Representative Faculty Peer-Reviewed Publications
(1995-present)
McCullough LA, Westfall TC. Neuropeptide Y inhibits depolarization-stimulated catecholamine synthesis in rat pheochromocytoma cells. European Journal of Pharmacology, 1995;287:271-277.
Cohen BH, Zeltzer PM, Boyett JM, Geyer JR, Allen JC, Finlay JL, McGuire-Cullen P, Milstein JM, Rorke LB, Stanley P,et al. Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial. Journal of Clinical Oncology, 1995;13:1687-1696.
Ferri RT, Levitt P (1995) Regulation of regional differences in the differentiation of cerebral cortical neurons by EGF family-matrix interactions. Development 121:1151-1160.
Shurtleff, HA, Massagli, TL, Hays, RM, Ross, BR, Sprunk-Greenfield, H. Screening of mild and moderate traumatic brain injury sequelae in children and adolescents to assist with school re-entry. Journal of Head Trauma Rehabilitation 1995;10:64-79.
Ferri RT, Eagleson KL, Levitt P (1996) Environmental signals influence expression of a cortical area phenotype in vitro independent of effects on progenitor cell differentiation. Developmental Biology 175:184-190.
Eagleson, KL, Ferri, RT, Levitt P (1996) Complementary distribution of Collagen type IV and the epidermal growth factor receptor in the rat embryonic telencephalon. Cerebral Cortex 6:540-549.
McCullough LA, Westfall TC. Mechanism of catecholamine synthesis inhibition by neuropeptide Y: role of Ca2+ channels and protein kinases. Journal of Neurochemistry, 1996,67:1090-1099.
Gospe SM, Zhou SS, and Pinkerton KE. Effects of environmental tobacco smoke exposure in utero and/or postnatally on brain development, Pediatric Research 1996; 39:494-498.
Gospe SM, Zhou SS, Saeed DB, and Zeman FJ. Development of a rat model of toluene-abuse embryopathy, Pediatric Research. 1996;40:82-87.
Saneto RP, Fitch JA and Cohen BH: Acute neurotoxicity of meperidine in an infant. Pediatric Neurology 1996:14;339-341.
Levitt P, Eagleson, KL, Chan AV, Ferri RT, Lillien L (1997) Signaling pathways that regulate specification of neurons in developing cerebral cortex. Developmental Neuroscience 19:6-8.
Shaw DW, Geyer JR, Berger MS, Milstein JM, Lindsley KL. Asymptomatic recurrence detection with surveillance scanning in children with medulloblastoma. Journal of Clinical Oncology, 1997;15:1811-1813.
Feldman KW, Weinberger E, Milstein JM, Fligner CL. Cervical spine MRI in abused infants. Child Abuse and Neglect, 1997;21:199-205.
McCullough LA, Egan TM, Westfall TC. Neuropeptide Y inhibition of calcium channels in PC-12 pheochromocytoma cells. American Journal of Physiology, 1998;274:C1290-C1297.
McCullough LA, Egan TM, Westfall TC. Neuropeptide Y receptors involved in calcium channel regulation in PC12 cells. Regulatory Peptides, 1998;75-6:101-107.
Gospe SM, and Zhou SS. Toluene-abuse embryopathy: longitudinal neurodevelopmental effects of prenatal toluene exposure in rats, Reproductive Toxicology 1998;12:119-126.
Gospe SM. Current perspectives on pyridoxine-dependent seizures, Journal of . Pediatrics, 1998;132:919-923.
Gospe SM, and Hecht ST. Longitudinal MRI findings in pyridoxine-dependent seizures, Neurology 1998;51:74-78.
Saneto RP, Applegate KE and Frankel DG: Atypical manifestation of two cases of trisomy 9 syndrome: rethinking developmental delay. American Journal of Medical Genetics 1998:80;42-45.
Zhou SS, and Gospe SM. Double labeling of proliferating neurons with anti-BrdU and anti-NeuN: an improved immunohistochemical technique utilizing microwave irradiation, Journal of Histotechnology 1998;21:201-204.
Zeltzer PM, Boyett JM, Finlay JL, Albright AL, Rorke LB, Milstein JM, Allen JC, Stevens KR, Stanley P, Li H, et al. Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study. Journal of Clinical Oncology, 1999;17:832-845.
