Journal of Pediatric Neurology - Volume 6, issue 3
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The
Journal of Pediatric Neurology is an English multidisciplinary peer-reviewed medical journal publishing articles in the fields of child neurology, pediatric neurosurgery, pediatric neuroradiology, child psychiatry and pediatric neuroscience.
The
Journal of Pediatric Neurology encourages submissions from authors throughout the world. The following articles will be considered for publication: editorials, original and review articles, rapid communications, case reports, letters to the editor and book reviews. The aim of the journal is to share and disseminate knowledge between all disciplines that work in the field of pediatric neurology.
Abstract: Strokes in childhood, although relatively uncommon, have high rates of morbidity and mortality. The clinical findings of ischemic and hemorrhagic strokes are reviewed; both have a significant recurrence risk while the latter carries a particularly high mortality, although outcome in survivors may be better. Neonatal strokes also have to be considered and have variable outcome, although death and recurrence are uncommon. Risk factors for childhood strokes differ from those causing strokes in adults. Cardiac and blood…disorders, for instance congenital heart disease and sickle cell disease, are obvious examples, and now more attention is being given to coagulation and metabolic disorders. Infections, especially those due to viruses such as varicella, can precipitate strokes in children, and there are a number of genetically determined conditions, that can lead to strokes. The latter include blood and metabolic diseases, as well as mitochondrial disorders, which can mimic childhood strokes. Other mimics may also be serious but others are not, such as migraine and syncope. The management of children who have suffered a stroke has to be considered in both the short and long term. For ischemic stroke, management considerations include the use of aspirin, anticoagulants and thrombolytic agents, while hemorrhagic stroke may require emergency neurosurgery and a multidisciplinary team to reduce the risk of recurrence. Rehabilitation requires the involvement of a variety of therapists and needs further study, but assessment of affected children for special educational needs will almost certainly be required.
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Abstract: To obtain basic data for improving genetic counseling, we focused on couples' decision-making with regard to having additional children after notification of their status as a carrier of a reciprocal balanced chromosomal translocation. Carrier status is typically determined after birth of a child with a birth defect derived from a chromosomal unbalanced translocation inherited from the parents' balanced translocation. From April 2004 to December 2004, 15 couples from Kanagawa prefecture of Japan were interviewed. Children…of the couples had unbalanced translocations, and one member of each couple was diagnosed or hypothesized to be a carrier of a reciprocal translocation. Clinical and familial information was obtained by personal interviews in our hospital, and then statistically analyzed. Fifteen couples were interviewed in which the mean age of propositi, fathers, and mothers were 4.7, 31.2, and 31.1 years, respectively. The couples were divided into two groups according to their decision-making process. Each couple that did not want more children already had at least one healthy child before the birth of the propositus. Each couple that did not disclose their own carrier status had a propositus younger than 2 years old. Each couple that had at least one healthy child before the birth of the propositus carrying an unbalanced translocation showed reluctance towards another pregnancy. On the other hand, each couple that did not have healthy children before the birth of the propositus showed strong willingness for a future pregnancy. Each couple that had a propositus younger than 2 years old needed more time to accept the evidence of their carrier status.
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Abstract: The high level of comorbidity between Developmental Coordination Disorder (DCD) and Attention Deficit Hyperactivity Disorder (ADHD) suggests that these disorders may have a shared etiology. We used a co-twin control design to study monozygotic (MZ) twins concordant and discordant for DCD and ADHD. In a total of 922 sets of MZ twins, 866 sets were eligible. We found equal numbers of DCD concordant and discordant sets; more ADHD concordant than discordant sets; nine sets in which…both twins met criteria for DCD+ADHD; 773 sets of twins did not meet criteria for either DCD or ADHD. The only significant sex difference between groups was for ADHD discordant sets, with more males than females. For DCD groups there were no significant sex differences, with slightly more girls than boys affected in both groups. There was a greater number of oxygen perfusion complications in DCD affected than unaffected twins, suggesting that, given equal numbers of DCD concordant and discordant sets and a similar number of DCD girls and boys, the role of pre- and perinatal environmental factors is stronger in the etiology of DCD than in ADHD. Factors such as placental difficulties and hypoxia have also been related to cerebral palsy, which suggests that DCD may fall on the upper end of a continuum of movement disorder that includes cerebral palsy. The results suggest different etiological pathways for DCD and ADHD. Second born twins were at greater risk for oxygen perfusion difficulties in sets concordant for DCD, ADHD, and unaffected for either.
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Abstract: Single photon emission tomography (SPECT) of the brain was studied in 22 children with primary autism with varying degrees of severity. Fifteen children had no perfusion abnormalities on SPECT imaging. Of the remaining seven, decreased perfusion in the left frontoparietal cortex was seen in three, and in the left inferior and medial frontal cortex in two children. No correlation could be found between SPECT, electroencephalography, and magnetic resonance imaging findings. Our findings are similar to those…previously reported in some studies, and present areas of further inquiry regarding therapeutic intervention or diagnosis.
