Scholars@Duke

• Background
• 

  Education, Training, & Certifications

  • Pediatric Neurology Fellowship, Pediatrics, Baylor College of Medicine 1983 - 1986
  • Residency, Pediatrics, Baylor College of Medicine 1981 - 1983
  • M.D., Duke University School of Medicine 1981
  • Ph.D., Duke University 1980
• 

  Duke Appointment History

  • Adjunct Professor in the Department of Pediatrics, Pediatrics, Neurology, Pediatrics 2017 - 2018
• 

  Academic Positions Outside Duke

  • Professor Emeritus of Neurology and Pediatrics, University of Washington. 2017 - 2018
  • Professor of Neurology and Pediatrics, University of Washington. 2000 - 2017
• Publications & Artistic Works
• 

  Selected Publications

  • Academic Articles

    • Coughlin, CR, Swanson, MA, Spector, E, Meeks, NJL, Kronquist, KE, Aslamy, M, Wempe, MF, van Karnebeek, CDM, Gospe, SM, Aziz, VG, Tsai, BP, Gao, H, Nagy, PL, Hyland, K, van Dooren, SJM, Salomons, GS, and Van Hove, JLK. "The genotypic spectrum of ALDH7A1 mutations resulting in pyridoxine dependent epilepsy: A common epileptic encephalopathy." Journal of Inherited Metabolic Disease 42, no. 2 (March 2019): 353-361.  Full Text
    • Lo, MD, and Gospe, SM. "Telemedicine and Child Neurology." Journal of Child Neurology 34, no. 1 (January 2019): 22-26.  Full Text
    • Gospe, SM. "Developmental outcome in pyridoxine-dependent epilepsy: Better late (onset) than early." European Journal of Paediatric Neurology : Ejpn : Official Journal of the European Paediatric Neurology Society 22, no. 4 (July 2018): 575-576.  Full Text
    • Oesch, G, Maga, AM, Friedman, SD, Poliachik, SL, Budech, CB, Wright, JN, Bok, LA, and Gospe, SM. "Geometric morphometrics reveal altered corpus callosum shape in pyridoxine-dependent epilepsy." Neurology 91, no. 1 (July 2018): e78-e86.  Full Text
    • de Rooy, RLP, Halbertsma, FJ, Struijs, EA, van Spronsen, FJ, Lunsing, RJ, Schippers, HM, van Hasselt, PM, Plecko, B, Wohlrab, G, Whalen, S, Benoist, JF, Valence, S, Mills, PB, and Bok, LA. "Pyridoxine dependent epilepsy: Is late onset a predictor for favorable outcome?." European Journal of Paediatric Neurology 22, no. 4 (July 2018): 662-666.  Full Text
    • Weisleder, P, Gospe, SM, Ng, Y-T, and Sahin, M. "The Pediatric Neurology Trainee Publication Award for 2015." Pediatric Neurology 63 (October 2016): 1-2.  Full Text
    • Tuschl, K, Clayton, PT, Gospe, SM, Gulab, S, Ibrahim, S, Singhi, P, Aulakh, R, Ribeiro, RT, Barsottini, OG, Zaki, MS, Del Rosario, ML, Dyack, S, Price, V, Rideout, A, Gordon, K, Wevers, RA, Chong, WKK, and Mills, PB. "Syndrome of Hepatic Cirrhosis, Dystonia, Polycythemia, and Hypermanganesemia Caused by Mutations in SLC30A10, a Manganese Transporter in Man." American journal of human genetics 99, no. 2 (August 2016): 521-.  Full Text
    • Byers, HM, Beatty, CW, Hahn, SH, and Gospe, SM. "Dramatic Response After Lamotrigine in a Patient With Epileptic Encephalopathy and a De NovoCACNA1A Variant." Pediatric Neurology 60 (July 2016): 79-82.  Full Text
    • Burns, TM, Smith, GA, Allen, JA, Amato, AA, Arnold, WD, Barohn, R, Benatar, M, Bird, SJ, Bromberg, M, Chahin, N, Ciafaloni, E, Cohen, JA, Corse, A, Crum, BA, David, WS, Dimberg, E, Sousa, EAD, Donofrio, PD, Dyck, PJB, Engel, AG, Ensrud, ER, Ferrante, M, Freimer, M, Gable, KL, Gibson, S, Gilchrist, JM, Goldstein, JM, Gooch, CL, Goodman, BP, Gorelov, D, Gospe, SM, Goyal, NA, Guidon, AC, Guptill, JT, Gutmann, L, Gutmann, L, Gwathmey, K, Harati, Y, Harper, CM, Hehir, MK, Hobson-Webb, LD, Howard, JF, Jackson, CE, Johnson, N, Jones, SM, Juel, VC, Kaminski, HJ, Karam, C, Kennelly, KD, Khella, S, Khoury, J, Kincaid, JC, Kissel, JT, Kolb, N, Lacomis, D, Ladha, S, Larriviere, D, Lewis, RA, Li, Y, Litchy, WJ, Logigian, E, Lou, J-S, MacGowen, DJL, Maselli, R, Massey, JM, Mauermann, ML, Mathews, KD, Meriggioli, MN, Miller, RG, Moon, J-S, Mozaffar, T, Nations, SP, Nowak, RJ, Ostrow, LW, Pascuzzi, RM, Peltier, A, Ruzhansky, K, Richman, DP, Ross, MA, Rubin, DI, Russell, JA, Sachs, GM, Salajegheh, MK, Saperstein, DS, Scelsa, S, Selcen, D, Shaibani, A, Shieh, PB, Silvestri, NJ, Singleton, JR, Smith, BE, So, YT, Solorzano, G, Sorenson, EJ, Srinivasen, J, Tavee, J, Tawil, R, Thaisetthawatkul, P, Thornton, C, Trivedi, J, Vernino, S, Wang, AK, Webb, TA, Weiss, MD, Windebank, AJ, and Wolfe, GI. "Editorial by concerned physicians: Unintended effect of the orphan drug act on the potential cost of 3,4-diaminopyridine." Muscle & Nerve 53, no. 2 (February 2016): 165-168.  Full Text
