Neuropediatrics
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Randomized Controlled Trial Clinical Trial
The effect of the N-methyl-D-aspartate receptor antagonist dextromethorphan on perioperative brain injury in children undergoing cardiac surgery with cardiopulmonary bypass: results of a pilot study.
Experimental evidence indicates a role of the N-methyl-D-aspartate receptor in the pathogenesis of brain injury occurring during cardiac surgery with cardiopulmonary bypass (CPB). Dextromethorphan is a noncompetitive antagonist of this receptor with a favorable safety profile. Thirteen children age 3-36 months undergoing cardiac surgery with expected CPB of 60 minutes or more were randomly assigned to treatment with dextromethorphan (36-38 mg/kg/day) or placebo administered by naso-gastric tube. ⋯ An increase of periventricular white matter lesions was visible in two placebo-treated children only. No elevations of cerebrospinal fluid enzymes were observed in either group. Although children with dextromethorphan showed less abnormalities in electroencephalography and MRI, dissimilarities of the treatment groups by chance diminished conclusions to possible protective effects of dextromethorphan at this time.
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A strategy for detection of mutations in CLN3, the gene for Batten disease or juvenile onset neuronal ceroid lipofuscinosis, has been devised using a technique which detects conformation polymorphisms and direct sequencing of genomic DNA fragments. We define two mutations found uniquely in Finnish patients, one a large deletion (2.8 kb), the other a point mutation affecting the 5'splice donor site of an intron.
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Case Reports
Coma with abnormal movements and prolonged cognitive disturbances: a new subset of acute encephalopathy.
We describe two children who presented with an acute encephalopathy preceded by a prodromal illness. The disease was marked by an active phase of coma or confusion with abnormal motor movements, followed by a recovery phase with a rapid return of motor function and a gradual improvement in speech and social behavior. No cause was found. These may be additional representative cases of a new syndrome of encephalopathy which is characterized by a distinctive course and a relatively good prognosis.
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Complex febrile convulsions were the initial clinical manifestation of miliary tuberculosis in a 4-year-old immigrant girl. The cerebral lesions were visible only after contrast-enhanced cranial computed tomography (CT) while native CT scan as well as cell count and glucose concentration in the cerebrospinal fluid were normal. ⋯ Treatment with isoniazid and rifampin for 12 months, pyrazinamide for 9 months, and ethambutol for the initial 6 weeks resulted in resolution of the cerebral lesions but a retinal scar after granuloma formation in the right eye caused reduced visus. This case demonstrates the importance of thorough search for tuberculosis even in the absence of overt clinical pulmonary signs especially in high-risk individuals such as immigrants.
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Persistent vegetative state (PVS, apallic syndrome) has become a significant medical and social problem. The outcome of young people with PVS is a matter of great interest. Therefore, we analysed the outcome of 127 children and adolescents who were in PVS for at least 30 days following traumatic (n = 82) or hypoxic (n = 45) brain injury. ⋯ Thirteen patients (16%) with TBI became independent in everyday life versus only two (4%) with HBI. These results underline the important contribution of hypoxia in severe and permanent brain impairment. They also may help to establish the prognosis of children in PVS.