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We report a young boy who presented with progressive weakness of lower extremities associated with areflexia and abnormal electrophysiological findings initially suggestive of chronic inflammatory demyelinating polyneuropathy. Initial lumbosacral spinal magnetic resonance imaging (MRI) showed thickened descending spinal nerve roots only. Immunomodulating therapy was given but with limited clinical response. Repeated spine magnetic resonance imaging showed cauda equina and also new spinal cord extramedullary contrast enhancement. The initial extensive investigations including open biopsy did not point to any specific diagnosis. Only through pursuing a repeated biopsy, the diagnosis of the spinal peripheral primitive neuroectodermal tumor was confirmed. This case highlights the diagnostic challenges of the spinal peripheral primitive neuroectodermal tumor that could have an initial chronic inflammatory demyelinating polyneuropathy-like presentation. The literature review confirms that this is a rare condition and cauda equina origin has only been reported in adults and teenagers, and this is the first reported case in a young child. [ABSTRACT FROM PUBLISHER]

We studied the efficacy of tongue and body acupuncture in affecting visual recovery in children with central and peripheral visual disorders. Twelve children (five boys, seven girls) (age range 18 months to 14.5 years) with visual disorder with static functional visual ability for at least 12 months were recruited for the study. The causes of cortical visual impairment (10) included severe perinatal asphyxia (4), postencephalitis (1), traumatic brain injury (1), hydrocephalus (1), and increased intracranial pressure (3). Peripheral causes (2) were due to congenital optic atrophy. We used the following assessment tools: clinical visual improvement, defined as improvement of vision by one grade in one or both eyes with measurement of visual acuity; the functional visual outcome scale of 0 to 5, with positive outcome defined as improvement in one level on a functional scale; visual evoked potential, with positive improvement defined as 10% improvement in P100 latency of one or both eyes; [18F]-fluorodeoxyglucose (FDG) positron emission tomography (PET) of the brain, with positive improvement defined as a 10% increase in glucose metabolism in one or both occipital lobes; and the Clinical Global Impression Scale (parental report). Tongue and body acupuncture consisted of 60 sessions, with 5 sessions per week. Four children showed clinical or functional improvement (33%). Of nine children with abnormal visual evoked potentials, five had improvement (56%). Of seven children who underwent PET, six had improvement in glucose metabolism in the visual cortex (86%). Seven parents (58%) reported improvement (three children had 75% improvement; four children had 25% improvement). There was a significant correlation between the interval of onset of visual impairment and starting treatment with clinical or functional outcome, with a longer interval resulting in a better outcome (P= .0282). However, there was no correlation between cause, severity, or clinical or functional visual outcome with improvement in the visual evoked potential or PET. We demonstrated that tongue and body acupuncture can improve the visual status of children with visual disorders, both peripheral and central in origin. As children with chronic visual impairment also showed some visual recovery, more studies should be done to assess the full potential of acupuncture as an adjunct to Western medicine in neuroplasticity. [ABSTRACT FROM AUTHOR]

We recruited 128 neonates with hyperbilirubinemia over a 5-year period (1995–2000) to study the short- and long-term effects of hemolytic hyperbilirubinemia on the auditory brainstem pathway and neurodevelopmental status. These children were divided into two groups: (1) a hemolytic group (n = 29; ABO incompatibility [n = 19], Rh incompatibility [n = 1], glucose-6-phosphate dehydrogenase deficiency [n = 8] and both ABO incompatibility and glucose-6-phosphate dehydrogenase deficiency [n = 1]) and (2) a nonhemolytic group (n = 99). All received phototherapy. Exchange transfusions were performed for four (13.8%) in the hemolytic group and three (3%) in the nonhemolytic group. The brainstem auditory evoked potential was recorded at a mean age of 3.2 months in the hemolytic group and 3.1 months in the nonhemolytic group. Serial brainstem auditory evoked potential assessments were performed until 2 years of age (3 in the hemolytic group and 18 in the nonhemolytic group). All had regular physical, neurologic, visual, and auditory evaluation until 3 years of age. The rate of exchange transfusion was significantly higher in the hemolytic group than in the nonhemolytic group (P < .05). Brainstem auditory evoked potential abnormalities at the initial assessment occurred in three (10.4%) in the hemolytic group (all related to ABO incompatibility) and nine (9.1%) in the nonhemolytic group. At 2 years, the brainstem auditory evoked potential returned to normal except in three cases with a slightly increased hearing threshold (one [3.5%] in the hemolytic group at 60 dB nHL and two [2%] in the nonhemolytic group at 50 dB nHL]). There were no significant differences in the rate of brainstem auditory evoked potential abnormalities at the initial or subsequent assessments between both groups. All except five cases had a normal neurodevelopmental outcome at 3 years (three [two with ABO incompatibility and one with glucose-6-phosphate dehydrogenase deficiency] in the hemolytic group [10.4%] and two [2%] in the nonhemolytic group). All had mild motor delay and hypotonia, which returned to normal at 3 years. The rate of abnormal neurodevelopmental outcome was higher in the hemolytic group than in the nonhemolytic group, although with no significant difference between both groups (P = .08). All five cases in both groups with abnormal neurodevelopment had a normal brainstem auditory evoked potential at the initial assessment. There was no relationship between the abnormal initial brainstem auditory evoked potential and the final neurodevelopmental outcome. The toxic effect of hyperbilirubinemia on the auditory brainstem pathway and neurodevelopmental status in our cohort was transient. The prognosis of neonatal hemolytic hyperbilirubinemia in our Chinese cohort is excellent, possibly owing to an aggressive early-intervention approach. [ABSTRACT FROM AUTHOR]