    • Poliachik, SL, Friedman, SD, Poliakov, AV, Budech, CB, Ishak, GE, Shaw, DWW, and Gospe, SM. "Corpus Callosum Diffusion and Connectivity Features in High Functioning Subjects With Pyridoxine-Dependent Epilepsy." Pediatric Neurology 54 (January 2016): 43-48.  Full Text
    • Mann, PC, Gospe, SM, Steinman, KJ, and Wilfond, BS. "Integrating neurocritical care approaches into neonatology: should all infants be treated equitably?." Journal of perinatology : official journal of the California Perinatal Association 35, no. 12 (December 2015): 977-981.  Full Text
    • Arredondo, J, Lara, M, Gospe, SM, Mazia, CG, Vaccarezza, M, Garcia-Erro, M, Bowe, CM, Chang, CH, Mezei, MM, and Maselli, RA. "Choline Acetyltransferase Mutations Causing Congenital Myasthenic Syndrome: Molecular Findings and Genotype-Phenotype Correlations." Human mutation 36, no. 9 (September 2015): 881-893.  Full Text
    • Bertini, E, and Gospe, SM. "SSADH deficiency in an adult: Venturing outside of the diagnostic box and inside of the registry." Neurology 85, no. 10 (September 2015): 842-843.  Full Text
    • Mefford, HC, Zemel, M, Geraghty, E, Cook, J, Clayton, PT, Paul, K, Plecko, B, Mills, PB, Nordli, DR, and Gospe, SM. "Intragenic deletions of ALDH7A1 in pyridoxine-dependent epilepsy caused by Alu-Alu recombination." Neurology 85, no. 9 (September 2015): 756-762.  Full Text
    • Ng, Y-T, Gospe, SM, and Sahin, M. "Pediatric Neurology 2014 Trainee Publication Award Winner: Dr. Mitchel T. Williams." Pediatric Neurology 53, no. 2 (August 2015): 103-104.  Full Text
    • Friedman, SD, Ishak, GE, Poliachik, SL, Poliakov, AV, Otto, RK, Shaw, DWW, Willemsen, MA, Bok, LA, and Gospe, SM. "Callosal alterations in pyridoxine-dependent epilepsy." Developmental Medicine and Child Neurology 56, no. 11 (November 2014): 1106-1110.  Full Text
    • Beatty, CW, Ko, P-R, Nixon, J, and Gospe, SM. "Delayed-onset movement disorder and encephalopathy after oxycodone ingestion." Seminars in pediatric neurology 21, no. 2 (June 18, 2014): 160-165.  Full Text
    • Lechpammer, M, Clegg, MS, Muzar, Z, Huebner, PA, Jin, L-W, and Gospe, SM. "Pathology of inherited manganese transporter deficiency." Annals of Neurology 75, no. 4 (April 14, 2014): 608-612.  Full Text
    • Pearl, PL, and Gospe, SM. "Pyridoxine or pyridoxal-5'-phosphate for neonatal epilepsy: the distinction just got murkier." Neurology 82, no. 16 (April 2014): 1392-1394.  Full Text
    • Richardson, RC, Tarleton, JC, Bird, TD, and Gospe, SM. "Truncating CLCN1 mutations in myotonia congenita: variable patterns of inheritance." Muscle & nerve 49, no. 4 (April 2014): 593-600.  Full Text
    • Roach, ES, Gospe, S, Ng, Y-T, and Sahin, M. "Trust but verify: the introduction of plagiarism detection software." Pediatric Neurology 50, no. 4 (April 2014): 287-null.  Full Text
    • Segal, MM, Williams, MS, Gropman, AL, Torres, AR, Forsyth, R, Connolly, AM, El-Hattab, AW, Perlman, SJ, Samanta, D, Parikh, S, Pavlakis, SG, Feldman, LK, Betensky, RA, and Gospe, SM. "Evidence-based decision support for neurological diagnosis reduces errors and unnecessary workup." Journal of child neurology 29, no. 4 (April 2014): 487-492.  Full Text
    • Wallace, SE, Conta, JH, Winder, TL, Willer, T, Eskuri, JM, Haas, R, Patterson, K, Campbell, KP, Moore, SA, and Gospe, SM. "A novel missense mutation in POMT1 modulates the severe congenital muscular dystrophy phenotype associated with POMT1 nonsense mutations." Neuromuscular Disorders : Nmd 24, no. 4 (April 2014): 312-320.  Full Text
    • Jansen, LA, Hevner, RF, Roden, WH, Hahn, SH, Jung, S, and Gospe, SM. "Glial localization of antiquitin: implications for pyridoxine-dependent epilepsy." Annals of Neurology 75, no. 1 (January 2, 2014): 22-32.  Full Text
    • van Karnebeek, CDM, Stockler-Ipsiroglu, S, Jaggumantri, S, Assmann, B, Baxter, P, Buhas, D, Bok, LA, Cheng, B, Coughlin, CR, Das, AM, Giezen, A, Al-Hertani, W, Ho, G, Meyer, U, Mills, P, Plecko, B, Struys, E, Ueda, K, Albersen, M, Verhoeven, N, Gospe, SM, Gallagher, RC, Van Hove, JKL, and Hartmann, H. "Lysine-Restricted Diet as Adjunct Therapy for Pyridoxine-Dependent Epilepsy: The PDE Consortium Consensus Recommendations." Jimd Reports 15 (January 2014): 1-11.  Full Text
    • Tully, HM, Dempsey, JC, Ishak, GE, Adam, MP, Mink, JW, Dobyns, WB, Gospe, SM, Weiss, A, Phillips, JO, and Doherty, D. "Persistent figure-eight and side-to-side head shaking is a marker for rhombencephalosynapsis." Movement Disorders : Official Journal of the Movement Disorder Society 28, no. 14 (December 2013): 2019-2023.  Full Text
