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James Howard Tonsgard

TitleClinical Associate Professor
InstitutionUniversity of Chicago
DepartmentPediatrics-Neurology
AddressChicago IL 60637
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    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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    PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Nakhate V, Ly I, Muzikansky A, Rapalino O, Allen JC, Blakeley JO, Campian JL, Clapp DW, Dhall G, Jain RK, Karajannis MA, Packer RJ, Tonsgard J, Ullrich NJ, Korf BR, Fisher MJ, Plotkin SR, Neurofibromatosis Clinical Trials Consortium. Effect of bevacizumab on non-target intracranial meningiomas and non-vestibular schwannomas in NF2-related schwannomatosis: NF104. J Neurooncol. 2025 May 28. PMID: 40434540.
      Citations:    Fields:    
    2. Al Ghriwati N, Struemph K, Martin S, Little P, Baker M, Levine J, MacKenzie C, Tonsgard J, Schorry EK, Walsh KS, Wolters PL. Development of patient reported outcome measures assessing tumor pain intensity and tumor pain interference for individuals with neurofibromatosis type 1 and plexiform neurofibromas: qualitative findings. J Patient Rep Outcomes. 2025 Apr 30; 9(1):46. PMID: 40304934; PMCID: PMC12044096.
      Citations:    Fields:    
    3. Plotkin SR, Allen J, Dhall G, Campian JL, Clapp DW, Fisher MJ, Jain RK, Tonsgard J, Ullrich NJ, Thomas C, Edwards LJ, Korf B, Packer R, Karajannis MA, Blakeley JO. Multicenter, prospective, phase II study of maintenance bevacizumab for children and adults with NF2-related schwannomatosis and progressive vestibular schwannoma. Neuro Oncol. 2023 08 03; 25(8):1498-1506. PMID: 37010875; PMCID: PMC10398799.
      Citations: 11     Fields:    Translation:HumansCTClinical Trials
    4. Wolters PL, Vranceanu AM, Thompson HL, Martin S, Merker VL, Baldwin A, Barnett C, Koetsier KS, Hingtgen CM, Funes CJ, Tonsgard JH, Schorry EK, Allen T, Smith T, Franklin B, Reeve S, REiNS International Collaboration. Current Recommendations for Patient-Reported Outcome Measures Assessing Domains of Quality of Life in Neurofibromatosis Clinical Trials. Neurology. 2021 08 17; 97(7 Suppl 1):S50-S63. PMID: 34230198; PMCID: PMC8594008.
      Citations: 12     Fields:    Translation:Humans
    5. Weiss BD, Wolters PL, Plotkin SR, Widemann BC, Tonsgard JH, Blakeley J, Allen JC, Schorry E, Korf B, Robison NJ, Goldman S, Vinks AA, Emoto C, Fukuda T, Robinson CT, Cutter G, Edwards L, Dombi E, Ratner N, Packer R, Fisher MJ. NF106: A Neurofibromatosis Clinical Trials Consortium Phase II Trial of the MEK Inhibitor Mirdametinib (PD-0325901) in Adolescents and Adults With NF1-Related Plexiform Neurofibromas. J Clin Oncol. 2021 03 01; 39(7):797-806. PMID: 33507822; PMCID: PMC8078274.
      Citations: 56     Fields:    Translation:HumansCTClinical Trials
    6. Polster SP, Dougherty MC, Zeineddine HA, Smith HL, MacKenzie C, Pytel P, Yang CW, Tonsgard JH, Warnke PC, Frim DM, Lyne SB. Dural Ectasia in Neurofibromatosis 1: Case Series, Management, and Review. Neurosurgery. 2020 05 01; 86(5):646-655. PMID: 31350851.
