To assess the effect of behavioral manipulation as therapy on the new neural network after brain damage, we studied the effect of skilled training on neurophysiological change in the intact cortex after unilateral damage to the forelimb sensorimotor cortex. Eighteen male Wistar rats (4-5 months) were assigned to 6 groups: (1) rats receiving unilateral forelimb sensorimotor cortex lesion (FSMC lesion) and postoperative training on the skilled task (L-skilled), (2) rats with unilateral FSMC lesion receiving simple repetitive task (L-simple), (3) rats with unilateral FSMC lesion receiving no task (L-non-training), (4)sham-operated rats receiving skilled task, (5) sham-operated rats receiving simple task, (6) sham-operated rats with no task. Rats were anesthized with halothene and followed with Ketamine HCI and electrical lesion was added at FSMC where was confirmed by electrical microstimulation (less than 20 μA). After chronic operations, rats were trained according to each task. Recovery of motor behavior was tested by the symmetry in the use of the forelimbs for upright postural support. Recovery from the asymmetrical use in the forelimbs indicated the recovery period was faster in the L-skilled and L-simple than L-non-training groups. Microstimulation mapping showed forelimb area was expanded in intact cortex of the L-skilled group especially. These findings suggested that therapeutic effect as behavioral manipulation makes neurophysiological change in whole brain, evening the intact side, after unilateral cortical lesion.

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X-linked myotubular myopathy (XLMTM) is a congenital myopathy characterized by severe hypotonia and muscle weakness, and respiratory insufficiency after birth. Surviving patients have prolonged ventilator dependence and severely delayed motor milestones. Myotubularin, which is encoded by MTMI gene, is a new family of putative tyrosine phosphatase and appears to be ubiquitously expressed, with a 3.9 kb transcript detected in all adult and fetal tissues studied. The mutations in XLMTM are occurred in all exons, and the types of them are various. These mutations caused a development of an inactivation of the putative enzymatic activity of myotubularin. We present clinical and molecular analysis of two patients with XLMTM in which we identified mutations of the MTMI gene. ln one patient, an AAAG deletion was identified at the position of nucleotide 193 in coding exon 4. These 4 bp deletions caused a shift of the reading frame and created a stop codon (frameshift mutation). His mother had both normal and mutant alleles heterozygously as a XLMTM carrier. In another patient, a C to T substitution of nucleotide 163, leading Arg 55 to stop codon (nonsense mutation) was identified. His mother, who was an identical twin, had both norma1 and mutant alleles heterozygously as a XLMTM carrier.

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