    • Jung, S, Tran, N-TB, Gospe, SM, and Hahn, SH. "Preliminary investigation of the use of newborn dried blood spots for screening pyridoxine-dependent epilepsy by LC-MS/MS." Molecular Genetics and Metabolism 110, no. 3 (November 2013): 237-240.  Full Text
    • Mefford, HC, Cook, J, and Gospe, SM. "Epilepsy due to 20q13.33 subtelomere deletion masquerading as pyridoxine-dependent epilepsy." American journal of medical genetics. Part A 158A, no. 12 (December 2012): 3190-3195.  Full Text
    • van Karnebeek, CDM, Hartmann, H, Jaggumantri, S, Bok, LA, Cheng, B, Connolly, M, Coughlin, CR, Das, AM, Gospe, SM, Jakobs, C, van der Lee, JH, Mercimek-Mahmutoglu, S, Meyer, U, Struys, E, Sinclair, G, Van Hove, J, Collet, J-P, Plecko, BR, and Stockler, S. "Lysine restricted diet for pyridoxine-dependent epilepsy: first evidence and future trials." Molecular Genetics and Metabolism 107, no. 3 (November 2012): 335-344.  Full Text
    • Gospe, SM. "Natural history of pyridoxine-dependent epilepsy: tools for prognostication." Developmental medicine and child neurology 54, no. 9 (September 2012): 781-782.  Full Text
    • Tuschl, K, Clayton, PT, Gospe, SM, Gulab, S, Ibrahim, S, Singhi, P, Aulakh, R, Ribeiro, RT, Barsottini, OG, Zaki, MS, Del Rosario, ML, Dyack, S, Price, V, Rideout, A, Gordon, K, Wevers, RA, Chong, WKK, and Mills, PB. "Syndrome of hepatic cirrhosis, dystonia, polycythemia, and hypermanganesemia caused by mutations in SLC30A10, a manganese transporter in man." American Journal of Human Genetics 90, no. 3 (March 2012): 457-466.  Full Text
    • Stockler, S, Plecko, B, Gospe, SM, Coulter-Mackie, M, Connolly, M, van Karnebeek, C, Mercimek-Mahmutoglu, S, Hartmann, H, Scharer, G, Struijs, E, Tein, I, Jakobs, C, Clayton, P, and Van Hove, JLK. "Pyridoxine dependent epilepsy and antiquitin deficiency: clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up." Molecular Genetics and Metabolism 104, no. 1-2 (September 2011): 48-60. (Review)  Full Text
    • Segal, EB, Grinspan, ZM, Mandel, AM, and Gospe, SM. "Biomarkers aiding diagnosis of atypical presentation of pyridoxine-dependent epilepsy." Pediatric neurology 44, no. 4 (April 2011): 289-291.  Full Text
    • Martin, ET, Kerin, T, Christakis, DA, Blume, HK, Gospe, SM, Vinje, J, Bowen, MD, Gentsch, J, and Zerr, DM. "Redefining outcome of first seizures by acute illness." Pediatrics 126, no. 6 (December 2010): e1477-e1484.  Full Text
    • Soltanzadeh, P, Friez, MJ, Dunn, D, von Niederhausern, A, Gurvich, OL, Swoboda, KJ, Sampson, JB, Pestronk, A, Connolly, AM, Florence, JM, Finkel, RS, Bönnemann, CG, Medne, L, Mendell, JR, Mathews, KD, Wong, BL, Sussman, MD, Zonana, J, Kovak, K, Gospe, SM, Gappmaier, E, Taylor, LE, Howard, MT, Weiss, RB, and Flanigan, KM. "Clinical and genetic characterization of manifesting carriers of DMD mutations." Neuromuscular Disorders : Nmd 20, no. 8 (August 2010): 499-504.  Full Text
    • Gospe, SM. "Pyridoxine-dependent epilepsy and pyridoxine phosphate oxidase deficiency: unique clinical symptoms and non-specific EEG characteristics." Developmental medicine and child neurology 52, no. 7 (July 2010): 602-603.  Full Text
    • Gospe, SM. "Neonatal vitamin-responsive epileptic encephalopathies." Chang Gung medical journal 33, no. 1 (January 2010): 1-12. (Review)
    • Sadilkova, K, Gospe, SM, and Hahn, SH. "Simultaneous determination of alpha-aminoadipic semialdehyde, piperideine-6-carboxylate and pipecolic acid by LC-MS/MS for pyridoxine-dependent seizures and folinic acid-responsive seizures." Journal of Neuroscience Methods 184, no. 1 (October 2009): 136-141.  Full Text
    • Basura, GJ, Hagland, SP, Wiltse, AM, and Gospe, SM. "Clinical features and the management of pyridoxine-dependent and pyridoxine-responsive seizures: review of 63 North American cases submitted to a patient registry." European journal of pediatrics 168, no. 6 (June 2009): 697-704.  Full Text
    • Bamford, NS, White, KK, Robinett, SA, Otto, RK, and Gospe, SM. "Neuromuscular hip dysplasia in Charcot-Marie-Tooth disease type 1A." Developmental medicine and child neurology 51, no. 5 (May 2009): 408-411.  Full Text
    • Bennett, CL, Chen, Y, Hahn, S, Glass, IA, and Gospe, SM. "Prevalence of ALDH7A1 mutations in 18 North American pyridoxine-dependent seizure (PDS) patients." Epilepsia 50, no. 5 (May 2009): 1167-1175.  Full Text
