Table 6.

Comparison of neuropsychological functional outcomes according to MRI pattern of demyelination following hematopoietic cell transplantation for cerebral X-linked adrenoleukodystrophy




Parietal-occipital lobes, n = 25

Frontal lobes or pyramidal tracts, n = 7

Total, n = 32

No neuropsychological function assessment after HCT because of death, n = 29

P
VIQ*      
   Baseline (range)   99 (59-127)   89 (70-107)  98 (59-127) 92 (74-124) NS 
   After HCT (range)   82 (46-123)   79 (57-113)   80.5 (46-123)   —   —  
   Mean change (CI)  –13.7 (–20.5 to –6.8)  –6.4 (–17.8 to 4.9)   –12.1 (–17.1 to –7.1)   —  .25 
PIQ*      
   Baseline (range)   96 (45-122.5)   92 (61-100)  94.5 (45-122.5) 77.5 (45-138) < .01 
   After HCT (range)   64 (45-122.5)   93 (54-108)   73 (45-122.5)   —   —  
   Mean change (CI)
 
–21.6 (–30.6 to –12.6)
 
0.4 (–6.8 to 7.7)
 
–16.8 (–24.6 to –8.9)
 

 
.03
 



Parietal-occipital lobes, n = 25

Frontal lobes or pyramidal tracts, n = 7

Total, n = 32

No neuropsychological function assessment after HCT because of death, n = 29

P
VIQ*      
   Baseline (range)   99 (59-127)   89 (70-107)  98 (59-127) 92 (74-124) NS 
   After HCT (range)   82 (46-123)   79 (57-113)   80.5 (46-123)   —   —  
   Mean change (CI)  –13.7 (–20.5 to –6.8)  –6.4 (–17.8 to 4.9)   –12.1 (–17.1 to –7.1)   —  .25 
PIQ*      
   Baseline (range)   96 (45-122.5)   92 (61-100)  94.5 (45-122.5) 77.5 (45-138) < .01 
   After HCT (range)   64 (45-122.5)   93 (54-108)   73 (45-122.5)   —   —  
   Mean change (CI)
 
–21.6 (–30.6 to –12.6)
 
0.4 (–6.8 to 7.7)
 
–16.8 (–24.6 to –8.9)
 

 
.03
 
*

VIQ and PIQ baseline evaluations and those after HCT: median (range). Bold denotes statistical comparisons and corresponding P values. VIQ indicates verbal intelligence quotient; PIQ, performance intelligence quotient; CI, 95% confidence interval

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