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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.
Leukoencephalopathy with neuroaxonal spheroids (LENAS) is believed to occur when presence of white matter degeneration and axonal spheroids are seen with a brain biopsy or an MRI. [6] [3] White matter consists of nerve fibers covered by a substance called myelin that insulates and protects them. [3]
The degeneration of white matter can be seen in an MRI scan and is used to diagnose leukodystrophy. Leukodystrophy is characterized by specific symptoms, including decreased motor function, muscle rigidity, and eventual degeneration of sight and hearing. While the disease is fatal, the age of onset is a key factor, as infants have a typical ...
The white matter with hidden but MRI-visible damage is known as "Normal-appearing white matter" (NAWM) [136] and is where lesions appear. [22] The NAWM is considered a non-visible kind of lesion, produces disability and it is responsive to natalizumab [137] The pathology of the NAWM differs from areas near the lesions or near the cortex.
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 ...
The damaged white matter is known as "Normal-appearing white matter" (NAWM) and is where lesions appear. [10] These lesions form in NAWM before blood–brain barrier breakdown. [64] BBB can be broken centripetally (the most normal) or centrifugally. [65] Several possible biochemical disrupters were proposed.
The cause of spongy degeneration of the CNS is the mutation in a gene coding for aspartoacylase (AspA), an enzyme that hydrolyzes N-acetyl aspartic acid (NAA). [6] In the absence of AspA, NAA accumulates and results in spongy degeneration. [7] The exact pathophysiological causes of the disease are currently unclear, but there are developing ...
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.