    • Gospe, SM, Joyce, JA, Siebert, JR, Jack, RM, and 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." Reproductive toxicology (Elmsford, N.Y.) 27, no. 1 (January 2009): 22-27.  Full Text
    • Bouldin, AA, Parisi, MA, Laing, N, Patterson, K, and Gospe, SM. "Variable presentation of nemaline myopathy: novel mutation of alpha actin gene." Muscle & nerve 35, no. 2 (February 2007): 254-258.  Full Text
    • Pearl, PL, and Gospe, SM. "Pyridoxal phosphate dependency, a newly recognized treatable catastrophic epileptic encephalopathy." Journal of Inherited Metabolic Disease 30, no. 1 (February 2007): 2-4.  Full Text
    • Gospe, SM. "Pyridoxine-dependent seizures: new genetic and biochemical clues to help with diagnosis and treatment." Current opinion in neurology 19, no. 2 (April 2006): 148-153. (Review)  Full Text
    • Barry, PA, Lockridge, KM, Salamat, S, Tinling, SP, Yue, Y, Zhou, SS, Gospe, SM, Britt, WJ, and Tarantal, AF. "Nonhuman primate models of intrauterine cytomegalovirus infection." Ilar Journal 47, no. 1 (January 2006): 49-64. (Review)  Full Text
    • Bennett, CL, Huynh, HM, Chance, PF, Glass, IA, and Gospe, SM. "Genetic heterogeneity for autosomal recessive pyridoxine-dependent seizures." Neurogenetics 6, no. 3 (September 2005): 143-149.  Full Text
    • Gospe, SM, and Bell, RMS. "Hospital pharmacy and emergency department availability of parenteral pyridoxine." Pediatric emergency care 21, no. 9 (September 2005): 586-588.  Full Text
    • Zerr, DM, Blume, HK, Berg, AT, Del Beccaro, MA, Gospe, SM, Allpress, AL, and Christakis, DA. "Nonfebrile illness seizures: a unique seizure category?." Epilepsia 46, no. 6 (June 2005): 952-955.  Full Text
    • Allan, WC, and Gospe, SM. "Seizures, syncope, or breath-holding presenting to the pediatric neurologist--when is the etiology a life-threatening arrhythmia?." Seminars in pediatric neurology 12, no. 1 (March 2005): 2-9. (Review)  Full Text
    • Gospe, S, and Camfield, P. "Cardiac causes of sudden death: virtual panel discussion of posed questions." Seminars in pediatric neurology 12, no. 1 (March 2005): 67-69.  Full Text
    • Baynes, K, Farias, ST, and Gospe, SM. "Pyridoxine-dependent seizures and cognition in adulthood." Developmental medicine and child neurology 45, no. 11 (November 2003): 782-785. (Review)  Full Text
    • Gospe, SM. "Pyridoxine-dependent seizures: findings from recent studies pose new questions." Pediatric neurology 26, no. 3 (March 2002): 181-185. (Review)  Full Text
    • Gospe, SM, Caruso, RD, Clegg, MS, Keen, CL, Pimstone, NR, Ducore, JM, Gettner, SS, and Kreutzer, RA. "Paraparesis, hypermanganesaemia, and polycythaemia: a novel presentation of cirrhosis." Archives of disease in childhood 83, no. 5 (November 2000): 439-442.  Full Text
    • Battaglioli, G, Rosen, DR, Gospe, SM, and Martin, DL. "Glutamate decarboxylase is not genetically linked to pyridoxine-dependent seizures." Neurology 55, no. 2 (July 2000): 309-311.  Full Text
    • Gospe, SM, and Zhou, SS. "Prenatal exposure to toluene results in abnormal neurogenesis and migration in rat somatosensory cortex." Pediatric research 47, no. 3 (March 2000): 362-368.  Full Text
    • Ebinger, M, Schultze, C, and König, S. "Demographics and diagnosis of pyridoxine-dependent seizures." The Journal of pediatrics 134, no. 6 (June 1999): 795-796. (Letter)  Full Text
    • Gospe, SM. "Reply." The Journal of pediatrics 134, no. 6 (June 1999): 795-796.
    • Gospe, SM, El-Schahawi, M, Shanske, S, Bruno, C, DiMauro, S, Hoye, E, Walsh, DA, and Gorin, FA. "Asymptomatic McArdle's disease associated with hyper-creatine kinase-emia and absence of myophosphorylase." Neurology 51, no. 4 (October 1998): 1228-1229.  Full Text
    • Gospe, SM, and Hecht, ST. "Longitudinal MRI findings in pyridoxine-dependent seizures." Neurology 51, no. 1 (July 1998): 74-78.  Full Text
    • Gospe, SM. "Current perspectives on pyridoxine-dependent seizures." The Journal of pediatrics 132, no. 6 (June 1998): 919-923. (Review)  Full Text
    • Gospe, SM, and Zhou, SS. "Toluene abuse embryopathy: longitudinal neurodevelopmental effects of prenatal exposure to toluene in rats." Reproductive toxicology (Elmsford, N.Y.) 12, no. 2 (March 1998): 119-126.  Full Text
    • Shah Shan Zhou, , and Gospe S.M., J. "Double labeling of proliferating neurons with anti-BrdU and anti-NeuN: An improved immunohistochemical technique utilizing microwave irradiation." Journal of Histotechnology 21, no. 3 (January 1, 1998): 201-204.
