EURORAD ESR

Case 15037

Methylmalonic Acidemia (MMA) in a child

Author(s)
Eliza Stavride, Katerina Manavi, Melpomeni Kosmidou, Charikleia Mavridou, Filippos Sarafis, Ioannis Tsitouridis

Papageorgiou General Hospital of Thessaloniki, Radiology; Pavlos Melas Street 56429 Thessaloniki, Greece; Email:elizasta@hotmail.com
 
Patient
male, 6 year(s)
 
 
  • Figure 1
    T2-weighted images
     

    Axial T2-weighted image showing hyperintensity in bilateral lentiform nuclei.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;

    Axial T2-weighted image showing hyperintensity in bilateral cerebral penducles.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;

    Coronal T2-weighted image showing hyperintensity in the basal ganglia.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;
     
     
  • Figure 2
    FLAIR Images
     

    Axial FLAIR image depicting hyperintensity in the basal ganglia.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;

    Axial FLAIR image showing hyperintensity in the brainstem.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;
     
     
  • Figure 3
    Gadolinium-enchanced T1-weighted images
     

    Axial gadolinium-enhanced T1-weighted image showing mild peripheral enhancement of the lentiform nuclei.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;

    Coronal gadolinium-enhanced T1-weighted image showing peripheral enhancement of the lentiform nuclei.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;
     
     
  • Figure 4
    Diffusion images
     

    Isotropic Diffusion (b value=1000) showing no restriction of diffusion since the lesions are not acute.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR-Diffusion/Perfusion; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;

    ADC map showing hyperintense lesions in globus pallidi, sign of chronicity.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: MR-Diffusion/Perfusion; Procedure: Diagnostic procedure; Special Focus: Metabolic disorders;
     
     
  • Figure 5
    Citric Acid Cycle

    An image explaining pathophysiology of MMA.

     
    Area of Interest: Neuroradiology brain; Imaging Technique: RIS; Procedure: Education; Special Focus: Metabolic disorders;
     
     
Axial T2-weighted image showing hyperintensity in bilateral lentiform nuclei.
 
Axial T2-weighted image showing hyperintensity in bilateral cerebral penducles.
 
Coronal T2-weighted image showing hyperintensity in the basal ganglia.
 
Axial FLAIR image depicting hyperintensity in the basal ganglia.
 
Axial FLAIR image showing hyperintensity in the brainstem.
 
Axial gadolinium-enhanced T1-weighted image showing mild peripheral enhancement of the lentiform nuclei.
 
Coronal gadolinium-enhanced T1-weighted image showing peripheral enhancement of the lentiform nuclei.
 
Isotropic Diffusion (b value=1000) showing no restriction of diffusion since the lesions are not acute.
 
ADC map showing hyperintense lesions in globus pallidi, sign of chronicity.
 
An image explaining pathophysiology of MMA.
 
 
 
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