Journal of child neurology
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This study was conducted to evaluate the feasibility of cerebral perfusion pressure-targeted therapy in children with raised intracranial pressure caused by central nervous system infection. A prospective observational pilot study was conducted in the pediatric intensive care unit of a tertiary care teaching hospital. Twenty children (ages 6 months to 12 years) with a clinical diagnosis of meningitis or meningoencephalitis were included. ⋯ All 4 patients with mean cerebral perfusion pressure less than 50 mm Hg died of intractable, raised intracranial pressure. In contrast, only 3 of 16 patients with mean cerebral perfusion pressure more than 50 mm Hg died. In children with raised intracranial pressure caused by central nervous system infection, it was feasible to achieve a cerebral perfusion pressure greater than 50 mm Hg, mainly by increasing the blood pressure within the first 24 hours and by reducing intracranial pressure after the first 24 hours.
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Autosomal recessive spinal muscular atrophy with respiratory distress type 1 (SMARD1), recently referred to as distal spinal muscular atrophy 1 (DSMA1; MIM#604320) and also known as distal hereditary motor neuropathy type 6 (dHMN6 or HMN6), results from mutations in the IGHMBP2 gene on chromosome 11q13.3 encoding the immunoglobulin micro-binding protein 2. In contrast to the infantile spinal muscular atrophy type 1 (SMA1; Werdnig-Hoffmann disease) with weakness predominantly of proximal muscles and bell-shaped thorax deformities due to intercostal muscle atrophy, infants with distal spinal muscular atrophy 1 usually present with distal muscle weakness, foot deformities, and sudden respiratory failure due to diaphragmatic paralysis that often requires urgent intubation. In this article, the authors review the clinical, neuropathological, and genetic aspects of distal spinal muscular atrophy 1 and discuss differential diagnoses.
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Transcranial magnetic stimulation (TMS) is a method for focal brain stimulation based on the principle of electromagnetic induction, where small intracranial electric currents are generated by a powerful, rapidly changing extracranial magnetic field. Over the past 2 decades TMS has shown promise in the diagnosis, monitoring, and treatment of neurological and psychiatric disease in adults, but has been used on a more limited basis in children. ⋯ The potential for applications of TMS in child neurology and psychiatry is significant. Given its excellent safety profile and possible therapeutic effect, this technique should develop as an important tool in pediatric neurology over the next decade.
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The object of this study was to investigate the epidemiologic pattern of autism spectrum disorder in Chinese children. An autism spectrum disorder registry has been established in Hong Kong since 1986 by collecting data in a single center (the only university-affiliated child assessment center in Hong Kong). Since 1997, inpatient data from all public hospitals under the Hospital Authority have been stored in a central computerized program and retrieved from the Clinical Data Analysis and Reporting System. ⋯ The male to female ratio was 6.58:1. This is the first reported epidemiologic study of autism spectrum disorder in Chinese children. The incidence rate is similar to those reported in Australia and North America and is lower than Europeans.
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A rare case of delayed lateral rectus palsy in a patient following resection of a pineal lesion in the sitting position is presented. Postoperative pneumocephalus is common following craniospinal surgical intervention in the sitting position. The sixth cranial nerve is frequently injured because of its prolonged intracranial course. ⋯ Even rarer is the report of this anomaly following craniotomy in the sitting position. This patient's symptoms manifested in a delayed fashion. Although uncommon, this complication should be considered in patients undergoing cranial or spinal surgical interventions in this position.