Radiology: MRI, give you a pair of eyes to see the natural history of white matter ablation!

White matter ablation (VWM) is a manifestation of leukodystrophy, in which white matter is the only or most prominent affected tissue

White matter ablation (VWM) is a manifestation of leukodystrophy, in which white matter is the only or most prominent affected tissue

VWM is caused by the double-copy sequence variation of any one of the EIF2B1-5 genes, which encode the five subunits of the eukaryotic translation initiation factor eIF2B

In the past 30 years, MRI has played an important role in the diagnosis of VWM

According to the age of onset, the genetically confirmed VWM patients were stratified into six groups: under 1 year old, 1 year old to under 2 years old, 2 years old to under 4 years old, 4 years old to under 8 years old, 8 years old to under 18 years old, and 18 years old.

This study evaluated a total of 461 examinations in 270 patients (median age, 7 years [interquartile range, 3-18 years]; 144 female patients); 112 patients underwent serial imaging

Figure shows the progression of white matter abnormalities in FLAIR images in patients with different ages of onset

Figure shows the progression of white matter abnormalities in FLAIR images in patients with different ages of onset

This study shows that the pathological type and progression of white matter ablation depend on the age of onset of the disease: the earlier the onset, the greater the cystic changes in the attenuation of white matter, and the faster the progress: the later the onset, the white matter atrophy and glial Hyperplasia is more dominant

Original source:

Menno D Stellingwerff , Murtadha L Al-Saady , Tim van de Brug , et al.

Menno D Stellingwerff Murtadha L Al-Saady Tim van de Brug ,et al.
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