Brain & development
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Brain & development · Mar 2010
Clinical TrialBilateral pallidal stimulation in children and adolescents with primary generalized dystonia--report of six patients and literature-based analysis of predictive outcomes variables.
Primary generalized dystonia is a rare movement disorder. Medical treatment rarely relieves symptoms. The aim of this study was to investigate the efficacy and safety of bilateral pallidal stimulation in 6 children and adolescents with primary generalized dystonia. In addition, we strived to find predictors for treatment outcome by review and analysis of previously published studies. ⋯ DBS is an effective treatment in children and adolescents with primary generalized dystonia. Predictive factors for a better treatment outcome are DYT1-positive status and minor motor impairment before surgery.
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Brain & development · Feb 2010
ReviewLeukodystrophies and other genetic metabolic leukoencephalopathies in children and adults.
Abnormalities of CNS white matter are frequently detected in patients with neurological disorders when MRI studies are performed. Among the many causes of such abnormalities, a large group of rare genetic diseases poses considerable diagnostic problems. ⋯ The table contains essentials such as age at onset of symptoms, clinical and MRI characteristics, basic defect, and useful diagnostic studies. The table serves as a diagnostic check list.
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Brain & development · Jan 2010
ReviewEpileptic encephalopathy in children possibly related to immune-mediated pathogenesis.
Severe epilepsy in the paediatric population negatively influences neurological and cognitive development. Different etiological factors could be responsible of these severe epilepsies, and an early diagnosis could change, in some cases, the neurological and cognitive development. Immune mechanisms have been reported in epilepsy. ⋯ Among children with acquired symptomatic severe epilepsy, we identified a group of previously normal children who had developed severe partial epilepsy after an acute/sub-acute illness resembling encephalitis. The etiological factors for those patients seems to remain unknown, and a possible immune-mediating or inflammatory process as pathogenesis of the disease could be hypothesized. More studies need to be addressed to finally define this peculiar epileptic entity.
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Although associated factors are important for the occurrence of neural damage in neonatal hypoglycemia, they are not fully understood. Sixty patients with neonatal hypoglycemia were studied through a review of their medical records in Tottori University Hospital. The patients were classified into two main groups: Group I were patients who had mental retardation, developmental delay, cerebral palsy or epilepsy while Group II were those who were normal in their follow-up. ⋯ The following factors were more frequent in Group I than in Group II: toxemia (33.3% and 8.3%, p=0.043), fetal distress (58.3% and 14.5%, p=0.004), an Apgar score of less than 5 at 1 min (33.3% and 6.4%, p=0.025), neonatal seizure (53.8% and 4.3%, p<0.001) and pathological jaundice (41.7% and 6.4%, p=0.006). Cranial CT or MRI revealed cerebral lesions in 8 of the 9 Group I patients in follow-up examinations. This study indicates that severe and prolonged neonatal hypoglycemia can cause cerebral lesions and other perinatal risk factors, such as hypoxia, neonatal seizure and pathological jaundice, would exacerbate hypoglycemic brain injuries.
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Brain & development · Oct 2009
Case ReportsA splenial lesion with transiently reduced diffusion in clinically mild encephalitis is not always reversible: A case report.
The MR imaging finding of a reversible splenial lesion with transiently reduced diffusion has been reported in patients with clinically mild encephalitis/encephalopathy, leading to a new clinical-radiological syndrome, clinically mild encephalitis/encephalopathy with a reversible splenial lesion. We recently experienced a 3-year-old boy with clinically mild encephalitis with a splenial lesion exhibiting transient reduced diffusion on admission. ⋯ Though the splenial lesion decreased in size, it was detected for over 5 months in T2-weighted imaging. It is suggested that a splenial lesion with transiently reduced diffusion in clinically mild encephalitis/encephalopathy is not always reversible, and could result in gliosis.