Leigh Syndrome
Lake N, Bird M, Isohanni P, Paetau A. Leigh Syndrome. Journal Of Neuropathology & Experimental Neurology 2015, 74: 482-492. PMID: 25978847, DOI: 10.1097/nen.0000000000000195.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsBrainElectron Transport Complex IHumansLeigh DiseaseMutationNADH DehydrogenaseConceptsLeigh syndromeAnimal modelsHypoxic-ischemic encephalopathyBilateral symmetrical lesionsCommon pediatric presentationMolecular mechanismsSevere ATP depletionNeuronal preservationPediatric presentationProgressive encephalopathySymmetrical lesionsBasal gangliaCapillary proliferationCerebrospinal fluidUnique pathologyPathogenic mechanismsLesion developmentPatient samplesReactive oxygen speciesGliosisHyperlacticacidemiaEncephalopathyATP depletionSyndromePathology