Published in Epilepsia on January 01, 2007
Mitochondrial energetics and therapeutics. Annu Rev Pathol (2010) 3.07
The neuroprotective properties of calorie restriction, the ketogenic diet, and ketone bodies. Brain Res Rev (2008) 2.39
The ketogenic diet as a treatment paradigm for diverse neurological disorders. Front Pharmacol (2012) 1.61
Neuroimaging of mitochondrial disease. Mitochondrion (2008) 1.43
Mitochondrial Dysfunction Plus High-Sugar Diet Provokes a Metabolic Crisis That Inhibits Growth. PLoS One (2016) 1.38
A modern approach to the treatment of mitochondrial disease. Curr Treat Options Neurol (2009) 1.32
Ketones prevent synaptic dysfunction induced by mitochondrial respiratory complex inhibitors. J Neurochem (2010) 0.99
Ketogenic diets in patients with inherited metabolic disorders. J Inherit Metab Dis (2015) 0.93
Treatment of mitochondrial disorders. Curr Treat Options Neurol (2014) 0.90
How can we treat mitochondrial encephalomyopathies? Approaches to therapy. Neurotherapeutics (2008) 0.89
Gastrointestinal dysfunction in autism spectrum disorder: the role of the mitochondria and the enteric microbiome. Microb Ecol Health Dis (2015) 0.89
Ketogenic diet in neuromuscular and neurodegenerative diseases. Biomed Res Int (2014) 0.89
Development of pharmacological strategies for mitochondrial disorders. Br J Pharmacol (2014) 0.87
Potential therapeutic use of the ketogenic diet in autism spectrum disorders. Front Pediatr (2014) 0.83
The elusive magic pill: finding effective therapies for mitochondrial disorders. Neurotherapeutics (2013) 0.83
Neuroprotection in metabolism-based therapy. Epilepsy Res (2011) 0.82
Mitochondrial disease in childhood: nuclear encoded. Neurotherapeutics (2013) 0.82
Mitochondrial vulnerability and increased susceptibility to nutrient-induced cytotoxicity in fibroblasts from leigh syndrome French canadian patients. PLoS One (2015) 0.82
Nutritional interventions in primary mitochondrial disorders: Developing an evidence base. Mol Genet Metab (2016) 0.81
Neurodevelopmental manifestations of mitochondrial disease. J Dev Behav Pediatr (2010) 0.81
Modified Atkins diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathy patients. EMBO Mol Med (2016) 0.81
Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy. Yonsei Med J (2010) 0.80
Mitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function. Mitochondrion (2015) 0.80
Therapies in inborn errors of oxidative metabolism. Trends Endocrinol Metab (2012) 0.78
Mitochondrial-associated metabolic disorders: foundations, pathologies and recent progress. Nutr Metab (Lond) (2013) 0.77
Ketogenic diets improve behaviors associated with autism spectrum disorder in a sex-specific manner in the EL mouse. Physiol Behav (2016) 0.77
Metabolic manipulators: a well founded strategy to combat mitochondrial dysfunction. J Inherit Metab Dis (2010) 0.76
Use of the Ketogenic Diet to Treat Intractable Epilepsy in Mitochondrial Disorders. J Clin Med (2017) 0.75
The use of ketogenic diet in special situations: expanding use in intractable epilepsy and other neurologic disorders. Korean J Pediatr (2012) 0.75
Arg! Post-translational modifications in mitochondrial proteins after status epilepticus. Epilepsy Curr (2013) 0.75
Mutations in MDH2, Encoding a Krebs Cycle Enzyme, Cause Early-Onset Severe Encephalopathy. Am J Hum Genet (2016) 0.75
Mendeliome sequencing enables differential diagnosis and treatment of neonatal lactic acidosis. Mol Cell Pediatr (2016) 0.75
Inborn Errors of Metabolism and Epilepsy: Current Understanding, Diagnosis, and Treatment Approaches. Int J Mol Sci (2017) 0.75
Novel acquired metallo-beta-lactamase gene, bla(SIM-1), in a class 1 integron from Acinetobacter baumannii clinical isolates from Korea. Antimicrob Agents Chemother (2005) 3.10
Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia (2008) 2.87
Diffusion-tensor MR imaging and fiber tractography: a new method of describing aberrant fiber connections in developmental CNS anomalies. Radiographics (2005) 1.86
Twinkle helicase (PEO1) gene mutation causes mitochondrial DNA depletion. Ann Neurol (2007) 1.67