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Abstract: One of the most consistent biological findings in autism is elevated blood serotonin levels. Immune abnormalities, including allergy, are also commonly observed in this disorder. Allergy may play a role in pathogenesis of autism wherein immune responses to allergens may induce the production of brain autoantibodies found in many autistic children. Hyperserotonemia may be the reason behind the increased frequency of allergic manifestations in autistic children through reduction of T-helper 1-type cytokines. Thus, we investigated…the possible connection between hyperserotonemia and the increased frequency of allergic manifestations in 40 autistic and 40 healthy matched children. Autistic children had significantly higher serum serotonin levels than controls [125 (250.75) vs. 41.5 (41.5) ng/mL, P < 0.001]. Fifty five percent (22/40) of autistic children had elevated serum serotonin. Allergic manifestations (bronchial asthma, atopic dermatitis and allergic rhinitis) were elicited in 45% of autistic patients which were significantly higher than controls (10%, P < 0.001). Moreover, autistic patients with allergic manifestations had significantly higher serum serotonin levels than those without (P < 0.001). Furthermore, there was a significant positive correlation between serum serotonin and total immunoglobulin E levels in autistic patients (r = 0.8, P < 0.001). In conclusion, hyperserotonemia may be a contributing factor to the increased frequency of allergic manifestations in some autistic children. Inclusion of serum serotonin levels as a correlate may be useful in future immune studies in autism to help unravel the long-standing mystery of hyperserotonemia and its possible role in the pathophysiology of this disorder. In addition, the effect of blood serotonin lowering drugs in hyperserotonemic autistic children, on amelioration of allergic manifestations and immune abnormalities, should be studied.
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Abstract: Massive intraventricular hemorrhage (IVH) in neonates is followed by progressive ventricular dilatation in 55–80% of cases if the infant survives. The initial mechanism of posthemorrhagic hydrocephalus (PHH) is thought to be obstruction by multiple small blood clots of the channels of the cerebrospinal fluid (CSF) to areas of absorption. Plasminogen activator inhibitor-1 (PAI-1) is the principal regulator of fibrinolysis in blood and one of the most highly controlled of the fibrinolytic components. The aim of this…study is to measure plasminogen and PAI-1 levels in plasma and CSF of the neonates after IVH to assess endogenous fibrinolytic activity and to predict the development of PHH. Fifteen full term and preterm neonates with IVH were enrolled in the study. Ten neonates without IVH were used as a control group. Cranial ultrasound was performed at age of 2 weeks and 2 months. Plasma and CSF plasminogen and PAI-1 levels were assessed for these neonates. CSF PAI-1 was significantly higher in infants with IVH than in the controls (P < 0.001). There was no significant difference in the CSF and plasma plasminogen between infants with IVH and controls (P > 0.05). CSF PAI-1 was significantly higher in infants with PHH than in infants with posthemorrhagic ventricular dilatation (P < 0.05), with a sensitivity (100%) and specificity (100%). CSF PAI-1 is a very sensitive and specific parameter than CSF plasminogen for prediction of PHH in neonates with IVH, and this might be useful to evaluate the specific therapeutic programs of these neonates.
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Abstract: Deficiencies of nutrients such as vitamin B12, folic acid and iron are frequently associated with impairment of memory, concentration and learning ability. Deficiencies of these micronutrients are very rare in Western countries, whereas they are common in developing countries. This study was carried out to determine the impact of vitamin B12 deficiency, folic acid deficiency and/or anemia on the academic achievement of elementary school children from a low socio-economic population, i.e., impoverished Bedouin…population living in southern Israel. Sixty-seven elementary school children, nine to eleven years of age, were randomly tested. Serum levels of vitamin B12, folic acid, and hemoglobin were measured using automated chemiluminescence systems. An individual questionnaire was filled out for each student, which included information on number of meat meals consumed per week, the number of people in the family and information about the father's employment status. Significant positive correlations were observed between number of meat meals consumed per week and low vitamin B12 levels and attainment of low marks in school, respectively. There was a negative correlation between the total number of family members and the attainment of low marks in school. No correlation between anemia or low folic acid levels and school performance was observed. Despite the small sample number, results indicated a high prevalence of vitamin B12 deficiency among these elementary school children, which could be linked to inadequate meat meal intake. This ultimately affected school performance of these children.
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Keywords: Impoverished Bedouin community, school performance, vitamin B12 deficiency
Abstract: Medical records of spinal muscular atrophy type 1 patients, who underwent elective laparoscopic Nissen fundoplication and gastrostomy tube insertion and extubation using a standardized clinical protocol, were retrospectively reviewed. All seven patients were successfully extubated using the protocol and discharged home on bi-level positive airway pressure without oxygen supplementation. There were no major respiratory complications in the post-operative period.
Abstract: Neurocysticercosis is common in children but calcification of falx cerebri is extremely rare. We report a 5-year-old male child with falcine calcification, who was diagnosed as neurocysticercosis based on clinical, epidemiological, serological and imaging findings. The child was treated with albendazole and carbamazepine and seizure free for last 2 years.
