Journal of Pediatric Neurology - Volume 3, issue 3

Authors: Iannetti, Paola | Mastrangelo, Mario | Di Netta, Sara

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 123-125, 2005

Authors: Dan, Bernard

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 127-129, 2005

Authors: Jan, Mohammed M.S.

Article Type: Research Article

Abstract: Childhood multiple sclerosis (MS) is a rare demyelinating autoimmune disease with different risk factors and clinical features than adult onset MS. Onset of MS is extremely uncommon in early childhood, particularly in those less than 10 years of age. The overall prevalence of MS varies significantly from 1–10 in 100,000 people in Japan to 248 in 100,000 in Canada. At least 5% of all MS patients have their first attack before 16 years of age with a female …to male ratio of 1.4:1. Overall, childhood MS is being increasingly recognized. In this paper, an updated overview of childhood MS will be presented in the context of the available literature and our experience. Research into the earliest events in MS pathogenesis is needed to enhance our information of this disease. As well, understanding the triggers and initial immunologic targets involved may lead to the development of new therapies. Prospective longitudinal studies are required to evaluate the physical, cognitive, and psychosocial impact of childhood MS and the long term benefit of various therapeutic modalities. Show more

Keywords: Multiple sclerosis, child, review, update

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 131-136, 2005

Authors: Bademci, Gulsah | Yucel, Engin | Aliefendioglu, Didem

Article Type: Research Article

Abstract: Metabolic disturbances such as anoxia and hypoglycemia may adversely alter the development of the neonatal brain. While rapid and appropriate diagnosis with adequate therapy has a good prognosis; delayed detection and/or inappropriate therapy increases the risk of the development of irreversible brain damage. Magnetic resonance imaging (MRI) studies are essential in neonatal hypoglycemia to define the characteristics and severity of cerebral lesions after hypoglycemia, to decide the efficacy of preferred treatment modality …and to predict the neurologic outcome. Although acute and long-term radiologic abnormalities associated with hypoglycemic episodes in children and adults are well documented, the details of the situation for neonatal hypoglycemia are still emerging. In this review, the impact of MRI findings of neonatal hypoglycemia on diagnosis, monitoring of treatment and neurologic outcome was discussed. Show more

Keywords: Hypoglycemia, newborn, magnetic resonance imaging, brain damage

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 137-140, 2005

Authors: Wu, Yvonne W. | Liljestrand, Petra | Ferriero, Donna M. | Hayward, Jean | Jeremy, Rita J. | Escobar, Gabriel J. | Newman, Thomas B.

Article Type: Research Article

Abstract: The prevalence of motor examination abnormalities among a general pediatric population is unknown. We determined the frequency of motor abnormalities noted at five years of age during a neurologic examination by a child neurologist. As part of a follow-up study of neonatal jaundice in a population of 106,627 births, we randomly selected 419 five-year-old children as healthy controls, of whom 168 consented. Child neurologists blinded to the children's history performed standard neurologic examinations to determine the …presence of definite or subtle motor abnormalities. Standardized data on motor and cognitive function were also obtained. Among eight (5%) children diagnosed with a definite motor abnormality, the most common finding was corticospinal tract dysfunction (four unilateral, two bilateral). Two children demonstrated hypotonia and hyporeflexia, one of whom also exhibited proximal weakness. An additional 12 (7%) children were noted to have subtle motor findings that were not clearly abnormal, such as subtle incoordination, mild dystonic posturing, tremor, posting, or hyperreflexia. Children with definite or subtle motor findings were more likely to receive an abnormal score on the Motor Performance Checklist (57% vs. 32%, P = 0.02). There was also a trend towards these children having a lower mean verbal IQ compared to children with normal motor examinations (95.3 vs. 102.1, P = 0.07). Motor examination abnormalities in otherwise healthy 5-year-old children may be more common than previously thought. However, the proportion of children with motor examination abnormalities who have an unrecognized neurologic disorder remains unknown. Show more

Keywords: Motor abnormalities, epidemiology, neurologic examination

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 141-146, 2005

Authors: Jan, Mohammed M.S.

Article Type: Research Article

Abstract: Pediatric neurological disorders are common and constitute up to 30% of children seen in general pediatrics. Information about the availability and practice of pediatric neurologists is vital for strategic planning of management of neurological disorders. The aim of this study was to audit current pediatric neurology services in Saudi Arabia and establish a correlation with regional population figures. Physicians practicing in the field of pediatric neurology in Saudi Arabia were identified and contacted. A …structured 17-item questionnaire designed to examine their clinical practice was completed and validated by a follow-up interview. Thirty-two full-time hospital based pediatric neurologists were concentrated in the 3 most heavily populated regions of the country. Saudi Arabia as a whole had a ratio of 0.4 pediatric neurologists per 100,000 children <15 years of age given that 38.3% of the general population was <15 years at the time of the study. Pediatric neurologists had an average of 3 half-day clinics per week with an average of 13.4 patients per clinic. The ratio of follow-up visits to new patients was 3:1. Waiting times for a new non-urgent consultation ranged from 1–6 months (mean 3.4 months). Inpatient hospital admissions ranged from 1–20 (mean 6.5) per week with an average of 5.7 in-hospital consultations per week. Major deficiencies were identified in the availability of specialized nurses, occupational therapists, and psychologists. The study documents a significant shortage of pediatric neurologists in our country. The currently practicing neurologists are required to see many patients with long waiting lists and inadequate support services in many institutions. I feel that a strong demand exists to train and recruit more pediatric neurologists and that generalists and pediatricians need to continue to take a proactive role in the routine care of children with neurological disorders in Saudi Arabia. Show more

Keywords: Pediatrics, child, neurology, audit, practice, Saudi Arabia

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 147-152, 2005

Authors: Adewuya, Abiodun O. | Ola, Bola A. | Okeniyi, John A.O.

