Journal of Pediatric Neurology - Volume 1, issue 1
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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.
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: The corticospinal and corticobulbar tracts (CST, CBT) are immature at birth, in neuroanatomical terms of myelination and terminal axonal sprouting for multiple synaptic contact; these developmental features are not mature until 2 years of age. Physiologically, the CST is mainly inhibitory. Nevertheless, these pathways have an important role to play in normal neurological function at this age, though different from their functions in the older child and adult. They are important in the neonate by 1)…inhibition of the monosynaptic stretch reflexes at spinal cord levels, beginning at 25 weeks gestation or earlier; 2) influence upon muscle tone, hence posture, by mediating proximal flexion in axial and limb girdle muscles and distal extension of the fingers and toes and abduction of the thumbs; 3) antagonism of the proximal extension and distal flexion and adduction from the medial subcorticospinal pathways of Lawrence and Kuypers; 4) reinforcement of tactile reflexes, the most important of which are suck and swallow; 5)early individualization of finger movements; 6) transmission of epileptic activity from the cerebral cortex. Understanding the unique roles of the CST at birth provide rational, physiological explanations of such neonatal phenomena as clonus, opisthotonus, strong distal flexion and adduction ("cortical thumb") and fisting), and poor suck and swallow in affected neonates, regardless of the cause of cerebral cortical impairment or its reversibility or irreversibility. In summary, the CST and CBT are indeed important functional pathways in the neonate, but many of their functions are very different than in the adult. (J Pediatr Neurol 2003; 1(1): 3-8).
Keywords: corticospinal tract, muscle tone, neonate, post ure, st retch reflexes, suck, swallow, tactile reflexes
Abstract: Behavioral neurology has been bridging the gap between neurology and psychiatry in children. There are several neuropsychiatric disorders of children in which aggression is a dominant symptom. Both global disorders like attention deficit hyperactivity disorder as well as localized dysfunction of the brain may lead to aggression. A number of neurometabolic disorders as well as post-epileptic and postsurgical states may present with aggression in children. Drugs are sometimes effective especially in combination with a…multimode approach. In this review some of the more common causes for aggression in neurologically impaired children, the associated co-morbidities and treatment are discussed. (J Pediatr Neurol 2003; 1(1): 9–14).
Abstract: The aim was to study the prevalence of behavioural and emotional problems among 8-year-old children with intellectual disability (ID) and to compare their results to those obtained among the general child population in the same cohort. In a prospective birth cohort study, parents filled in two questionnaires and teachers assessed children's behaviour using the Rutter scale (RB2). At the age of seven, the Northern Finland Birth cohort 1985/86 included 9357 children, of whom 106 had an…intellectual disability. 44.4% of the children with ID and 14.1% of the children without ID showed probable psychiatric disturbances. In the group of children with ID, behavioural (20.8%) and emotional (18.1%) problems were almost equally common, and hyper-activity problems were frequent (36.1%), whereas in the group of children without ID, behavioural problems (9.1%) and hyperactivity (9.3%) were more common than emotional problems (4.9%). In both groups, boys had more problems than girls, even though the difference was not statistically significant among the children with ID. Over one third of the children with ID had additional disabilities, but these did not increase the risk of having behavioural problems. Because children's psychiatric disorders and behavioural problems are not only very distressing to them and their families but also have a negative impact on their learning at school, peer relationships and social competence, more attention should be paid to preventing them by educational and environmental interventions that support parents and teachers. (J Pediatr Neurol 2003; 1(1): 15–24).
Abstract: We have previously described a technique for the partial bisection of the corpus callosum in the cadaver in order to increase the minimally invasiveness of this procedure. We now present our experience with this procedure in an animal model. Following a 1.0 cm midline skin incision, cranial trephinations (approximately 0.5 cm) were performed in five adult pigs under general endotracheal anesthesia. A rigid endoscope (AESCULAP, Tuttlingen, Germany) was next introduced and used to transect the approximate…anterior two-thirds of the corpus callosum. Following the procedure, brains were observed for gross injury to vascularities or non callosal nervous tissue. No vascular complications were encountered in any of our animal specimens (e.g. superior sagittal sinus, anterior cerebral, frontopolar, or pericallosal arteries). The corpus callosum was easily transected in each specimen. All animals survived the procedure. We believe this technique could provide a less invasive mechanism for patients primarily children, who require corpus callosotomy and will minimize much of the morbidity associated with the traditional methods of sectioning the corpus callosum. Human studies are now necessary to confirm our findings. (J Pediatr Neurol 2003; 1(1): 25–28).
