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Hypomyelination with atrophy of basal ganglia and cerebellum
Other Names for this Disease
- Leukodystrophy, hypomyelinating, 6
- Leukodystrophy, hypomyelinating, with atrophy of the basal ganglia and cerebellum
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 These symptoms occur because certain brain cells in individuals with H-ABC are not fully covered by myelin (hypomyelination), a substance that usually surrounds nerve cells to help them work better. Also, this condition causes the breakdown (atrophy) of two parts of the brain that help to coordinate movement - the basal ganglia and cerebellum. The cause of H-ABC is currently unknown.Hypomyelination with atrophy of basal ganglia and cerebellum (H-ABC) is a disease that affects certain parts of the brain. Symptoms usually begin in infancy or early childhood and vary in severity; they include movement difficulties and delay in mental development or learning problems.
Last updated: 3/28/2012
- Schiffmann R, van der Knaap MS. The latest on leukodystrophies. Current opinion in neurology. 2004; 17:187-192. http://www.ncbi.nlm.nih.gov/pubmed/15021247. Accessed 3/27/2012.
- Wakusawa K, Haginoya K, Kitamura T, Togashi N, Ishitobi M, Yokoyama H, Higano S, Onuma A, Nara T, Iinuma K. Effective treatment with levodopa and carbidopa for hypomyelination with atrophy of the basal ganglia and cerebellum. Tohoku Journal of Experimental Medicine. 2006; 209:163-167. http://www.ncbi.nlm.nih.gov/pubmed/16707859. Accessed 3/28/2012.
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- The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Hypomyelination with atrophy of basal ganglia and cerebellum. Click on the link to go to OMIM and review these resources.
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