OBJECTIVE: The purpose of our study was to compare MRI findings with neurobehavioral development in infants with Krabbe's disease. MATERIALS AND METHODS: Nine infants with Krabbe's disease underwent a total of 19 MR studies during the first year of life as well as tests of mental development, gross motor skills, and fine motor skills (score range: 0-100) within 1 month of imaging. MR scans were scored using the Loes severity scale based on signal abnormality and atrophy, ranging from 0 (best) to 32. We performed three comparisons (Student's t test): each test versus total brain Loes score, fine motor and gross motor tests versus Loes score for the pyramidal tract, and fine motor and gross motor tests versus Loes score for the internal capsule. RESULTS: Mean test results were 65+/-31 for mental development, 48+/-39 for gross motor score, 57+/-35 for fine motor score, and mean total brain score was 7.79+/-6.20. Correlations for total Loes score were -0.78 (p=0.003) for mental development, -0.74 (p=0.003) for gross motor function, and -0.80 (p<0.001) for fine motor function. Correlations for pyramidal system Loes scores were -0.73 (p=0.003) for fine motor function and -0.58 (p=0.028) for gross motor function. Correlation between Loes scores for internal capsule and fine motor function was -0.38 (p>0.05) and between Loes scores for internal capsule and gross motor function was -0.35 (p>0.05). CONCLUSION: The very good correlation between testing results and Loes scores for the entire brain and moderately good correlation between test results and scores for specific brain regions indicate the Loes scoring system likely provides a reasonable means for assessing prognosis and therapeutic response for infants with Krabbe's disease.
OBJECTIVE: The purpose of our study was to compare MRI findings with neurobehavioral development in infants with Krabbe's disease. MATERIALS AND METHODS: Nine infants with Krabbe's disease underwent a total of 19 MR studies during the first year of life as well as tests of mental development, gross motor skills, and fine motor skills (score range: 0-100) within 1 month of imaging. MR scans were scored using the Loes severity scale based on signal abnormality and atrophy, ranging from 0 (best) to 32. We performed three comparisons (Student's t test): each test versus total brain Loes score, fine motor and gross motor tests versus Loes score for the pyramidal tract, and fine motor and gross motor tests versus Loes score for the internal capsule. RESULTS: Mean test results were 65+/-31 for mental development, 48+/-39 for gross motor score, 57+/-35 for fine motor score, and mean total brain score was 7.79+/-6.20. Correlations for total Loes score were -0.78 (p=0.003) for mental development, -0.74 (p=0.003) for gross motor function, and -0.80 (p<0.001) for fine motor function. Correlations for pyramidal system Loes scores were -0.73 (p=0.003) for fine motor function and -0.58 (p=0.028) for gross motor function. Correlation between Loes scores for internal capsule and fine motor function was -0.38 (p>0.05) and between Loes scores for internal capsule and gross motor function was -0.35 (p>0.05). CONCLUSION: The very good correlation between testing results and Loes scores for the entire brain and moderately good correlation between test results and scores for specific brain regions indicate the Loes scoring system likely provides a reasonable means for assessing prognosis and therapeutic response for infants with Krabbe's disease.
Authors: P I Dickson; A R Pariser; S C Groft; R W Ishihara; D E McNeil; D Tagle; D J Griebel; S G Kaler; J W Mink; E G Shapiro; K J Bjoraker; L Krivitzky; J M Provenzale; A Gropman; P Orchard; G Raymond; B H Cohen; R D Steiner; S F Goldkind; R M Nelson; E Kakkis; M C Patterson Journal: Mol Genet Metab Date: 2010-12-02 Impact factor: 4.797