Mitochondrial respiratory chain defects: underlying etiology in various epileptic conditions. Epilepsia (2008) 1.60
Early- and late-onset complications of the ketogenic diet for intractable epilepsy. Epilepsia (2004) 1.55
Mitochondrial DNA depletion is a prevalent cause of multiple respiratory chain deficiency in childhood. J Pediatr (2007) 1.54
A case of Ohtahara syndrome with mitochondrial respiratory chain complex I deficiency. Brain Dev (2009) 1.53
Leukoencephalopathy with vanishing white matter caused by compound heterozygous mutations in mitochondrial complex I NDUFS1 subunit. Mol Genet Metab (2010) 1.40
A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis. Hum Genet (2003) 1.17
Efficacy and safety of the ketogenic diet for intractable childhood epilepsy: Korean multicentric experience. Epilepsia (2005) 1.15
Novel FH mutations in families with hereditary leiomyomatosis and renal cell cancer (HLRCC) and patients with isolated type 2 papillary renal cell carcinoma. J Med Genet (2011) 1.14
Mitochondrial complex I deficiency of nuclear origin I. Structural genes. Mol Genet Metab (2011) 1.07
Resective pediatric epilepsy surgery in Lennox-Gastaut syndrome. Pediatrics (2009) 1.06
Use of a modified Atkins diet in intractable childhood epilepsy. Epilepsia (2007) 1.04
Neuroimaging in identifying focal cortical dysplasia and prognostic factors in pediatric and adolescent epilepsy surgery. Epilepsia (2011) 1.03
Efficacy and tolerability of the ketogenic diet according to lipid:nonlipid ratios--comparison of 3:1 with 4:1 diet. Epilepsia (2007) 1.01
Diffusion tensor MRI visualizes decreased subcortical fiber connectivity in focal cortical dysplasia. Neuroimage (2004) 0.99
Comparison of corpus callosotomy and vagus nerve stimulation in children with Lennox-Gastaut syndrome. Brain Dev (2007) 0.96
Defects in maintenance of mitochondrial DNA are associated with intramitochondrial nucleotide imbalances. Hum Mol Genet (2007) 0.96
Respiratory chain defects may present only with hypoglycemia. J Clin Endocrinol Metab (2005) 0.96
Diet therapy in refractory pediatric epilepsy: increased efficacy and tolerability. Epileptic Disord (2006) 0.95
Frequency of mitochondrial defects in patients with chronic intestinal pseudo-obstruction. Gastroenterology (2009) 0.95
Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia. Am J Hum Genet (2013) 0.94
Revisiting pitfalls, problems and tentative solutions for assaying mitochondrial respiratory chain complex III in human samples. Curr Med Chem (2004) 0.93
Mitochondrial Infantile Liver Disease due to TRMU Gene Mutations: Three New Cases. JIMD Rep (2013) 0.91
Vagus nerve stimulation in intractable childhood epilepsy: a Korean multicenter experience. J Korean Med Sci (2007) 0.89
Caregiver's burden and quality of life in mitochondrial disease. Pediatr Neurol (2010) 0.87
Prevalence of rare mitochondrial DNA mutations in mitochondrial disorders. J Med Genet (2013) 0.87
MFN2, a new gene responsible for mitochondrial DNA depletion. Brain (2012) 0.87
Ketogenic diet for treatment of infantile spasms. Brain Dev (2006) 0.86
Decylubiquinol impedes mitochondrial respiratory chain complex I activity. Mol Cell Biochem (2008) 0.86
Clinical and biochemical heterogeneity associated with fumarase deficiency. Hum Mutat (2011) 0.86
Benefits of the nonfasting ketogenic diet compared with the initial fasting ketogenic diet. Pediatrics (2004) 0.85
Quantitative analysis of gray- and white-matter volumes and glucose metabolism in Sturge-Weber syndrome. J Child Neurol (2003) 0.85
Mitochondrial complex I deficiency of nuclear origin II. Non-structural genes. Mol Genet Metab (2011) 0.85
Clinical characteristics of patients with non-specific and non-categorized mitochondrial diseases. Acta Paediatr (2009) 0.85
Increased susceptibility to liver fibrosis with age is correlated with an altered inflammatory response. Rejuvenation Res (2011) 0.84
Mitochondria DNA polymorphisms are associated with susceptibility to endometriosis. DNA Cell Biol (2011) 0.84
Nonspecific mitochondrial disease with epilepsy in children: diagnostic approaches and epileptic phenotypes. Childs Nerv Syst (2007) 0.84