We aimed to assess the efficacy of tongue and body acupuncture with clinical function and brain glucose metabolism in children with a severe type of cerebral palsy. Four children were recruited. The motor function belonged to grade 5 of the Gross Motor Function Measure (ie, completely nonambulatory). Daily tongue and body acupuncture was applied for 5 days a week for 8 weeks. The Functional Independence Scale for Children (WeeFIM), Clinical Global Impression Scale (CGIS), and positron emission tomography of the brain with [18F]fluorodeoxyglucose (FDG) were performed at baseline and after acupuncture. None of the children had any significant change in the Functional Independence Scale for Children score, despite the fact that all mothers scored 3 on the Clinical Global Impression Scale (ie, 25% improvement) in overall function. The brain glucose metabolism, however, showed a > 10% increase in the frontal, parietal, temporal, and occipital cortices and cerebellum. Thus, a short course of tongue and body acupuncture was shown to increase brain glucose metabolism, despite lacking any clinical functional improvement seen within the 8-week course, possibly owing to the severity of the motor dysfunction and the short duration of treatment. The objective increase in brain glucose metabolism might serve as a surrogate marker for assessing the subclinical efficacy of an alternative treatment before any objective clinical improvement is evident. A larger-scale study for different degrees of severity of cerebral palsy and an impairment model should be undertaken to correlate clinical with neurometabolic change. [ABSTRACT FROM AUTHOR]

We studied the effects of hyperbilirubinemia on brainstem auditory pathways and neurodevelopmental status in 99 full-term neonates with severe nonhemolytic hyperbilirubinemia (total serum bilirubin level = 301 to 500 µmol/L) born between 1995 and 2000. These were divided into three groups: group 1, moderate hyperbilirubinemia (n = 30; mean maximum total serum bilirubin = 320.7 µmol/L or 18.9 mg%); group 2, severe hyperbilirubinemia (n = 63; mean maximum total serum bilirubin = 369.0 µmol/L or 21.7 mg%); and group 3, super hyperbilirubinemia (n = 6; mean maximum total serum bilirubin = 457.2 µmol/L or 26.9 mg%). All received phototherapy, and three neonates also had exchange transfusion. Initial brainstem auditory evoked potentials were recorded in all at the mean age of 3.1 months (range 1-9 months). At initial assessment, only nine neonates (9.1%) had abnormal brainstem auditory evoked potentials. All except two returned to normal at 2 years. These two children had a hearing threshold at 50 nHL. We then compared serial brainstem auditory evoked potentials until 2 years for these nine cases with initial abnormal brainstem auditory evoked potentials, and nine cases with initial normal brainstem auditory evoked potentials were recruited for comparison. All 99 children had regular physical, neurologic, visual, and auditory assessments every 3 to 6 months until the age of 3 years. There was no significant correlation between demographic factors (gender, gestational age, or birthweight), maximum total serum bilirubin, and total serum bilirubin at discharge with an abnormal brainstem auditory evoked potential. There was no significant difference in the rate of brainstem auditory evoked potential abnormalities between the three groups: moderate (10%), severe (7.9%), and super (16.7%). All had normal neurodevelopmental status at 3 years. Only two children had transient mild motor delay and hypotonia, and both had normal brainstem auditory evoked potentials. There was no relationship between the abnormalities of the brainstem auditory evoked potentials and neurodevelopmental status. None of the three children receiving exchange transfusion had abnormal brainstem auditory evoked potentials or neurodevelopmental outcome. With the neurophysiologic and clinical outcomes in our cohort with severe nonhemolytic hyperbilirubinemia, we propose that the toxic effect of hyperbilirubinemia on auditory brainstem pathways might be transient provided that prompt treatment is initiated. [ABSTRACT FROM AUTHOR]