    • Bakshi, N, Maselli, RA, Gospe, SM, Ellis, WG, McDonald, C, and Mandler, RN. "Fulminant demyelinating neuropathy mimicking cerebral death." Muscle and Nerve 20, no. 12 (December 12, 1997): 1595-1597.  Full Text
    • Bakshi, N, Maselli, RA, Gospe, SM, Ellis, WG, McDonald, C, and Mandler, RN. "Fulminant demyelinating neuropathy mimicking cerebral death." Muscle & Nerve 20, no. 12 (December 1997): 1595-1597.  Full Text
    • Bakerink, JA, Gospe, SM, Dimand, RJ, and Eldridge, MW. "Multiple organ failure after ingestion of pennyroyal oil from herbal tea in two infants." Pediatrics 98, no. 5 (November 1996): 944-947.
    • Gospe, SM, Zhou, SS, Saeed, DB, and Zeman, FJ. "Development of a rat model of toluene-abuse embryopathy." Pediatric research 40, no. 1 (July 1996): 82-87.  Full Text
    • Lowe, DW, Gospe, SM, and Hecht, ST. "Spinal arteriovenous malformation presenting as meningitis." Developmental medicine and child neurology 38, no. 6 (June 1996): 549-553.
    • Gospe, SM, Zhou, SS, and Pinkerton, KE. "Effects of environmental tobacco smoke exposure in utero and/or postnatally on brain development." Pediatric research 39, no. 3 (March 1996): 494-498.  Full Text
    • Gospe, SM, Gietzen, DW, Summers, PJ, Lunetta, JM, Miller, JW, Selhub, J, Ellis, WG, and Clifford, AJ. "Behavioral and neurochemical changes in folate-deficient mice." Physiology & behavior 58, no. 5 (November 1995): 935-941.  Full Text
    • Gospe, SM. "Transient cortical blindness in an infant exposed to methamphetamine." Annals of emergency medicine 26, no. 3 (September 1995): 380-382.  Full Text
    • Tedeschi, G, Schiffmann, R, Barton, NW, Shih, HH, Gospe, SM, Brady, RO, Alger, JR, and Di Chiro, G. "Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination." Neurology 45, no. 8 (August 1995): 1526-1532.  Full Text
    • Engel, DG, Gospe, SM, Tracy, KA, Ellis, WG, and Lie, JT. "Fatal infantile polyarteritis nodosa with predominant central nervous system involvement." Stroke 26, no. 4 (April 1995): 699-701.  Full Text
    • Pranzatelli, MR, Huang, Y, Tate, E, Stanley, M, Noetzel, MJ, Gospe, SM, and Banasiak, K. "Cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid in the pediatric opsoclonus-myoclonus syndrome." Annals of Neurology 37, no. 2 (February 1995): 189-197.  Full Text
    • Gospe, SM, Saeed, DB, Zhou, SS, and Zeman, FJ. "The effects of high-dose toluene on embryonic development in the rat." Pediatric research 36, no. 6 (December 1994): 811-815.  Full Text
    • Jones, AD, Dunlap, MR, and Gospe, SM. "Stable-isotope dilution GC-MS for determination of toluene in submilliliter volumes of whole blood." Journal of Analytical Toxicology 18, no. 5 (September 1994): 251-254.  Full Text
    • Gospe, SM, Olin, KL, and Keen, CL. "Reduced GABA synthesis in pyridoxine-dependent seizures." Lancet (London, England) 343, no. 8906 (May 1994): 1133-1134.  Full Text
    • Gospe, SM, and Al-Bayati, MAS. "Comparison of oral and inhalation exposures to toluene." Journal of the American College of Toxicology 13, no. 1 (January 1, 1994): 21-32.  Full Text
    • Gospe, SM, and al-Bayati, MAS. "Comparison of Oral and Inhalation Exposures to Toluene." International Journal of Toxicology 13, no. 1 (January 1, 1994): 21-32.  Full Text
    • Kurowski, HL, Gospe, SM, Zeman, FJ, and Grivetti, LE. "Nutritional factors and anticonvulsant therapies: effect on growth in children with epilepsy." The American journal of clinical nutrition 58, no. 6 (December 1993): 858-861.  Full Text
    • Hansen, RL, Struthers, JM, and Gospe, SM. "Visual evoked potentials and visual processing in stimulant drug-exposed infants." Developmental medicine and child neurology 35, no. 9 (September 1993): 798-805.
    • Saeed, D, Goetzman, BW, and Gospe, SM. "Brain injury and protective effects of hypothermia using triphenyltetrazolium chloride in neonatal rat." Pediatric neurology 9, no. 4 (July 1993): 263-267.  Full Text
    • Wennberg, RP, Gospe, SM, Rhine, WD, Seyal, M, Saeed, D, and Sosa, G. "Brainstem bilirubin toxicity in the newborn primate may be promoted and reversed by modulating PCO2." Pediatric research 34, no. 1 (July 1993): 6-9.  Full Text
    • Gospe, SM. "Routine monitoring of the electrocardiogram Q-T interval in the EEG laboratory." American Journal of Eeg Technology 32, no. 1 (January 1, 1992): 58-64. (Review)
    • Gospe, SM, and Gabor, AJ. "Electroencephalography laboratory diagnosis of prolonged QT interval." Annals of neurology 28, no. 3 (September 1990): 387-390.  Full Text
