Do children with tuberous sclerosis complex have superior musical skill?--A unique tendency of musical responsiveness in children with TSC.

BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that manifests with symptoms that might include mental retardation, epilepsy, skin lesions, and hamartomas in the heart, brain, and kidneys. Anecdotal reports have characterized children with TSC as having high music responsiveness despite their developmental delay. This study is intended to investigate this putative musical skill of children with TSC and to elucidate the presence of non-delayed facets of their development. MATERIAL/METHODS: This study examined 11 children with TSC: 10 children with DSM-IV autism and 92 healthy children who participated as control subjects. Correlation was examined between results obtained using Non-Verbal MMRC, which is a validated musical responsiveness battery, and results of a scientifically accepted standardized pediatric developmental test: the New Edition of the Kyoto Scale of Psychological Development. Inter-rater reliability among the three raters was also assessed. RESULTS: The rhythm or melody score on the Non-Verbal MMRC and DA among children with TSC showed no significant correlation. In contrast, a significant correlation was found among normal children and those with autism. Moreover, the inter-rater reliability was good. CONCLUSIONS: The results demonstrate that children with TSC show high responsiveness to musical stimuli despite otherwise delayed development (e.g., language, cognition, motor skills). This report is the first stating that children with TSC have a unique tendency in terms of correlation between music and developmental age. These findings indicate a non-delayed area of TSC children's development and suggest the use of music as therapeutic intervention.

Correlation between musical responsiveness and developmental age among early age children as assessed by the Non-Verbal Measurement of the Musical Responsiveness of Children.

BACKGROUND: The currently-available standardized music tests are not suitable for administration to young children and children with special needs because they are complicated and require verbal instructions and verbal responses. A test that was named the Non-Verbal Measurement of the Musical Responsiveness of Children, was developed to assess the musical responsiveness of young children. This test does not depend on verbal instructions, and is composed of two parts, Rhythm and Melody. MATERIAL/METHODS: Ninety-two children [age, range, 6-69 months; 36.39+/-17.61 (mean +/-standard deviation) months] who attended mainstream pre-schools were studied. Each child was tested to see whether the child correctly imitated 7 different patterns of rhythm and 6 different patterns of melody that were delivered by clapping of hands or the voice of the examiner, respectively. The examiner rated whether the child could imitate each pattern and the total score was the sum of successfully reproduced patterns. Two independent observers viewed videotapes of the testing sessions and assigned scores in a similar manner. The inter-rater reliability among the three raters was assessed. RESULTS: The total score in Melody (R=0.63, p<0.001) and the total score in Rhythm (R=0.81, p<0.001) were each correlated with developmental age. The inter-rater reliability was good (Melody: Kendall's W=0.78, Rhythm: Kendall's W=0.95). CONCLUSIONS: The degree of musical responsiveness of normal young children is correlated with general development. This measurement tool is valid and reliable for use in young children who lack sufficient verbal understanding to take standardized music tests. This test may also be administered to children with special needs.

Aortic atherosclerosis and postoperative neurological dysfunction in elderly coronary surgical patients.

BACKGROUND: Arteriosclerosis of the ascending aorta is an important risk factor for cerebral embolism. However, the association between arteriosclerosis of the ascending aorta and neurologic dysfunction after coronary artery surgery has not been evaluated prospectively. METHODS: We examined whether varying degrees of arteriosclerosis in the ascending aorta, assessed by epiaortic ultrasonography, increased the incidence of neuropsychologic dysfunction and stroke in 463 elderly patients (> or = 60 years old) after coronary artery surgery. RESULTS: Patients with severe arteriosclerosis (n = 76) had higher rates of postoperative neuropsychologic dysfunction (26%) and intraoperative stroke (10.5%); the moderately atherosclerotic patients (n = 57) had rates of 7% and 1.8%, respectively; whereas control patients (almost normal or mild arteriosclerosis, n = 330) had rates of 8% and 1.2%, respectively (all p < 0.001). Univariate analysis indicated that multiple small infarctions or broad infarctions, cerebral arterial stenosis, circulatory arrest, maximal thickness of intima around the site of aortic manipulation, and deformities due to clamp or cannulation were associated significantly with intraoperative strokes in patients with severe arteriosclerosis. CONCLUSIONS: Severe arteriosclerosis of the ascending aorta significantly increased the risk of postoperative neuropsychologic dysfunction and stroke after coronary artery bypass grafting. If a thick plaque is noted near the manipulation site, a nontouch method of the ascending aorta should be applied to reduce the incidence of neurologic dysfunction.