Journal of child neurology
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The authors conducted this study to identify whether bacille Calmette-Guérin (BCG) vaccination leads to an altered spectrum of neuroimaging findings outcome in pediatric patients with tuberculous meningitis. This retrospective study was conducted through chart review and review of computed tomography (CT) scans and magnetic resonance imaging (MRI) of patients with confirmed central nervous system tuberculosis from the year 1992 to 2005, at a large tertiary care hospital in Karachi, Pakistan. A total of 108 pediatric patients with tuberculous meningitis were included in the analysis. ⋯ There was no difference in terms of severity of clinical presentation and outcome between vaccinated and unvaccinated group. There were no significant differences in CT or MRI findings between the 2 groups except for tuberculomas on MRI, which were significantly higher in the non-bacille Calmette-Guérin vaccinated group (52.2% vs 22.7%, P = .042). Bacille Calmette-Guérin vaccination appears to translate into less tuberculoma formation on MRI.
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Inherited urea cycle disorders represent one of the most common groups of inborn errors of metabolism. Late-onset urea cycle disorders caused by partial enzyme deficiencies may present with unexpected clinical phenotypes. We report 9 patients followed up in our hospital presenting late-onset urea cycle disorders who initially manifested neuropsychiatric/neurodevelopmental symptoms (the most prevalent neuropsychiatric/neurodevelopmental diagnoses were mental retardation, attention-deficit hyperactivity disorder [ADHD], language disorder, and delirium). ⋯ Regarding biochemical data, 2 patients showed normal ammonium but high glutamine levels. This study highlights the fact that neuropsychiatric/neurodevelopmental findings are common among the initial symptomatology of late-onset urea cycle disorders. The authors recommend that unexplained or nonresponsive neuropsychiatric/neurodevelopmental symptoms appearing during childhood or adolescence be followed by a study of ammonia and amino acid plasmatic levels to rule out a urea cycle disorder.
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We assessed the psychosocial and educational consequences of Tourette syndrome using a structured interview and child behavior checklist in 314 children with Tourette syndrome and 81 healthy controls. Of the children with Tourette syndrome, 59.0% needed some kind of educational support, 44.7% had been teased, and 61.8% withheld themselves from taking part in social activities because of Tourette syndrome-related problems. ⋯ A higher rate of these problems was also seen if the comorbidities attention-deficit hyperactivity disorder (ADHD) and/or obsessive compulsive disorder were present. It is very important for the physicians, teachers, and other professionals to be aware of the high prevalence of these social and educational problems to be able to deal with them and to teach the families to cope with them.
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Case Reports
Severe meningoencephalitis due to late reactivation of Varicella-Zoster virus in an immunocompetent child.
Recurrent reactivation of latent Varicella-Zoster virus may cause various neurological complications including encephalitis, myelitis, stroke episodes, and meningitis. It occurs mainly in elderly or immunocompromised patients and is very rare in children. We report a 14-year girl who presented with meningoencephalitis due to reactivation of Varicella-Zoster virus 10 years after she had chickenpox and 4 years after she had zoster. ⋯ Although rare, Varicella-Zoster virus may reactivate to cause significant central nervous system disease even in immunocompetent children. Diagnosis depends on a high degree of suspicion because the typical rash may not associate the disease. Characteristic lesions on MRI and the presence of Varicella-Zoster virus DNA in cerebrospinal fluid are key findings for the correct diagnosis.
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Type II methemoglobinemia is a somatic deficiency of cytochrome b5 reductase with severe global neurologic impairment. We report a novel mutation in exon 3 of the CYB5R3 gene on chromosome 22 consisting of homozygous 1-base pair (bp) deletion noted as c.215delG; p. ⋯ The patient had improvement of gross motor skills, chewing, and swallowing that may be due to the initiation of daily ascorbic acid therapy. We hypothesize that a possible response to ascorbic acid may be related to the effect of making additional ferrous iron available for its role as a cofactor in carnitine synthesis.