Giza, CC, Kuratani, JD, Cokely, H, Sankar, R. Periventricular nodular heterotopia and childhood absence epilepsy, Pediatric Neurology 1999;20:315-318
Battaglioli G, Rosen DR, Gospe SM, and Martin DL. Glutamate decarboxylase is not genetically linked to pyridoxine-dependent seizures, Neurology 2000;55:309-311.
Gospe SM, Caruso RD, Clegg MS, Keen CL, Pimstone NR, Ducore JM, Gettner SS, and Kreutzer RA: Paraparesis, hypermanganesemia, and polycythemia: a novel presentation of cirrhosis, Archives of Disease in Childhood 2000:83:439-442.
Gospe SM, and Zhou SS. Prenatal exposure to toluene results in abnormal neurogenesis and migration in rat somatosensory cortex, Pediatric Research 2000;47:362-368.
Kinsman SL, Plawner LL, Hahn JS. Holoprosencephaly: recent advances and new insights. Current Opinion in Neurology 2000;13:127-132.
Saneto RP and Wyllie E: Epilepsy surgery in infancy. Seminars in Pediatric Neurology 2000:7;187-193.
Moore DF, Scott LT, Gladwin MT, Altarescu G, Kaneski C, Pease-Fye M, Ferri RT, Brady RO, Herscovitch P, Schiffmann R (2001) Regional cerebral hyperperfusion and nitric oxide pathway dysregulation in Fabry disease: reversal by enzyme replacement therapy. Circulation 104:1506-1512.
Moore DF, Altarescu G, Ling GS, Jeffries N, Frei KP, Weibel T, Charria-Ortiz G, Ferri RT, Arai AE, Brady RO, Schiffmann R. (2002) Elevated cerebral blood flow velocities in Fabry disease with reversal after enzyme replacement. Stroke 33(2):525-31.
Bamford NS, Trojaborg W, Sherbany AA, DeVivo, DC: Congenital Guillain-Barre syndrome associated with maternal inflammatory bowel disease is responsive to intravenous immunoglobulin. European Journal of Paediatric Neurology 2002;6:1-5.
Gospe SM. Pyridoxine-dependent seizures: Findings from recent studies pose new questions. Pediatric Neurology 2002;26:181-185.
Plawner LL, Delgado MD, Miller VS, Levey EB, Kinsman SL, Barkovich AJ, Simon, EM, Clegg NJ, Sweet VT, Stashinko EE, Hahn JS. Neuroanatomy of holoprosencephaly as predictor of function: beyond the face predicting the brain. Neurology 2002;59:1058-1066.
Wong M, Ess KC, Uhlmann EJ, Jansen LA, Li W, Crino PB, Mennerick S, Yamada KA, Gutmann DH. Impaired glial glutamate transport in a mouse tuberous sclerosis epilepsy model. Annals of Neurology, 2003;54:251-256.
Doherty MJ, Simon, E, Sotero de Menezes MA, Kuratani JD, Saneto RP, Holmes, MD, Farrell DF, Watson NF, Dodrill, CB, Miller JW. When might hemispheric favouring of epileptiform discharges begin? Seizure, 2003;12:595-598.
Baynes K, Tomaszewski Farias S, Gospe SM. Pyridoxine-dependent seizures and cognition in adulthood. Developmental Medicine and Child Neurology 2003;45:782-785.
Bamford NS, Zhang H, Schmitz Y, Wu NP, Cepeda C, Levine MS, Schmauss C, Zakharenko SS, Zablow L, and Sulzer D. Heterosynaptic dopamine neurotransmission selects sets of corticostriatal terminals. Neuron 2004;42:653-663.
Bamford, NS, Robinson, S, Palmiter, RD, Joyce, JA, Moore, C, Meshul, CK Dopamine modulates release from corticostriatal terminals. J Neurosci 2004;24:9541-9552.
Hahn JS, Plawner LL. Evaluation and management of children with holoprosencephaly. Pediatric Neurology 2004;31:79-88.
Saneto, RP, Kotagal, P, Rothner, AD, Baker, J, Kotagal, LL. Valproic acid use in pediatric partial epilepsy after initial medication failure. J of Pediatric Neurology 2004;2:199-203.
