EURORAD ESR

Case 15689

Biotin thiamine responsive basal ganglia disease

Author(s)
Klemen Mihelcic, Jernej Avsenik

University Medical Centre Ljubljana; Zaloska 7, 1000 Ljubljana, Slovenia; Email: mihelcicklemen@gmail.com
 
Patient
female, 6 year(s)
 
 
  • Figure 1
    Sagital T1

    T1 sagittal images show extensive areas of volume loss involving predominantly subcortical white matter of parietal and occipital lobes as well as the dorsal aspects of superior frontal gyri.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Imaging sequences; Special Focus: Metabolic disorders;
     
     
  • Figure 2
    Sagital T2

    Sagittal T2 images show extensive areas of volume loss and signal change involving cortex as well as subcortical white matter of frontoparietal and occipital region.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Imaging sequences; Special Focus: Metabolic disorders;
     
     
  • Figure 3
    Axial T2

    Axial T2 images show extensive symmetric bilateral atrophy cortically and subcortically in frontoparietal regions.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Imaging sequences; Special Focus: Metabolic disorders;
     
     
  • Figure 4
    Axial T1

    Low intensity signal and volume loss is clearly present in both putamina. Subtle changes are also in present in both thalami. Findings are consistent with atrophy.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Imaging sequences; Special Focus: Metabolic disorders;
     
     
  • Figure 5
    Axial T2

    Hyperintensity with volume loss is visible in putamina and in lateral aspects of both thalami. Subtle hyperintensities can be appreciated in caudate heads. Findings are consistent with chronic sequelae of BTBGD.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Imaging sequences; Special Focus: Metabolic disorders;
     
     
T1 sagittal images show extensive areas of volume loss involving predominantly subcortical white matter of parietal and occipital lobes as well as the dorsal aspects of superior frontal gyri.
 
Sagittal T2 images show extensive areas of volume loss and signal change involving cortex as well as subcortical white matter of frontoparietal and occipital region.
 
Axial T2 images show extensive symmetric bilateral atrophy cortically and subcortically in frontoparietal regions.
 
Low intensity signal and volume loss is clearly present in both putamina. Subtle changes are also in present in both thalami. Findings are consistent with atrophy.
 
Hyperintensity with volume loss is visible in putamina and in lateral aspects of both thalami. Subtle hyperintensities can be appreciated in caudate heads. Findings are consistent with chronic sequelae of BTBGD.
 
 
 
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