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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]
Leukoencephalopathy with vanishing white matter; Leukoencephalopathy with neuroaxonal spheroids; Reversible posterior leukoencephalopathy syndrome; Megalencephalic leukoencephalopathy with subcortical cysts. It can also refer to gene MLC1 or Megalencephalic leukoencephalopathy with subcortical cysts 1, a human gene related to the former disease.
These small regions of high intensity are observed on T2 weighted MRI images (typically created using 3D FLAIR) within cerebral white matter (white matter lesions, white matter hyperintensities or WMH) [1] [2] or subcortical gray matter (gray matter hyperintensities or GMH). The volume and frequency is strongly associated with increasing age. [2]
In subcortical dementia, there is targeted damage to regions lying under the cortex. The pathological process that result in subcortical dementia shows neuronal changes that involve primarily the thalamus , basal ganglia , and rostral brain-stem nuclei and mostly, some projections in the white matter from these regions to the cortex, with ...
They do not spread into the subcortical white matter and never show gadolinium enhancement. Over a one-year period, CLs can increase their number and size in a relevant proportion of MS patients, without spreading into the subcortical white matter or showing inflammatory features similar to those of white matter lesions. [29]
"Leukoaraiosis, or periventricular white matter disease, is the result of multiple small-vessel infarcts within the subcortical white matter... The pathophysiologic basis of the disease is lipohyalinosis of small penetrating arteries within the white matter, likely produced by chronic hypertension." [12]
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 ...
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.