Article Type: Research Article

Abstract: To investigate the rate and type of psychiatric diagnosis in a group of Nigerian adolescents with epilepsy. Adolescents with epilepsy (n=166) aged 12 to 18 years and healthy controls (n=170) and their adult caretakers were interviewed with the youth and parent versions of the Diagnostic Interview Schedule for Children version IV (DISC IV). The rate of any psychiatric disorder in adolescents with epilepsy is 65.1%; any anxiety disorder is 33.1%; any depressive disorder is …30.1% and any disruptive disorder is 25.9%. Adolescents with epilepsy have significant higher risk for generalized anxiety disorder, separation anxiety disorders, major depressive disorders, dysthymia, attention deficit hyperactivity disorder and conduct disorder than healthy controls. Psychiatric morbidity is mainly associated with frequency of seizures (P < 0.001) and number of antiepilepsy drugs (P < 0.001). Nigerian adolescents with epilepsy have a higher rate of a wide variety of psychiatric disorders than their healthy counterparts. Interesting associations with conduct disorders have been found in our Nigerian sample. All these should be taken into account when planning management of these youth. Show more

Keywords: Adolescents, childhood, psychiatric morbidity, epilepsy, anxiety, depression, disruptive disorders, cross-cultural

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 153-158, 2005

Authors: Campos, José Guevara | de Guevara, Lucía González

Article Type: Research Article

Abstract: We analyzed the treatment of 25 children with infantile spasm, 52% were males and 48% females. Of the cases studied 80% were symptomatic and 20% cryptogenic. Twenty-three children were treated with synthetic adrenocorticotropic hormone (ACTH), in 15 children the ACTH was administered with antiepileptic drugs and two children received vigabatrin. In the symptomatic cases of West syndrome, we found hypoxic-ischemic encephalopathy, tuberous sclerosis, brain malformations and Down syndrome. ACTH was efficacious in …symptomatic infantile spasms and in cryptogenic spasms. The side effects most frequently found were: irritability, edema, Cushingoid obesity (moon face), hypertension and hypokalemia. Vigabatrin was used in patients with tuberous sclerosis and was efficacious in the control of infantile spasms. Infantile spasms should be treated as soon as the diagnosis is made. ACTH is still first-choice treatment in patients with West syndrome. The drug of choice of the treatment of infantile spasms in children with tuberous sclerosis is vigabatrin. Show more

Keywords: Infantile spasms, hypsarrhythmia, seizures, mental retardation, epilepsy, ACTH

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 159-163, 2005

Authors: Pisani, Francesco | Scarano, Angela

Article Type: Research Article

Abstract: Homozygosis for the thermolabile variant (C677T) of the methylene-tetrahydropholate reductase (MTHFR) gene has been related to a high incidence of neural tube defects, pre-eclampsia, retardation in fetal growth and hyperhomocysteinemia. The latter represents a known risk factor for thrombosis. We report the case of a newborn with severe cerebral damage and neonatal epilepsy that needed treatment with antiepileptic drugs. The mother was to be found homozygote for the C677T variant of the MTHFR gene and the …child was to be found heterozygote for the same mutation. We underline the possible correlation between the fetal-perinatal adverse events and the mother's genetic metabolic risks. Show more

Keywords: C677T MTHFR gene mutation, risk factors, cerebral damage, homocysteine, metabolic disorders, thrombophilia

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 165-168, 2005

Authors: Shaharao, Vijaya B. | Agarwal, Rinku | Muranjan, Mamta N. | Bavdekar, Sandeep B.

Article Type: Research Article

Abstract: An 18-month-old infant who presented with delayed motor development, hypotonia and absent deep tendon reflexes and normal cognitive development was diagnosed to have merosin-positive congenital muscular dystrophy (MP-CMD) on the basis of raised serum levels of creatine kinase, features suggestive of myopathy on electrophysiological studies, dystrophic muscle pathology and normal immunohistochemistry for merosin (laminin-α2). Neuroimaging studies demonstrated white matter hyperintensities on T_2 -weighted images similar to that seen in patients with …merosin negative (MN-CMD). Electrophysiological studies also demonstrated features of axonal involvement. This finding has not been previously described in association with MP-CMD. Although we have not been able to exclude abnormal glycosylation of α-dystroglycan, this case expands the clinical phenotype of MP-CMD and suggests that neuroimaging of children with CMD may be useful in the identification of variants of MP-CMD. Show more

Keywords: Floppy infant, leukodystrophy, congenital muscular dystrophy

Citation: Journal of Pediatric Neurology, vol. 3, no. 3, pp. 169-172, 2005