Other Names for this Disease
- Subacute necrotizing encephalopathy
- Necrotizing encephalopathy infantile subacute of Leigh
- Leigh's necrotizing encephalopathy
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neurodegenerative condition. It usually begins in infancy, often after a viral infection. Rarely, it begins in the teenage or adult years. Signs and symptoms usually progress rapidly and may get worse after a viral infection. Early signs may include poor sucking ability; loss of head control and motor skills; loss of appetite; vomiting; and seizures. As the disorder progresses, symptoms may include weakness and lack of muscle tone; spasticity; movement disorders; cerebellar ataxia; and peripheral neuropathy. Complications can lead impairment of respiratory, heart and kidney function. Leigh syndrome may be caused by changes (mutations) in either mitochondrial DNA or nuclear DNA:Leigh syndrome is a rare, inherited
- Mitochondrial DNA-associated Leigh syndrome follows a mitochondrial inheritance pattern (also called maternal inheritance)
- Nuclear gene-encoded Leigh syndrome may be inherited in an autosomal recessive or X-linked manner, depending on the responsible gene.
Last updated: 2/29/2016
- Leigh's Disease Information Page. National Institute of Neurological Disorders and Stroke. December, 2011; http://www.ninds.nih.gov/disorders/leighsdisease/leighsdisease.htm.
- Thornburn DR, Rahman SR. Mitochondrial DNA-Associated Leigh Syndrome and NARP. GeneReviews. April 17, 2014; http://www.ncbi.nlm.nih.gov/books/NBK1173/.
- Leigh syndrome. Genetics Home Reference. October, 2011; http://ghr.nlm.nih.gov/condition/leigh-syndrome.
- Rahman S & Thorburn D. Nuclear Gene-Encoded Leigh Syndrome Overview. GeneReviews. October 1, 2015; http://www.ncbi.nlm.nih.gov/books/NBK320989/.
- Genetics Home Reference (GHR) contains information on Leigh syndrome. This website is maintained by the National Library of Medicine.
- The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- The Neuromuscular Disease Center at Washington University provides information about Leigh syndrome.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Leigh syndrome. Click on the link to view a sample search on this topic.
- Huntsman RJ, Sinclair DB, Bhargava R, Chan A. Atypical presentations of Leigh syndrome: A case series and review. Pediatr Neurol 2005;32:334-340.