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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.
HDLS falls under the category of brain white matter diseases called leukoencephalopathies that are characterized by some degree of white matter dysfunction. HDLS has white matter lesions with abnormalities in myelin sheath around axons, where the causative influences are being continually explored based upon recent genetic findings. Studies by ...
Binswanger's disease, also known as subcortical leukoencephalopathy and subcortical arteriosclerotic encephalopathy, [1] is a form of small-vessel vascular dementia caused by damage to the white brain matter. [2] White matter atrophy can be caused by many circumstances including chronic hypertension as well as old age. [3]
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. [1] [2] On MRI, leukoaraiosis changes appear as white matter hyperintensities (WMHs) in T2 FLAIR images.
These included increased T2-weighted signal abnormalities involving the globus pallidi bilaterally and symmetrically as well as the presence of subcortical white matter. Similar abnormalities have been identified in the brainstem and cerebellar dentate nucleus. [2] Signal intensity on a T2 image may be a result of edema or an
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 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.
Neuroimaging techniques such as Computed Tomography (CT) scan or Magnetic Resonance imaging (MRI) are typically used to detect the presence of degenerative subcortical white matter. [26] Microscopy of the cerebrospinal fluid can also be used for diagnosis, where swollen astrocytes with distorted and elongated mitochondria can be seen in ...