Journal of child neurology
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In recent years, it has been possible for patients with Down syndrome to live longer with advanced medical treatment and social support. As a result, the problems of these patients, such as thyroid diseases, leukemia, and Alzheimer disease, would be encountered more frequently. In this study, we aimed to perform the brain perfusion of children with Down syndrome by technetium 99m hexamethylpropylene amine oxime (99mTc-HMPAO) single-photon emission computed tomography (SPECT) and to determine the relationship between brain perfusion and epilepsy, thyroid function tests, congenital heart disease, and level of mental and motor development. ⋯ When motor and mental development levels were compared, it was found that cases in group 1 were significantly more retarded in personal-social and fine motor skills (P<.05). The present study showed that cerebral hypoperfusion in children with Down syndrome is mostly related to epilepsy and the other coexisting conditions, congenital heart disease and hypothyroidism. Patients with cerebral hypoperfusion also have more retarded developmental levels, especially in personal-social and fine motor skills.
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Following cerebral hemispherectomy, the corticospinal tract is believed to undergo reorganizational changes, which can induce enhanced function of the contralateral motor pathway and mediate partial recovery of motor function. The aim of this study was to use diffusion tensor imaging to investigate the effects of hemispherectomy on the corticospinal tract, with particular attention to the corticospinal tract contralateral to the resection. Diffusion tensor imaging would presumably detect microstructural abnormalities through quantitative measurements of the fiber tract integrity and orientation. ⋯ None of the patients with anatomic hemispherectomy or subtotal hemispherectomy showed significant changes in either apparent diffusion coefficient or fractional anisotropy values in the corticospinal tract contralateral to the resected hemisphere, whereas increased apparent diffusion coefficient and decreased fractional anisotropy were observed in the ipsilateral rostral pons, midpons, and caudal pons of all patients with anatomic hemispherectomy, as well as in the ipsilateral cerebral peduncle of one patient with subtotal hemispherectomy. Increased apparent diffusion coefficient values were also noted in the ipsilateral internal capsule of the same patient. This study revealed no evidence of significant reinforcement of the contralateral corticospinal tract in patients with hemispherectomy, at least from diffusion tensor imaging measurements, but suggests that wallerian degeneration most likely occurs in the ipsilateral motor pathway.
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Bickerstaff brainstem encephalitis is a clinical syndrome of ophthalmoplegia, cerebellar ataxia, and central nervous system signs and is associated with the presence of anti-GQ1b antibodies. There is a clinical continuum between Bickerstaff brainstem encephalitis and Miller Fisher syndrome. We describe the case of an 11-year-old boy with encephalopathy, external ophthalmoplegia, brainstem signs, and ataxia with raised titers of anti-GQ1b antibodies. ⋯ M pneumoniae infection has been associated with both Bickerstaff brainstem encephalitis and Miller Fisher syndrome. This is only the second case in the literature of Bickerstaff brainstem encephalitis with raised titers of anti-GQ1b antibodies described in association with M pneumoniae infection. The patient responded to intravenous immunoglobulin administration.
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Intravenous ketamine, often administered concomitantly with midazolam, is one of the most commonly used drugs for procedural sedation in children. Although it is considered safe for use in children, ketamine can increase intracranial pressure. Hence, this study was carried out to determine whether adding ketamine to midazolam prior to performing lumbar puncture would be associated with a higher cerebrospinal fluid opening pressure in children with suspected aseptic meningitis. ⋯ Mean cerebrospinal fluid opening pressure in group A was 24.4 cm H2O (median 23 cm H2O) compared with 20 cm H2O (median 19.8 cm H2O) in group B (P = .011). Intravenous ketamine prior to performing lumbar puncture is associated with a significantly higher lumbar cerebrospinal fluid opening pressure in children with aseptic meningitis. However, no outcome differences have been found between the two groups.
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Many neurologic manifestations of Epstein-Barr virus (EBV) infection have been documented, including encephalitis, aseptic meningitis, transverse myelitis, and Guillain-Barré syndrome. These manifestations can occur alone or coincidentally with the clinical picture of infectious mononucleosis. Since 1994, The Hospital for Sick Children has maintained a prospective registry of all children admitted with acute encephalitis. ⋯ Most patients with Epstein-Barr virus encephalitis do not show typical symptoms of infectious mononucleosis. Establishing a diagnosis of Epstein-Barr virus encephalitis can be difficult, and, consequently, a combination of serologic and molecular techniques should be used when investigating a child with acute encephalitis. Most children make full recoveries, but residual neurologic sequelae and even death can and do occur.