Gospe, SM, Bell, RMS: Hospital pharmacy and emergency department availability of parenteral pyridoxine, Ped Emerg Care 2005;9:586-588.
Zerr, DM, Blume, HK, Berg, AT, Del Beccaro, MA, Gospe, SM, Allpress, AL, Christakis, DA: Nonfebrile illness seizures: a unique seizure category? Epilepsia 2005;46:952-955.
Bennett, CL, Huynh, HM, Chance, PF, Glass, IA, Gospe, SM. Genetic Heterogeneity for Autosomal Recessive Pyridoxine-Dependent Seizures. Neurogenetics 2005;6:143-149.
Jansen LA, Uhlmann EJ, Crino PB, Gutmann DH, Wong M. Epileptogenesis and reduced inward rectifier potassium current in tuberous sclerosis complex-1-deficient astrocytes. Epilepsia. 2005;46:1871-80.
Saneto, RP, Bouldin, A. A boy with muscle weakness, hypercarbia, and the mitochondrial DNA A3243G mutation. J Child Neurol 2006;21:77-79.
Everett, LL, Van Rooyen, IF, Warner, MH, Shurtleff, HA, Saneto, RP, Ojemann, JG. Use of dexmedetomidine in awake craniotomy in adolescents: report of two cases. Pediatric Anesthesia 2006;16:338-342.
Gospe SM. Pyridoxine-dependent seizures: new genetic and biochemical clues to help with diagnosis and treatment, Current Opinion in Neurology 2006;19:148-153.
Bouldin AA, Pinter JD. Resolution of arterial stenosis in a patient with periarterial neurocysticercosis treated with oral prednisone. J Child Neurol. 2006 21:1064-7.
Saneto RP, Sotero de Menezes MA, Ojemann JG, Bournival BD, Murphy PJ, Cook WB, Avellino AM, Ellenbogen RG. Vagus nerve stimulation for intractable seizures in children. Pediatr Neurol. 2006;35:323-6.
Barry PA, Lockridge KM, Salamat S, Tinling SP, Yue Y, Zhou SS, Gospe SM, Britt WJ, Tarantal AF. Nonhuman primate models of intrauterine cytomegalovirus infection. ILAR J. 2006;47:49-64.
Gurnett CA, Dobbs MB, Keppel CR, Pincus ER, Jansen LA, Bowcock A. Confirmation of a locus for complex febrile and afebrile seizures on chromosome 12q22-23.3. Neurogenetics 2007;8:61-3.
Arthur TM, Saneto RP, Sotero de Menezes MA, Devinsky O, Lajoie J, Murphy PJ, Cook WB, Ojemann JG. Vagus nerve stimulation in children with mitochondrial electron transport chain deficiencies. Mitochondrion. 2007;7:279-283.
Smyth MD, Limbrick DD Jr, Ojemann JG, Zempel J, Robinson S, O'Brien DF, Saneto RP, Goyal M, Appleton RE, Mangano FT, Park TS. Outcome following surgery for temporal lobe epilepsy with hippocampal involvement in preadolescent children: emphasis on mesial temporal sclerosis. J Neurosurg. 2007;106(3 Suppl):205-10.
Bouldin AA, Parisi MA, Laing N, Patterson K, Gospe SM. Variable presentation of nemaline myopathy: novel mutation of alpha actin gene. Muscle Nerve. 2007;35:254-8.
Pearl PL, Gospe SM. Pyridoxal phosphate dependency, a newly recognized treatable catastrophic epileptic encephalopathy. J Inherit Metab Dis 2007;30:2-4.
Haas RH, Parikh S, Falk MJ, Saneto RP, Wolf NI, Darin N, Cohen BH. Mitochondrial disease: a practical approach for primary care physicians. Pediatrics 2007;120:1326-33.
Miyagawa T, Sotero M, Avellino AM, Kuratani J, Saneto RP, Ellenbogen RG, Ojemann JG. Apnea caused by mesial temporal lobe mass lesions in infants: report of 3 cases. J Child Neurol. 2007;22:1079-83.
Saneto RP, Sotero de Menezes M. Persistence of suppression-bursts in a patient with Ohtahara syndrome. J Child Neurol. 2007;22:631-4.
