Kenton R. Holden, MD

Senior Clinical Research Neurologist

KenHoldenDr. Holden's professional career has been primarily devoted to the study of neurodevelopmental disabilities presenting from the prenatal period through young adulthood. He has directed and participated in multiple clinical outcome studies and has authored numerous peer-reviewed articles for journals on epilepsy, neurodevelopment, pediatrics, and neuroradiology. Following his tenure on the staff at Johns Hopkins Hospital as well as in the practice of pediatrics and pediatric neurology, Dr. Holden was recruited to the Medical University of South Carolina (MUSC) in Charleston, SC in 1990 as a clinical director to establish an outpatient clinic program and inpatient consultative service in pediatric neurology. After this program was successfully established, he stepped aside to pursue new initiatives in child neurology, which included establishing outreach pediatric neurology clinics throughout South Carolina.  While on sabbatical from MUSC, he joined the Greenwood Genetic Center (GGC) in 1999 to pursue the goal of combining clinical genetics and neurology into an outreach neuroscience initiative.

Dr. Holden currently assists the clinicians and counselors with certain patients in delineating their neurologic features and making recommendations for further neurological workup. His efforts are also focused on the infrastructure necessary for research on mental and physical disabilities in South Carolina and in a developing country (Honduras). Dr. Holden's vision is that the neuroscience initiative at GGC will contribute to the future expansion of applications of genetic research to patient care, especially with regard to innovative treatment programs.

Selected Publications

  • Lockrow JP, Holden KR, Dwivedi A, Matheus MG, Lyons MJ. LIS1 Duplication: Expanding the Phenotype. J Child Neurol. 2012 Jun;27(6):791-5.

  • Stevenson RE, Holden KR, Rogers RC, Schwartz CE. Seizures and X-linked intellectual disability. Europ J Med Genet 2012 Feb:307-312.
  • Dukes-Rimsky L, Guzauskas GF, Holden KR, Griggs R, Ladd S, Del Carmen Montoya M, Dupont BR, Srivastava AK. Microdeletion at 4q21.3 is associated with intellectual disability, dysmorphic facies, hypotonia, and short stature. Am J Med Genet A. 2011 Sep;155(9):2146-53.

  • Edens AC, Lyons MJ, Duron RM, DuPont BR, Holden KR. Autism in two females with duplications involving Xp11.22-p11.23. Dev Med Child Neurol. 2011 May;53(5):463-6.
  • Medina MT, Aguilar-Estrada RL, Alvarez A, Durón RM, Martínez L, Dubón S, Estrada AL, Zúniga C, Cartagena D, Thompson A, Ramirez E, Banegas L, Osorio JR, Delgado-Escueta AV, Collins JS, Holden KR. Reduction in rate of epilepsy from neurocysticercosis by community interventions: the Salamá, Honduras study. Epilepsia. 2011 Jun;52(6):1177-85.

  • Schroer RJ, Holden KR, Tarpey PS, Matheus MG, Griesemer DA, Friez MJ, Fan JZ, Simensen RJ, Strømme P, Stevenson RE, Stratton MR, Schwartz CE. Natural history of Christianson syndrome. Am J Med Genet A. 2010 Nov;152A(11):2775-83.

  • Rollins JD, Collins JS and Holden KR: United States Head Circumference Growth Reference Charts: Birth to 21 Years. J Pediatr 156:907-13,2010.

  • Slaughter JL, Espinoza L, Molinero I, Wood TC, Duron C, Flores A, Porter R, Tomashitis K and Holden KR: Utilization of blood spot testing for metabolic-genetic disorders in Honduras: is it time for newborn screening? J Child Neurol 25:306-311, 2010.

  • Cantagrel V, Silhavy JL, Bielas SL, Swistun D, Marsh SE, Bertrand JY, Audollent S, Attié-Bitach T, Holden KR, Dobyns WB, Traver D, Al-Gazali L, Ali BR, Lindner TH, Caspary T, Otto EA, Hildebrandt F, Glass IA, Logan CV, Johnson CA, Bennett C, Brancati F; International Joubert Syndrome Related Disorders Study Group, Valente EM, Woods CG and Gleeson JG: Mutations in the cilia gene ARL13B lead to the classical form of Joubert syndrome. American Journal of Human Genetics 83:170-179, 2008.

  • Amador C, Mathews AM, Montoya MC, Laughridge ME, Everman DB and Holden KR: Expanding the neurologic phenotype of oculodentodigitial dysplasia in a 4-generation Hispanic family. Journal Child Neurology 23:901-905, 2008.

