low white matter
Introduction
Introduction Less white matter is a manifestation of white matter malnutrition. The white matter of the brain is the place where the nerve fibers gather inside the brain. Because its area is lighter than the surface layer of the brain where the cells are concentrated, it is called white matter. For example, myelin damage of central nervous cells in the white matter of the brain causes leukoencephalopathy.
Cause
Cause
Leukodystrophy refers to a disease caused by genetic factors that affect the normal myelin growth of the central nervous system. Including a variety of genetic diseases caused by white matter myelin abnormalities, such as: lysosomal disease (metaplastic leukodystrophy - MLD, etc.), peroxisome disease (adrenal leukodystrophy - ALD, etc.), mitochondrial disease (Cerebrospinal gastrointestinal disease - MNGIE, etc.), myelin protein-coding gene defects (Pelizaeus-Merzbacher disease), amino acids, organic acidosis (PKU, propionateemia, etc.), and other unexplained leukoencephalopathy (Alexander) Sick, etc.). With the increasing use of MRI technology in pediatric neurology, the diagnosis of white matter lesions has increased significantly. Some of them are known to be genetic or biochemical mechanisms of leukoencephalopathy, which is a category of white matter malnutrition; some are leukoencephalopathy of unknown etiology, some of which are genetic diseases, and also belong to white matter malnutrition, whose genetic and biochemical basis is still Others are due to non-hereditary acquired factors such as immunity, inflammation, and environment, and belong to white matter demyelinating lesions.
Examine
an examination
Related inspection
Brain CT examination brain MRI examination EEG examination brain pool imaging
The clinical manifestations of white matter dystrophy are characterized by gradual progression, and early symptoms are often overlooked. In normal infants or children, changes in muscle tone, posture, exercise, gait, language, eating movements, vision, memory learning, behavioral thinking, etc. can occur gradually. These signs can be gradually aggravated, and the rate of progression of the disease is faster in children.
The main reasons for diagnosis are: 1. Clinical features; 2. Positive family history; 3. Specific biochemical tests; 4. Neuroimaging. Some developed countries have clinical biochemical and pathological morphological diagnosis pathways for leukoencephalic dystrophy in children at several levels: 1. First-line biochemical examination with CSF, cortisol ACTH test; 2. First-line morphological examination with peripheral lymphocytes (or brain) Sediment in living tissue; 3. Second-line biochemical examination includes plasma long-chain fatty acid (VLCFA), urinary sulfatide, leukolysin, etc. 4. Second-line morphological examination has skin, nerve, muscle or brain living specimens; 5. Three-line biochemical examination is a molecular genetic method.
Diagnosis
Differential diagnosis
Lean body white matter: white matter sparse hair occurs in people over 50 years old, common in dementia and cerebrovascular patients. CT features manifested as bilateral white matter symmetry low density lesions, or complicated with cerebral infarction, cerebral hemorrhage and brain atrophy.