Keywords: minimally invasive, epilepsy, seizure, surgery
Abstract: Several controlled studies have shown that in children with cerebral palsy, botulinum toxin A (BTX/A) can decrease muscular hyperactivity associated with spasticity and improve function. Studies have hitherto focused on the dimensions of impairment and functional limitations. In this pilot study with BTX/A in children with cerebral palsy, we used the Pediatric Evaluation of Disability Inventory (PEDI) to evaluate the effect of treatment. PEDI is a reliable and valid instrument that focuses on assessing disability in…daily life. Patients with cerebral palsy (n = 17, median age 5.5 years, age range 2.5 to 16.5 years) were treated with BTX/A for pes equinus (n = 8) or adductor spasm (n = 9). PEDI assessment was carried out before and 1 month after the first treatment with BTX/A. Scaled scores were calculated according to the user's manual for the Mobility domain with scores near "0" reflecting low capability and scores near "100" reflecting high capability. We found a significant improvement in the mobility domain-caregiver dimension from 52.3% ± 26.6% to 56.6% ± 26.7% (mean, standard deviation P < 0.05), as well as in the self care domain-functional skills from 63.6% ± 18.7% to 65.2% ± 19.6% (mean, standard deviation P < 0.05). Our data indicate that in young patients with cerebral palsy, BTX/A therapy of the lower extremity can reduce the disability. For these patients PEDI is a valuable assessment instrument that reflects the effect of treatment with BTX/A on the disability. (J Pediatr Neurol 2003; 1(1): 29–34).
Keywords: Botulinum toxin A, cerebral palsy, therapy evaluation evaluation, Pediatric Evaluation of Disability Inventory, PEDI
Abstract: The aim of this study is to evaluate serum beta 2-microglobulin and neopterin levels in children with febrile illness and to clarify their relation to influenza and febrile seizures (FS). We studied 34 patients with febrile illness whose age was between 1 and 6 years. Influenza A antigen in the throat swab was positive in 17 patients (Flu group) and was negative in 17 (control group). Serum levels of beta 2-microglobulin and neopterin were evaluated on…admission. FS were observed in 13 patients (6 in Flu group and 7 in control group). Serum beta 2-microglobulin and neopterin levels were higher in Flu group than in control group. On the other hand, there were no significant differences in serum beta 2-microglobulin or neopterin levels between those with and without FS. Higher serum beta 2-microglobulin and neopterin levels in Flu group suggested that stronger activation of immune system was caused by influenza than by infection with other agents. (J Pediatr Neurol 2003; 1(1): 35–38).
Abstract: We report the clinical, electroencephalographic and neuroradiologic findings of a patient with supernumerary der(22) syndrome – partial 22 trisomy, derived from a (11; 22) maternal translocation – and a wide spectrum of cerebral malformation. Magnetic resonance study evidenced a partial midline defect (hypoplasia of the corpus callosum, mild dilatation of the mid portion of the occipital horn of the lateral ventricles and a mild enlargement of the frontal horns) and a malrotation of the body and…the tail of both hippocampi, which present a vertical position. This patient was severely mentally retarded and he was affected by drugresistant focal epilepsy: the seizures were of fronto-temporal origin with possible secondary generalization. The electroencephalographic studies showed a pattern similar to that observed in other cortical malformations. About 30% of the patients with der(22) have cerebral malformation but none presents an hippocampal malrotation. Moreover, this defect seems to be linked to the epilepsy. (J Pediatr Neurol 2003; 1(1): 39–42).
Abstract: Three girls, aged between 7 months and 5 years, were referred with recurrent paroxysmal manifestations since the age of 6 to 10 months. All three had an entirely normal clinical examination, were initially thought to have epileptic fits, their electroencephalogram was unremarkable, and the 'fits' remained refractory to anticonvulsant therapy. They all had stereotypical and reproducible manifestations, with clear evidence of distractibility. Videotaping the manifestations by parents was crucial for the diagnosis. All…three were diagnosed to have autostimulatory behaviour or infantile masturbation. No pathological psychosocial issues were identified in any of the families who accepted the diagnosis with great relief. Infantile masturbation mimics common pediatric problems, and, if unrecognized, may lead to considerable parental anxiety, unnecessary investigations and inappropriate and potentially harmful therapy. (J Pediatr Neurol 2003; 1(1): 43–45).
Abstract: Wilms' tumor is the most common renal tumor of childhood. However, spinal cord involvement has rarely been reported in children with Wilms' tumor. In this article, we report a 5-year-old boy with Wilms' tumor who had the unusual presentation of spinal cord involvement. Our purpose is to emphasize that spinal cord involvement may be seen in children with Wilms' tumor. (J Pediatr Neurol 2003; 1(1): 47–50).