An inherited brain disease that occurs mainly in children. and follows a chronic progressive course with additional episodes of rapid deterioration following stress from febrile infection or minor head trauma.
Leukoencephalopathy with vanishing white matter (VWM) is due to mutations in either of two genes — in either the EIF2B5 gene on chromosome 3q27 or the EIF2B2 gene on chromosome 14q24. These two genes encode the epsilon and beta subunits, respectively, of the translation initiation factor eIF2B. Since eIF2B plays an essential role in the regulation of translation under different conditions, including stress, this may explain the rapid deterioration in persons with VWM under stress.
The disease is also known as childhood ataxia with central nervous system hypomyelinization.
A shortage of white blood cells.
A white spot or patch on the mucous membranes in the mouth (for instance, inside the cheeks, on the gums, on the tongue) that may become cancerous.
A process used to filter and remove white blood cells from whole blood before transfusion. The reason why white blood cells (leukocytes) are removed from blood is because they provide no benefit to the recipient but can carry bacteria and viruses to the recipient. Patients who receive blood that has not been leukoreduced may have […]
One of a group of chemicals produced by the body that accompanies inflammation. Leukotrienes are believed to play a major role in causing the symptoms of hay fever and asthma.
- Level 1 biosafety
Level 1 — This level applies to agents that do not ordinarily cause human disease. Level 2 — This level is appropriate for agents that can cause human disease, but whose potential for transmission is limited. Level 3 — This level applies to agents that may be transmitted by the respiratory route which can cause […]