No to hattatsu. Brain and development
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Initial outcome of intrathecal baclofen (ITB) treatment in Japan is being reported on. ⋯ This is the first report of outcome of ITB for severely disabled children with spasticity in Japan. It was confirmed that ITB for children with severe spasticity is a safe and effective treatment.
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Clinical Trial
[Treatment with ramelteon for sleep disturbance in severely disabled children and young adults].
The aim of this study was to determine the efficacy and safety of ramelteon for severely disabled children and young adults who had already been treated for sleep disturbance with melatonin at a dose of 3 mg. ⋯ This preliminary study showed the efficacy and safety of ramelteon for sleep disturbance in severely disabled children and young adults. Further trials are necessary to determine optimal dosage and safety of ramelteon in children.
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This report describes a male case of subcortical band heterotopia (SBH) with somatic mosaicism of doublecortin (DCX) mutation. His brain MRI revealed bilateral SBH with anterior dominant pachygyria. Although he had infantile spasms from 5-months old and showed mild developmental delay, he responded well to vitamin B6 and ACTH therapy. ⋯ Only the present case showed the mixture pattern of missense mutation (c. 167 G>C) and normal sequence of DCX gene indicating that the present case resulted from somatic mosaicism of de novo DCX mutation. Male patients with DCX mutations generally present with the classical type of lissencephaly, severe developmental delay, and intractable epilepsy. However, somatic mosaic mutation of DCX can lead to SBH in males.