Background. The incidence of pediatric stroke was estimated to be 2.5 to 2.7 cases per 100 000 children per year in North America and 13 cases per 100 000 children per year in France. Stroke is among the top 10 causes of death among children in the United States, with the highest incidence in the first 1 year of life. The annual mortality rate was 0.34 deaths per 100 000 person-years, with an average of 244 deaths per year. Interethnic differences have been demonstrated to be important in pediatric stroke. However, most population-based studies on pediatric stroke were from Europe or North America, and there was a lack of data on the incidence of stroke among Chinese or Asian children. Whether the etiologic patterns and risk factors for death and morbidity among Chinese children with stroke were similar to those described for other ethnic groups was unknown. Objectives. To calculate the incidence of stroke among Chinese children in Hong Kong and to examine the clinical spectrum, causes, patterns, risk factors, and outcomes of pediatric stroke among Chinese subjects. Methods. The population of Hong Kong was 6.7 million in 2001, and >98% of our population is Chinese in origin. In Hong Kong, public hospitals under the Hospital Authority provide >95% of the hospital service for the region. We identified children (>1 month to <15 years of age) who were admitted and given a discharge diagnosis of stroke from the Clinical Data Analysis and Reporting System, which is a centralized computerized database for all public hospitals. The discharge coding of stroke used codes from the International Classification of Diseases, 9th Revision, Clinical Modification. Only first admissions during the study period were included. We excluded any subsequent admissions by using multiple demographic characteristics of the patients. The incidence of pediatric stroke was estimated as the number of first hospitalizations divided by the... [ABSTRACT FROM AUTHOR]

The Hong Kong Children's Seizure and Epilepsy Registry was set up for all children with a history of seizures or epilepsy followed up in the Epilepsy Clinic of the University of Hong Kong. The aim was to study the pattern of seizures and epileptic disorders in Chinese children. The objective was to study the period prevalence of epilepsy and the pattern of epilepsy in Chinese children. A prospective study of seizure and epilepsy in Chinese children was conducted from 1985 to 1997 in Hong Kong. The population census of 1997 was used to calculate the period prevalence of epilepsy in Chinese children. Altogether, 1103 children aged < 19 years at first assessment with epilepsy were included in the study. The period prevalence rate of epilepsy in 1997 (January to December) is estimated to be 4.5 per 1000 children aged < 19 years. The estimated period prevalence rate of children and adolescents with epilepsy in our children is 4.5 per 1000 children. (J Child Neural 2004; 19:19-25). [ABSTRACT FROM AUTHOR]

The objective of this study was to assess the efficacy of botulinum toxin for upper limb spasticity in cerebral palsy. An open-label study was conducted in 11 children with cerebral palsy. Post-botulinum toxin assessment was conducted in weeks 1, 4, and 16, with averaged scores being analyzed. The Clinical Global Impression Scale of the mothers showed marked, moderate, and mild improvement in five, four, and two cases, respectively. The Modified Ashworth Spasticity Scale score showed a statistically significant decrease in the mean spasticity score (P <.003). Other tests were performed depending on the cognition of the child. Increase in joint motion occurred in all five children assessed using web space (P = .043). For the Jebson Hand Function Test, improvement occurred in all five cases assessed (P < .03). Four of five (80%) children could perform tasks that they failed before they were given botulinum toxin. Botulinum toxin is useful in decreasing spasticity and improving the upper limb function of young children with cerebral palsy with normal cognition. Motivated families should be selected with a specific target of using botulinum toxin as an adjunct in a habilitation program. (J Child Neurol 2002;17:138–142). [ABSTRACT FROM AUTHOR]