Abstract: Two children with refractory seizures had significant weight loss on addition of levetiracetam. Despite improved seizure control, we had to withdraw levetiracetam. Withdrawal of the drug resulted in gradual improvement of weight. Similar findings were reported in adult patients. Although, levetiracetam is generally believed not to affect weight, these recent reports indicate that this drug has the potential of inducing considerable weight loss in both adults and children. Parents need to be warned about this potential…side effect.
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Abstract: Goldenhar syndrome is a rare disorder characterized by a wide spectrum of physical features particularly craniofacial. We report an infant with developmental delay, and seizures who had features of Goldenhar syndrome and had an arachnoid cyst. Though the syndrome itself is not very rare, the presence of arachnoid cyst and hydrocephalus prompted us to report this case. Arachnoid cyst relates to developmental errors in the neural crest and thus presence of arachnoid cyst in the index…case supports the argument of neural migration defect in the pathogenesis in Goldenhar syndrome. Only one such case in the world literature has been reported earlier.
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Abstract: Grisel's syndrome is a rare condition characterized by atlantoaxial subluxation following an inflammatory process in the head and neck region. It occurs more commonly in children and usually presents with cervical pain and torticollis, in addition to symptoms of the primary infection. A case of Grisel's syndrome is discussed here who suddenly developed quadriplegia with severe respiratory compromise and ultimately diagnosed as reducible atlantoaxial subluxation with hematomyelia. Hematomyelia was extending from medulla…to the third cervical vertebrae level of cord. The case was managed successfully by posterior fusion. Though the clinical improvement occurred following posterior fusion, but there was no optimum radiological reduction of the reducible atlantoaxial subluxation. This case emphasizes the importance of posterior fusion in Grisel's syndrome where an offending posterior indentation of upper cervical cord by dislocated posterior arch, if dealt with; the clinical improvement can be achieved even without sufficient anterior reduction of subluxation. The reasons of hematomyelia and nonreduction atlantoaxial subluxation are discussed here.
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Abstract: Situs inversus (also called situs transversus) is a rare congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The association of spinal dyraphism with situs inversus totalis is rare and only few cases have been reported till date. The authors report an infant who presented with meningomyelocele, with split cord malformation type-1 and situs inversus totalis. It is hypothesized that defects of the midline and laterality defects (e.g., situs…inversus) are etiologically related and determining genetic locus or implicating various chemical agents that are known to produce situs inversus independently, as the causative factor for spinal dysraphism would further enhance our understanding about the pathogenesis of the above conditions and hence help in better management. A thorough physical examination supplemented by imaging studies of the entire neuraxis with a high index of suspicion for associated developmental abnormalities in a patient with congenital spinal abnormality is necessary.
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Keywords: Situs inversus totalis, split cord malformation, meningomyelocele
Abstract: Here we describe clinical and cytogenetic data on 2-year-old female child with partial trisomy for the distal part of the long arm of chromosome 10 (10q22–>qter) and a concomitant monosomy 3(p25–>pter) as a result of a maternal balanced reciprocal translocation. Her karyotype was ascertained as 46,XX,der(3)t(3;10)(p25;q22). The father had normal karyotype. The mother had an apparently balanced translocation involving chromosome 3 and 10 [46,XX,t(3;10)(p25;q22)]. This is the second reported case of partial…trisomy 10q and partial trisomy 3p. Clinical features of this case and a few published cases will be reviewed briefly.
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Abstract: Viral encephalitis is often followed by cognitive sequelae including intellectual decline whose pathophysiology remains obscure till date. Based on recent laboratory studies implicating the cholinergic system in viral encephalitis, the authors postulate the hypothesis that cholinergic deficit might account for some of the cognitive sequelae in at least some types of viral encephalitis, which might be benefited by central acetyl cholinesterase inhibitors. The case of an adolescent with postencephalitic intellectual decline responding…robustly to donepezil is presented.
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Keywords: Donepezil, central acetylcholinesterase inhibitors, encephalitis, mental retardation
Abstract: Stroke resulting from traumatic brain injury in children is uncommon. Three such cases are reported here, to bring this entity to attention and to highlight their good prognosis. Possible mechanisms that may lead to stroke in children following head trauma are discussed No other causes of stroke were identified, and magnetic resonance angiography and Doppler ultrasound ruled out injury to the carotid vessels in all three cases.
Abstract: Orbital abscess following trivial blunt trauma to the eye are uncommon. Few cases are known and reported in the literature. The clinical presentation can be myriad varying from localized presentation to children with marked systemic toxicity. Visual loss is the most dreaded complication and a rapid deterioration in vision may occur if the treatment is delayed. Radiological investigations usually offer a prompt diagnosis but considerable degree of confusion may occur, especially in those cases that lack…systemic manifestations of infective pathology. Aggressive medical and surgical treatment is mandatory for a favorable outcome in these cases. We report the case of a child who presented to us with orbital abscess of the left eye following a trivial blunt trauma. No other infective focus was identified. Subsequent imaging revealed abscess of the superior rectus muscle. The child improved following intravenous antibiotics and ultrasonography guided aspiration of the abscess. The clinical and radiological presentation of such cases, management and outcome and a brief review of literature are discussed.
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Keywords: Orbital abscess, superior rectus, blunt trauma, non-axial proptosis, visual loss