What are axonal spheroids?
Axonal spheroids are bubble-like biological features that form on most degenerating axons, yet little is known about their influence on degenerative processes.
Is leukoencephalopathy a genetic disorder?
Leukoencephalopathy with vanishing white matter is a genetic condition caused by mutations in any of 5 genes – EIF2B1, EIF2B2, EIF2B3, EIF2B4, and EIF2B5. These genes give the body instructions to make the five parts (subunits) of a protein called eIF2B.
What is hereditary diffuse leukoencephalopathy?
Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a rare autosomal dominant disorder characterized by cerebral white matter degeneration with axonal spheroids leading to progressive cognitive and motor dysfunction.
How long can you live with leukodystrophy?
Children diagnosed with late infantile MLD typically live another five to 10 years. In juvenile MLD, the life expectancy is 10 to 20 years after diagnosis. If the symptoms don’t appear until adulthood, people typically live 20 to 30 years after the diagnosis.
What is pigmented glia?
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a neurological condition characterized by changes to certain areas of the brain. A hallmark of ALSP is leukoencephalopathy, which is the alteration of a type of brain tissue called white matter.
What is adult onset leukoencephalopathy?