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Leukoencephalopathy with vanishing white matter (VWM disease) is an autosomal recessive neurological disease. The cause of the disease are mutations in any of the 5 genes encoding subunits of the translation initiation factor eIF2B : EIF2B1 , EIF2B2 , EIF2B3 , EIF2B4 , or EIF2B5 .
The white matter in preterm born children is particularly vulnerable during the third trimester of pregnancy when white matter developing takes place and the myelination process starts around 30 weeks of gestational age. [3]
While all leukodystrophies are the result of genetic mutations, [3] other demyelinating disorders have an autoimmune, infectious, or metabolic etiology. [4] When damage occurs to white matter, subsequent immune responses can lead to inflammation in the central nervous system (CNS), along with the loss of myelin.
Leukoencephalopathy (leukodystrophy-like diseases) is a term that describes all of the brain white matter diseases, whether their molecular cause is known or unknown. [1] It can refer specifically to any of these diseases: Progressive multifocal leukoencephalopathy; Toxic leukoencephalopathy
The exact cause for the varied collection of symptoms found in the different ALD phenotypes is not clear. The white matter of the brain, the Leydig cells of the testes and the adrenal cortex are the most severely affected systems. [1] The excess VLCFA can be detected in almost all tissues of the body, despite the localization of symptoms. [1]
Cerebellar white matter and dentate nucleus lesions usually occur in children less than ten years of age. [12] Langerhans cell histiocytosis (LCH): LCH is an aggressive disorder due to proliferation of Langerhans cell histiocytes, and the dentate nucleus is believed to be involved in up to 40 percent of patients. [12]
Toxic leukoencephalopathy is a rare condition that is characterized by progressive damage (-pathy) to white matter (-leuko-) in the brain (-encephalo-), particularly myelin, due to causes such as exposure to substance use, environmental toxins, or chemotherapeutic drugs. The prevalence of this disease is infrequent and often goes unreported ...
These children may have excessive vomiting, difficulty swallowing and speaking, poor coordination, and loss of motor control. Adult-onset forms of Alexander disease are less common. The symptoms sometimes mimic those of Parkinson's disease or multiple sclerosis , or may present primarily as a psychiatric disorder .