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The MRI of patients with VWM shows a well defined leukodystrophy. These MRIs display reversal of signal intensity of the white matter in the brain. Recovery sequences and holes in the white matter are also visible. [4] Over time, the MRI is excellent at showing rarefaction and cystic degeneration of the white matter as it is replaced by fluid.
MRI image: Leukoaraiosis in a 90-year-old patient with cerebral atrophy. Head CT showing periventricular white matter lesions. Leukoaraiosis is a particular abnormal change in appearance of white matter near the lateral ventricles. It is often seen in aged individuals, but sometimes in young adults.
Leukodystrophies are a group of, usually, inherited disorders, characterized by degeneration of the white matter in the brain. [1] The word leukodystrophy comes from the Greek roots leuko, "white", dys, "abnormal" and troph, "growth".
Seizures and delays in motor development are also prevalent. Additionally, mild mental retardation can be observed. Patients often exhibit diffuse swelling of the cerebral white matter and large subcortical cysts in the frontal and temporal lobes, with cysts developing on the tips of the temporal and subcortical areas.
Occasional MRI abnormalities include cortical malformations as polymicrogyria, lissencephaly, pachygyria. [1] [2] [15] [5] In LAMA2-MD there seems to be a directly proportional relationship between the magnitude of white mater and cortical abnormalities on brain MRI and the degree of motor dysfunction in terms of the ability to sit and walk. [15]
Demyelinating lesions begin with the appearance of some areas named NAWM (normal appearing white matter) which despite its name, is abnormal in several parameters. These areas show axonal transections and stressed oligodendrocytes (the cells responsible for maintaining the myelin), and randomly, they show clusters of activated microglia named ...
The presence of incidental MRI findings in the CNS white matter: Ovoid and well-circumscribed homogeneous foci, with or without involvement of the corpus callosum; T2 hyperintensities larger than 3 mm in diameter, which fulfill at least 3 of the 4 Barkhof MRI criteria [7] for DIS; The CNS abnormalities are not consistent with a vascular condition
MRI scans showing hyperintensities. A hyperintensity or T2 hyperintensity is an area of high intensity on types of magnetic resonance imaging (MRI) scans of the brain of a human or of another mammal that reflect lesions produced largely by demyelination and axonal loss.