    • Gospe, SM. "A pediatrician's personal reflections on varicella." Pediatrics 86, no. 3 (September 1990): 494-. (Letter)
    • Gospe, SM. "Toluene dementia." Neurology 40, no. 8 (August 1990): 1320-1321. (Letter)  Full Text
    • Gospe, SM, Mora, BJ, and Glick, SD. "Measurement of spontaneous rotational movement (circling) in normal children." Journal of child neurology 5, no. 1 (January 1990): 31-34.  Full Text
    • Gospe, SM, Lazaro, RP, Lava, NS, Grootscholten, PM, Scott, MO, and Fischbeck, KH. "Familial X-linked myalgia and cramps: a nonprogressive myopathy associated with a deletion in the dystrophin gene." Neurology 39, no. 10 (October 1989): 1277-1280.  Full Text
    • Gospe, SM, and Choy, M. "Hereditary long Q-T syndrome presenting as epilepsy: electroencephalography laboratory diagnosis." Annals of neurology 25, no. 5 (May 1989): 514-516.  Full Text
    • McGuire, SA, Gospe, SM, and Dahl, G. "Acute vincristine neurotoxicity in the presence of hereditary motor and sensory neuropathy type I." Medical and pediatric oncology 17, no. 6 (January 1989): 520-523. (Review)  Full Text
    • Van Antwerpen, CL, Gospe, SM, and Wade, NA. "Myeloradiculopathy associated with wasp sting." Pediatric neurology 4, no. 6 (November 1988): 379-380. (Review)  Full Text
    • Gospe, SM, and Calaban, MJ. "Central nervous system distribution of inhaled toluene." Fundamental and applied toxicology : official journal of the Society of Toxicology 11, no. 3 (October 1988): 540-545.  Full Text
    • Van Antwerpen, CL, Gospe, SM, and Dentinger, MP. "Nemaline myopathy associated with hypertrophic cardiomyopathy." Pediatric neurology 4, no. 5 (September 1988): 306-308.  Full Text
    • Gospe, SM, and Calaban, MJ. "Central Nervous System Distribution of Inhaled Toluene." Toxicological Sciences 11, no. 1 (1988): 540-545.  Full Text
    • Gospe, SM, Armstrong, DL, Gresik, MV, and Hawkins, HK. "Life-threatening congestive heart failure as the presentation of centronuclear myopathy." Pediatric neurology 3, no. 2 (March 1987): 117-120.  Full Text
    • Gospe, SM, Armstrong, DL, Gresik, MV, and Hawkins, HK. "Life-threatening congestive heart failure as the presentation of centronuclear myopathy." Pediatric Neurology 3, no. 2 (January 1, 1987): 117-120.  Full Text
    • Hrachovy, RA, Frost, JD, Gospe, SM, and Glaze, DG. "Infantile spasms following near-drowning: a report of two cases." Epilepsia 28, no. 1 (January 1987): 45-48.  Full Text
    • Gospe, SM, and Jankovic, J. "Drug-induced dystonia in neuronal ceroid-lipofuscinosis." Pediatric neurology 2, no. 4 (July 1986): 236-237.  Full Text
    • Gospe, SM. "Studies of dopamine pharmacology in molluscs." Life sciences 33, no. 20 (November 1983): 1945-1957. (Review)  Full Text
    • Gospe, SM, and Wilson, WA. "Burst-firing inhibition of cell R 15 in Aplysia californica: Pharmacological studies of the effects of tyramine, β-phenethylamine and D-amphetamine." Comparative Biochemistry and Physiology. Part C, Comparative 71, no. 2 (January 1, 1982): 249-254.  Full Text
    • Gospe, SM, and Wilson, WA. "Pharmacological studies of a novel dopamine-sensitive receptor mediating burst-firing inhibition of neurosecretory cell R 15 in Aplysia californica." The Journal of Pharmacology and Experimental Therapeutics 216, no. 2 (February 1981): 368-377.
    • Gospe, SM, Cook, LL, Crutcher, KA, and Wilson, WA. "Biochemical and histochemical studies of the effect of reserpine in Aplysia californica." Comparative Biochemistry and Physiology. Part C, Comparative 70, no. 2 (January 1, 1981): 273-276.  Full Text
    • Gospe, SM, and Wilson, WA. "Dopamine inhibits burst-firing of neurosecretory cell R 15 in Aplysia californica: establishment of a dose-response relationship." The Journal of pharmacology and experimental therapeutics 214, no. 1 (July 1980): 112-118.
    • Gospe, SM, and Wilson, WA. "Inhibition of burst-firing in cell R 15 of Aplysia californica: Effects of catecholamine releasing agents." Neuroscience Letters 19, no. SUPPL. 5 (January 1, 1980).
    • Gospe, SM, Bias, RR, and Winkler, SR. "The Edgemont Community Clinic: Durham's student-operated free clinic begins its second decade." North Carolina medical journal 40, no. 6 (June 1979): 357-361.
    • Snapp, BD, Heller, HC, and Gospe, SM. "Hypothalamic thermosensitivity in California quail (Lophortyx californicus)." Journal of Comparative Physiology ■ B 117, no. 3 (January 1, 1977): 345-357.  Full Text