      Citations: 5     Fields:    Translation:Humans
    7. Payne JM, Haebich KM, MacKenzie R, Walsh KS, Hearps SJC, Coghill D, Barton B, Pride NA, Ullrich NJ, Tonsgard JH, Viskochil D, Schorry EK, Klesse L, Fisher MJ, Gutmann DH, Rosser T, Packer RJ, Korf B, Acosta MT, Bellgrove MA, North KN. Cognition, ADHD Symptoms, and Functional Impairment in Children and Adolescents With Neurofibromatosis Type 1. J Atten Disord. 2021 06; 25(8):1177-1186. PMID: 31838937.
      Citations: 17     Fields:    Translation:Humans
    8. Payne JM, Hearps SJC, Walsh KS, Paltin I, Barton B, Ullrich NJ, Haebich KM, Coghill D, Gioia GA, Cantor A, Cutter G, Tonsgard JH, Viskochil D, Rey-Casserly C, Schorry EK, Ackerson JD, Klesse L, Fisher MJ, Gutmann DH, Rosser T, Packer RJ, Korf B, Acosta MT, North KN, NF Clinical Trials Consortium. Reproducibility of cognitive endpoints in clinical trials: lessons from neurofibromatosis type 1. Ann Clin Transl Neurol. 2019 12; 6(12):2555-2565. PMID: 31797581; PMCID: PMC6917317.
      Citations: 15     Fields:    Translation:HumansCTClinical Trials
    9. Plotkin SR, Duda DG, Muzikansky A, Allen J, Blakeley J, Rosser T, Campian JL, Clapp DW, Fisher MJ, Tonsgard J, Ullrich N, Thomas C, Cutter G, Korf B, Packer R, Karajannis MA. Multicenter, Prospective, Phase II and Biomarker Study of High-Dose Bevacizumab as Induction Therapy in Patients With Neurofibromatosis Type 2 and Progressive Vestibular Schwannoma. J Clin Oncol. 2019 12 10; 37(35):3446-3454. PMID: 31626572; PMCID: PMC7098833.
      Citations: 52     Fields:    Translation:HumansCTClinical Trials
    10. Payne JM, Barton B, Ullrich NJ, Cantor A, Hearps SJ, Cutter G, Rosser T, Walsh KS, Gioia GA, Wolters PL, Tonsgard J, Schorry E, Viskochil D, Klesse L, Fisher M, Gutmann DH, Silva AJ, Hunter SJ, Rey-Casserly C, Cantor NL, Byars AW, Stavinoha PL, Ackerson JD, Armstrong CL, Isenberg J, O'Neil SH, Packer RJ, Korf B, Acosta MT, North KN, NF Clinical Trials Consortium. Randomized placebo-controlled study of lovastatin in children with neurofibromatosis type 1. Neurology. 2016 Dec 13; 87(24):2575-2584. PMID: 27956565; PMCID: PMC5207004.
      Citations: 45     Fields:    Translation:Humans
    11. Wolters PL, Martin S, Merker VL, Tonsgard JH, Solomon SE, Baldwin A, Bergner AL, Walsh K, Thompson HL, Gardner KL, Hingtgen CM, Schorry E, Dudley WN, Franklin B, REiNS International Collaboration. Patient-reported outcomes of pain and physical functioning in neurofibromatosis clinical trials. Neurology. 2016 Aug 16; 87(7 Suppl 1):S4-S12. PMID: 27527648; PMCID: PMC5578357.
      Citations: 21     Fields:    Translation:Humans
    12. Rojnueangnit K, Xie J, Gomes A, Sharp A, Callens T, Chen Y, Liu Y, Cochran M, Abbott MA, Atkin J, Babovic-Vuksanovic D, Barnett CP, Crenshaw M, Bartholomew DW, Basel L, Bellus G, Ben-Shachar S, Bialer MG, Bick D, Blumberg B, Cortes F, David KL, Destree A, Earl D, Escobar L, Eswara M, Frayling IM, Frydman M, Gardner K, Gripp KW, Heyrman K, Ibrahim J, Janssens S, Keena BA, Llano-Rivas I, Leppig K, McDonald M, Misra VK, Mulbury J, Narayanan V, Orenstein N, Galvin-Parton P, Pedro H, Pivnick EK, Powell CM, Randolph L, Raskin S, Rosell J, Rubin K, Seashore M, Schaaf CP, Scheuerle A, Schultz M, Schorry E, Schnur R, Siqveland E, Tkachuk A, Tonsgard J, Upadhyaya M, Verma IC, Wallace S, Williams C, Zackai E, Zonana J, Lazaro C, Claes K, Korf B, Legius E, Messiaen L, Duat-Rodriguez A, Ezquieta B, Hernández-Chico C, Martin Y. High Incidence of Noonan Syndrome Features Including Short Stature and Pulmonic Stenosis in Patients carrying NF1 Missense Mutations Affecting p.Arg1809: Genotype-Phenotype Correlation. Hum Mutat. 2015 Nov; 36(11):1052-63. PMID: 26178382; PMCID: PMC5049609.
