Pediatric neurology
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Pediatric neurology · Aug 1998
Choreoathetosis after cardiac surgery with hypothermia and extracorporeal circulation.
Eleven children, 4-48 months old, with congenital cyanotic heart defects developed choreoathetoid movements 2-12 days after cardiac surgery with hypothermia and extracorporeal circulation (ECC). The abnormal movements mainly involved the limbs, facial musculature, and tongue, leading to a severe dysphagia. The symptoms had an acute onset, after a period of apparent neurologic normality, and had a variable outcome. ⋯ No specific finding was observed in the CT scans, cerebrospinal fluid examination, or EEG that could be related to the abnormal movements. Symptomatic therapy with haloperidol with or without benzodiazepines led to symptomatic improvement in six children, although there was no evidence that this treatment modified the evolution of the disease. The authors conclude that the choreoathetoid syndrome after cardiac surgery with deep hypothermia and ECC is an ill-defined entity requiring additional study to better understand its pathogenesis so that preventive measures can be taken to avoid a condition that can lead to permanent and incapacitating neurologic sequelae.
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Patterns of cerebral parenchymal injury and their relationship to outcome morbidity are evaluated in this retrospective study of 14 children with confirmed nonaccidental head injury (NAHI). The mean age at time of injury was 12 months 6 days, mean Children's Coma Score was 5.36, and mean postinjury follow-up was 17 months 12 days. All patients had acute subdural hematoma (interhemispheric or convexity) on initial CT imaging. ⋯ Clinical progression of symptoms was confirmed in nine patients (mean Childrens Coma Score was 4.0 +/- 0.33). None had a lucid interval. This is the first study using strict inclusion criteria that documents the range of infarction patterns and potential age-dependent differences in postinjury response cascades after nonaccidental head injury.
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Pediatric neurology · May 1998
Review Case ReportsPseudotumor cerebri in Lyme disease: a case report and literature review.
Pseudotumor cerebri is an unusual presentation of Lyme disease. The case of an 8-year-old girl with pseudotumor cerebri secondary to acute neuroborreliosis is reported. She presented with acute onset of headache, papilledema, sixth nerve palsy, increased intracranial pressure, and cerebrospinal fluid pleocytosis. ⋯ Twelve reported cases that mostly presented with systemic findings and signs of Lyme disease before development of pseudotumor cerebri were reviewed. We conclude that acute neuroborreliosis can present with pseudotumor cerebri as an initial manifestation. It is important to include Lyme disease in the differential diagnosis of pseudotumor cerebri in an area endemic for Lyme disease.
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Pediatric neurology · May 1998
Visual function in children with merosin-deficient and merosin-positive congenital muscular dystrophy.
This study evaluates whether abnormalities of visual function are present in children with congenital muscular dystrophy and whether these, if present, are associated with merosin status or magnetic resonance imaging (MRI) findings. Twenty children (age range 5-17 years) with a diagnosis of classical congenital muscular dystrophy were assessed on visual acuity, stereopsis, and visual fields and the results compared with merosin status and MRI findings. Visual-evoked potential results were available for 14 of 20 children. ⋯ Unlike the other forms of congenital muscular dystrophy, which are associated with structural brain changes and eye involvement, visual function was always normal in the classical form of congenital muscular dystrophy. Interestingly, visual function was normal also in the group of children with merosin-deficient congenital muscular dystrophy who manifested white matter changes involving the occipital lobes on MRI and abnormal visual evoked potentials. Further studies are needed to specify the nature of the white matter changes observed with MRI and the reason for the dissociation between clinical and neurophysiologic findings.
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Pediatric neurology · Apr 1998
ReviewOutcome of children with cerebral edema caused by fulminant hepatic failure.
Mortality is high in patients with fulminant hepatic failure (FHF). Neurologic complications of encephalopathy and cerebral edema are major contributors to mortality. Orthotopic liver transplantation has improved survival in these patients. ⋯ Termination of care is a reasonable option. Comprehensive monitoring of cerebral function and intracranial pressure is required in children with FHF. Orthotopic liver transplantation should be performed in children with severe and worsening encephalopathy before the development of radiographically apparent cerebral edema.