  • Rogers RC, Stevenson RE, Simensen RJ, Holden KR, Schwartz CE. Finding new etiologies of mental retardation and hypotonia: X marks the spot. Dev Med Child Neurol 50:104-111, 2008.

  • Lance EI, DuPont BR, Holden KR. Expansion of the deletion 13q syndrome phenotype: A case report. J Child Neurol 22:1124-1127, 2007

  • Dodds JA, Srivastava AK, Holden KR. Unusual phenotypic expression of an XLRS1 mutation in X-linked juvenile retinoschisis. J Child Neurol 21:331-333, 2006.

  • Holden KR, Zuniga OF, May MM, Su H, Molinero MR, Rogers RC, Schwartz CE. X-linked MCT8 gene mutations: characterization of the pediatric neurologic phenotype. J Child Neurol 20:852-857, 2005.

  • Medina MT, Duron RM, Martinez L, Osorio JR, Estrada AL, Zuniga C, Cartagena D, Collins JS, Holden KR. Prevalence, incidence, and etiology of epilepsies in rural Honduras: The Salama study. Epilepsia 46:124-131, 2005.

  • Vervoort VS, Holden KR, Ukadike KC, Collins JS, Saul RA, Srivastava AK. POMGnT1 gene alterations in a family with neurological abnormalities. Annals Neurol 56:143-148, 2004.

  • Cason AL, Ikeguchi Y, Skinner C, Wood TC, Holden KR, Lubs HA, Martinez F, Simensen RJ, Stevenson R, Pegg AE, Schwartz CE. X-linked spermine synthase gene (SMS) defect: The first polyamine deficiency syndrome. Eur J Hum Genet 11:937-944, 2003.

  • Holden KR, Collins JS, Greene J, Hinkle S, Nave AF, Portillo J, Page G, Stevenson RE, and the Honduran Neural Tube Defect Project Team. Dietary intake and blood folate levels in Honduran women of childbearing age. J Child Neurol 17:341-346, 2002.

  • Hahn KA, Salomons GS, Tackels-Horne D, Wood TC, Taylor HA, Schroer RJ, Lubs HA, Jakobs C, Olson RL, Holden KR, Stevenson RE, Schwartz CE. X-linked mental retardation with seizures and carrier manifestations is caused by a mutation in the creatine transporter gene (SLC6A8) located in Xq28. Am J Hum Genet 7:1349-1356, 2002.

  • Holden KR, Titus MO, Van Tassel P. Cranial magnetic resonance imaging examination of normal term neonates: A pilot study. J Child Neurol 14:708-710, 1999.

  • Holden KR, Sessions JC, Cure JK, Whitcomb DS, Sade RM. Neurological outcomes in children with post-pump choreoathetosis. Journal of Pediatrics 132:162-164, 1998.

  • Holden KR, Clarke SL, Griesemer DA. Long-term outcomes of conventional therapy for infantile spasms. Seizure 6:201-205, 1997.

  • Holden KR, Jabs EW, Sponseller PD. Roberts/pseudothalidomide syndrome and normal intelligence: approaches to diagnosis and management. Dev Med Child Neurol 34:534-539, 1992.

  • Shinnar S, Vining EPG, Mellits ED, D'Souza BJ, Holden K, Baumgardner RA, Freeman JM. Discontinuing antiepileptic medication in children with epilepsy after two years without seizures: A prospective study. New England Journal of Medicine 313:976-980, 1985.

  • Holden KR, Mellits ED, Freeman JM. Neonatal seizures I: Correlation of prenatal and perinatal events with outcomes. Pediatrics 70:165-176, 1982.

  • Holden KR and Dekaban AS. Neurological involvement in nevus unis lateris and nevus linearis sebaceus. Neurology (MN) 22:879-887, 1972.

  • Holden KR and Alexander F. Diffuse neonatal hemangiomatosis. Pediatrics 46:411-421, 1970.

Contact Information

Office (843) 216-5557
Fax (843) 216-5558


  • B.A., University of Virginia, Charlottesville, VA
  • M.D., Medical College of Virginia, Richmond, VA
  • Resident, Pediatrics, Johns Hopkins Hospital, Baltimore, MD
  • Clinical Assoc. Fellowship, Child Neurology, NINDS, NIH, Bethesda, MD
  • Instr./Asst. Professor, Neurology and Pediatrics, Johns Hopkins Hospital
  • Assoc. Professor, Neurology and Pediatrics, MUSC, Charleston, SC
  • Professor, Neurosciences and Pediatrics, MUSC, Charleston, SC
  • Visiting professor, Neurology and Pediatrics, National Autonomous University of Honduras, Tegucigalpa, Honduras