  • Book Sections

    • Natarajan, N, and Gospe, SM. "Neonatal Seizures." In Avery's Diseases of the Newborn: Tenth Edition, 961-970.e4. December 27, 2017.  Full Text
    • Van Karnebeek, C, and Gospe, SM. "Disorders of Pyridoxine Metabolism." In Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease: Fifth Edition, 541-555. November 13, 2014.  Full Text
    • Gospe, SM. "Pyridoxine-dependent epilepsy." In The Causes of Epilepsy: Common and Uncommon Causes in Adults and Children, 237-241. January 1, 2011.  Full Text
    • Firestone, JA, and Gospe, SM. "Organic solvents." In Clinical Neurotoxicology: Syndromes, Substances, Environments, 401-414. December 1, 2009.  Full Text
    • Gospe, SM. "Other organic chemicals." In Clinical Neurotoxicology: Syndromes, Substances, Environments, 415-420. December 1, 2009.  Full Text

  • Conference Papers

    • Tuschl, K, Clayton, PT, Jr, GSM, Shamshad, G, Ibrahim, S, Singhi, P, Ribeiro, RT, Zaki, MS, Luz del Rosario, M, Dyack, S, Price, V, Wevers, RA, and Mills, PB. "The first inborn error of manganese metabolism caused by mutations in SLC30A10, a newly identified manganese transporter." February 27, 2013.  Link to Item
    • Ishak, GE, Friedman, SD, Poliakov, AV, Poliachik, SL, Shaw, DWW, and Gospe, SM. "CALLOSAL AND WHITE MATTER FEATURES IN PYRIDOXINE-DEPENDENT EPILEPSY (PDE)." September 2012.  Link to Item
    • Tuschl, K, Clayton, PT, Jr, GSM, Shamshad, G, Ibrahim, S, Singhi, P, Ribeiro, RT, Zaki, MS, del Rosario, ML, Dyack, S, Price, V, Wevers, RA, and Mills, PB. "Syndrome of hepatic cirrhosis, dystonia, polycythaemia and hypermanganesaemia - Caused by mutations in SLC30A10, a manganese transporter in man." June 2012.  Link to Item
    • Jansen, LA, Hevner, RF, Roden, WH, Hahn, S, Jung, S, and Gospe, SM. "Disruption of Antiquitin (ALDH7A1) Function in Astrocytes is Associated with Neuronal Migration Defects in Pyridoxine-Dependent Epilepsy." 2012.  Link to Item
    • Segal, MM, Williams, MS, Gropman, AL, Torres, AR, Forsyth, R, Connolly, AM, El-Hattab, AW, Perlman, SJ, Samanta, D, Parikh, S, Pavlakis, SG, Betensky, RA, and Jr, GSM. "An Evidence-Based, Open-Database Approach to Pediatric Neurologic Diagnostic Decision Support Reduces Errors in Diagnosis and Testing." 2012.  Link to Item
    • Tuschl, K, Mills, PB, Shamshad, G, Singhi, P, Ribeiro, R, Zaki, M, Dyack, S, Gospe, SM, Wevers, R, and Clayton, PT. "SYNDROME OF HYPERMANGANESAEMIA, DYSTONIA AND LIVER CIRRHOSIS-PHENOTYPES OF 14 NEW PATIENTS." 2011.  Link to Item
    • Richardson, RC, Tarleton, JC, Bird, TD, and Gospe, SM. "Variable Heritability of Truncating CLCN1 Mutations in Myotonia Congenita." March 17, 2009.  Link to Item
    • Hahn, SH, Sadilkova, K, Kerfoot, S, and Jr, GSM. "BIOMARKER ANALYSIS FOR PYRIDOXINE DEPENDENT SEIZURES AND FOLINIC ACID RESPONSIVE SEIZURES." 2009.  Link to Item