Zeng LH, Ouyang Y, Gazit V, Cirrito JR, Jansen LA, Ess KC, Yamada KA, Wozniak DF, Holtzman DM, Gutmann DH, Wong M. Abnormal glutamate homeostasis and impaired synaptic plasticity and learning in a mouse model of tuberous sclerosis complex. Neurobiol Dis. 2007;28:184-96.
Juul SE, Aylward E, Richards T, McPherson RJ, Kuratani J, Burbacher TM. Prenatal cord clamping in newborn Macaca nemestrina: a model of perinatal asphyxia. Dev Neurosci. 2007;29:311-20.
Bamford NS, Zhang H, Joyce JA, Scarlis CA, Hanan W, Wu NP, André VM, Cohen R, Cepeda C, Levine MS, Harleton E, Sulzer D. Repeated exposure to methamphetamine causes long-lasting presynaptic corticostriatal depression that is renormalized with drug readministration. Neuron. 2008;58:89-103.
Holmes MD, Brown M, Tucker DM, Saneto RP, Miller KJ, Wig GS, Ojemann JG. Localization of extratemporal seizure with noninvasive dense-array EEG. Comparison with intracranial recordings. Pediatr Neurosurg. 2008;44:474-9.
Saneto RP, Friedman SD, Shaw DW. Neuroimaging of mitochondrial disease. Mitochondrion. 2008;8:396-413.
Wong LJ, Naviaux RK, Brunetti-Pierri N, Zhang Q, Schmitt ES, Truong C, Milone M, Cohen BH, Wical B, Ganesh J, Basinger AA, Burton BK, Swoboda K, Gilbert DL, Vanderver A, Saneto RP, Maranda B, Arnold G, Abdenur JE, Waters PJ, Copeland WC. Molecular and clinical genetics of mitochondrial diseases due to POLG mutations. Hum Mutat. 2008;29:E150-E172.
Mitochondrial Medicine Society's Committee on Diagnosis, Haas RH, Parikh S, Falk MJ, Saneto RP, Wolf NI, Darin N, Wong LJ, Cohen BH, Naviaux RK. The in-depth evaluation of suspected mitochondrial disease. Mol Genet Metab. 2008;94:16-37.
Jansen LA, Peugh LD, Ojemann JG. GABA(A) receptor properties in catastrophic infantile epilepsy. Epilepsy Res. 2008;81:188-97.
Blume HK, Li CI, Loch CM, Koepsell TD. Intrapartum fever and chorioamnionitis as risks for encephalopathy in term newborns: a case-control study. Dev Med Child Neurol. 2008;50:19-24.
Bennett CL, Chen Y, Hahn S, Glass IA, Gospe SM Jr. Prevalence of ALDH7A1 mutations in 18 North American pyridoxine-dependent seizure (PDS) patients. Epilepsia. 2009;50:1167-75.
Gospe SM Jr, Joyce JA, Siebert JR, Jack RM, Pinkerton KE. Exposure to environmental tobacco smoke during pregnancy in rats yields less effect on indices of brain cell number and size than does postnatal exposure. Reprod Toxicol. 2009;27:22-7.
Basura GJ, Hagland SP, Wiltse AM, Gospe SM Jr. Clinical features and the management of pyridoxine-dependent and pyridoxine-responsive seizures: review of 63 North American cases submitted to a patient registry. Eur J Pediatr. 2009;168:697-704.
Bamford NS, White KK, Robinett SA, Otto RK, Gospe SM Jr. Neuromuscular hip dysplasia in Charcot-Marie-Tooth disease type 1A. Dev Med Child Neurol. 2009;51:408-11.
Joshi PR, Wu NP, André VM, Cummings DM, Cepeda C, Joyce JA, Carroll JB, Leavitt BR, Hayden MR, Levine MS, Bamford NS. Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease. J Neurosci. 2009;29:2414-27.
Saneto RP, Anderson GD. Onset of action and seizure control in Lennox-Gaustaut syndrome: focus on rufinamide. Ther Clin Risk Manag. 2009;5:271-80.
Blume HK, Garrison MM, Christakis DA. Neonatal seizures: treatment and treatment variability in 31 United States pediatric hospitals. J Child Neurol. 2009;24:148-54.