hemiplegia was seen in 20 cases and acquired causes occurred in 5 patients. CT scan was normal in 8 children [32%]. CT abnormalities included: maldevelopmental lesions [2 cases: Sturge Weber syndrome, closed lip schizencephaly], non-mal developmental lesions [central hemiatrophy in 4 cases, peripheral hemiatrophy in 7 cases, and 4 combined in 4 cases, calcification in 3 cases]. Fifteen cases had average general intelligence quotient. CT findings were not significantly related to the presence of degree of motor handicapping but was significantly related to the presence of associated deficits. The mean general IQ was not significantly related to the findings on CT findings. However, abnormal CT findings were significantly associated with lower scores on abstract visual and verbal reasoning IQ subsets. In conclusion, though abnormal CT findings were significantly present in hemiplegic CP, their predictive clinical role is only partially relevant in view of the presence of re-organization of motor pathways following this early CNS damage