    • Zerr, DM, Blume, HK, DelBeccaro, MA, Gospe, SM, Allpress, A, and Christakis, DA. "Nonfebrile provoked seizures: A unique seizure category?." April 2004.  Link to Item
    • Gospe, SM, and Zhou, SS. "Prenatal Exposure to Toluene Results in Abnormal Neuronal Proliferation and Migration." : Springer Nature, April 1999.  Full Text
    • Bespalova, IN, Adkins, S, Malygina, NA, Markova, ED, Dietrich, M, Pranzatelli, M, Gospe, SM, and Burmeister, M. "Cystatin B mutations in Unverricht-Lundborg Progressive Myoclonus Epilepsy (EPM1) patients: intrafamilial instability and genotype/phenotype correlations." October 1997.  Link to Item
    • Gospe, SM, and Hecht, ST. "Longitudinal MRI findings in pyridoxine-dependent seizures." September 1997.  Link to Item
    • Gospe, SM, ElSchahawi, M, Shanske, S, Bruno, C, DiMauro, S, Hoye, E, Walsh, DA, and Gorin, FA. "Asymptomatic patient with myophosphorylase deficiency, high CK, and the L396P mutation." March 1997.  Link to Item
    • Phillips, SA, Carter, J, Jenkins, D, Lee, EK, and Gospe, SM. "Etiology of status epilepticus in institutionalized developmentally disabled clients." February 1996.  Link to Item
    • GOSPE, SM, ZHOU, SS, and PINKERTON, KE. "EFFECTS OF ENVIRONMENTAL TOBACCO-SMOKE EXPOSURE IN-UTERO AND OR POSTNATALLY ON BRAIN-DEVELOPMENT." April 1995.  Link to Item
    • GOSPE, SM, GIETZEN, DW, SUMMERS, PJ, LUNETTA, JM, SELHUB, J, ELLIS, WG, and CLIFFORD, AJ. "FOLATE-DEPLETION AND FOOD SPILLING IN MICE - NEUROCHEMICAL AND NEUROPATHOLOGICAL STUDIES." March 18, 1994.  Link to Item
    • GOSPE, SM, SAEED, DB, ZHOU, SS, and ZEMAN, FJ. "EFFECTS OF PRENATAL TOLUENE EXPOSURE ON BRAIN-DEVELOPMENT - AN ANIMAL-MODEL OF TOLUENE EMBRYOPATHY (FETAL SOLVENTS SYNDROME)." September 1991.  Link to Item
    • GOSPE, SM, SAEED, DB, and ZEMAN, FJ. "DEVELOPMENT OF AN ANIMAL-MODEL OF TOLUENE EMBRYOPATHY (FETAL SOLVENTS SYNDROME)." April 1991.  Link to Item
    • GOSPE, SM, and GABOR, AJ. "ELECTROENCEPHALOGRAPHY LABORATORY DIAGNOSIS OF PROLONGED QT INTERVAL." September 1990.  Link to Item
    • GOSPE, SM, LAZARO, RP, LAVA, NS, GROOTSCHOLTEN, DM, SCOTT, MO, and FISCHBECK, KH. "FAMILIAL X-LINKED MYALGIA AND CRAMPS - A NONPROGRESSIVE MYOPATHY ASSOCIATED WITH A DELETION IN THE DYSTROPHIN GENE." September 1989.  Link to Item
    • GOSPE, SM, MORA, BJ, and GLICK, SD. "MEASUREMENT OF SPONTANEOUS ROTATIONAL MOVEMENT (CIRCLING) IN NORMAL-CHILDREN." August 1988.  Link to Item
    • GOSPE, SM, GLAZE, DG, HRACHOVY, RA, and FROST, JD. "DEVELOPMENT OF INFANTILE SPASMS IN SURVIVORS OF NEAR-DROWNING OCCURRING AFTER THE 1ST YEAR OF LIFE." April 1986.  Link to Item
    • GOSPE, SM, ARMSTRONG, DL, and GRESIK, MV. "PROFOUND CONGESTIVE HEART-FAILURE AS THE PRESENTATION OF CENTRONUCLEAR MYOPATHY." 1985.  Link to Item
    • GOSPE, SM, and WILSON, WA. "DOPAMINE INHIBITS BURST-FIRING IN CELL R 15 OF APLYSIA-CALIFORNICA - STUDIES OF AGONIST AND ANTAGONIST COMPOUNDS." 1980.  Link to Item

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