Comparison of short- versus long-term ketogenic diet for intractable infantile spasms. Epilepsia (2011) 0.83
Seizure outcome of infantile spasms with focal cortical dysplasia. Brain Dev (2013) 0.83
Comparison of temporal lobectomies of children and adults with intractable temporal lobe epilepsy. Childs Nerv Syst (2009) 0.82
Comparative trial of low- and high-dose zonisamide as monotherapy for childhood epilepsy. Seizure (2011) 0.82
Landau-Kleffner syndrome with mitochondrial respiratory chain-complex I deficiency. Pediatr Neurol (2006) 0.82
Mitochondrial disease and epilepsy. Brain Dev (2013) 0.81
A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome. Mol Genet Metab (2007) 0.81
Various indications for a modified Atkins diet in intractable childhood epilepsy. Brain Dev (2011) 0.81
Long-term outcome of the ketogenic diet for intractable childhood epilepsy with focal malformation of cortical development. Pediatrics (2008) 0.81
Factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome. Pediatr Neurol (2009) 0.81
Efficacy and tolerability of adjunctive therapy with zonisamide in childhood intractable epilepsy. Brain Dev (2009) 0.81
Rufinamide as an adjuvant treatment in children with Lennox-Gastaut syndrome. Seizure (2012) 0.81
Will seizure control improve by switching from the modified Atkins diet to the traditional ketogenic diet? Epilepsia (2010) 0.81
Localization of ictal onset zones in Lennox-Gastaut syndrome using directional connectivity analysis of intracranial electroencephalography. Seizure (2011) 0.81
Early surgery of hamartoma of the floor of the fourth ventricle: a case report. Brain Dev (2008) 0.81
Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy. Yonsei Med J (2010) 0.80
Evaluation of algorithms for intracranial EEG (iEEG) source imaging of extended sources: feasibility of using iEEG source imaging for localizing epileptogenic zones in secondary generalized epilepsy. Brain Topogr (2011) 0.80
Mitotane alters mitochondrial respiratory chain activity by inducing cytochrome c oxidase defect in human adrenocortical cells. Endocr Relat Cancer (2013) 0.80
Effect of a ketogenic diet on EEG: analysis of sample entropy. Seizure (2008) 0.80
Secondary Mitochondrial Respiratory Chain Defect Can Delay Accurate PFIC2 Diagnosis. JIMD Rep (2013) 0.80
Diagnostic mutational analysis of MECP2 in Korean patients with Rett syndrome. Exp Mol Med (2006) 0.80
Comparison of various imaging modalities in localization of epileptogenic lesion using epilepsy surgery outcome in pediatric patients. Seizure (2009) 0.80
Clinicopathological aspects of the neuropathy of neurogastrointestinal encephalomyopathy (MNGIE) in four patients including two with a Charcot-Marie-Tooth presentation. J Neurol (2005) 0.80
Catch-up growth after long-term implementation and weaning from ketogenic diet in pediatric epileptic patients. Clin Nutr (2012) 0.80
The effects on cognitive function and behavioral problems of topiramate compared to carbamazepine as monotherapy for children with benign rolandic epilepsy. Epilepsia (2007) 0.80
Behavioral improvement after transplantation of neural precursors derived from embryonic stem cells into the globally ischemic brain of adolescent rats. Brain Dev (2009) 0.79
Efficacy and safety of adjunctive levetiracetam therapy in pediatric intractable epilepsy. Pediatr Neurol (2010) 0.79
Rapid screening for nuclear genes mutations in isolated respiratory chain complex I defects. Mol Genet Metab (2009) 0.79
Combined use of multiple computational intracranial EEG analysis techniques for the localization of epileptogenic zones in Lennox-Gastaut syndrome. Clin EEG Neurosci (2014) 0.79
Prognostic factors of status epilepticus in children. Yonsei Med J (2005) 0.79
Endoscopic disconnection of hypothalamic astrocytoma causing gelastic epilepsy. Case report. J Neurosurg Pediatr (2009) 0.79
A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the PDX1 gene. Ann Neurol (2003) 0.78
Electrocardiography as an early cardiac screening test in children with mitochondrial disease. Korean J Pediatr (2010) 0.78