      Citations: 86     Fields:    Translation:Humans
    13. Weiss B, Widemann BC, Wolters P, Dombi E, Vinks A, Cantor A, Perentesis J, Schorry E, Ullrich N, Gutmann DH, Tonsgard J, Viskochil D, Korf B, Packer RJ, Fisher MJ. Sirolimus for progressive neurofibromatosis type 1-associated plexiform neurofibromas: a neurofibromatosis Clinical Trials Consortium phase II study. Neuro Oncol. 2015 Apr; 17(4):596-603. PMID: 25314964; PMCID: PMC4483073.
      Citations: 64     Fields:    Translation:HumansCTClinical Trials
    14. Wolters PL, Martin S, Merker VL, Gardner KL, Hingtgen CM, Tonsgard JH, Schorry EK, Baldwin A, REiNS International Collaboration. Patient-reported outcomes in neurofibromatosis and schwannomatosis clinical trials. Neurology. 2013 Nov 19; 81(21 Suppl 1):S6-14. PMID: 24249806; PMCID: PMC3908341.
      Citations: 24     Fields:    Translation:Humans
    15. Klein-Tasman BP, Janke KM, Luo W, Casnar CL, Hunter SJ, Tonsgard J, Trapane P, van der Fluit F, Kais LA. Cognitive and psychosocial phenotype of young children with neurofibromatosis-1. J Int Neuropsychol Soc. 2014 Jan; 20(1):88-98. PMID: 24229851; PMCID: PMC4249943.
      Citations: 15     Fields:    Translation:Humans
    16. Stricker TP, Henriksen KJ, Tonsgard JH, Montag AG, Krausz TN, Pytel P. Expression profiling of 519 kinase genes in matched malignant peripheral nerve sheath tumor/plexiform neurofibroma samples is discriminatory and identifies mitotic regulators BUB1B, PBK and NEK2 as overexpressed with transformation. Mod Pathol. 2013 Jul; 26(7):930-43. PMID: 23370767.
      Citations: 21     Fields:    Translation:Humans
    17. Pytel P, Karrison T, Tonsgard JH, Krausz T, Montag AG. Neoplasms with schwannian differentiation express transcription factors known to regulate normal schwann cell development. Int J Surg Pathol. 2010 Dec; 18(6):449-57. PMID: 20034979.
      Citations: 7     Fields:    Translation:HumansCells
    18. Evans DG, Kalamarides M, Hunter-Schaedle K, Blakeley J, Allen J, Babovic-Vuskanovic D, Belzberg A, Bollag G, Chen R, DiTomaso E, Golfinos J, Harris G, Jacob A, Kalpana G, Karajannis M, Korf B, Kurzrock R, Law M, McClatchey A, Packer R, Roehm P, Rubenstein A, Slattery W, Tonsgard JH, Welling DB, Widemann B, Yohay K, Giovannini M. Consensus recommendations to accelerate clinical trials for neurofibromatosis type 2. Clin Cancer Res. 2009 Aug 15; 15(16):5032-5039. PMID: 19671848; PMCID: PMC4513640.
      Citations: 27     Fields:    Translation:HumansAnimals
    19. Tonsgard JH. Clinical manifestations and management of neurofibromatosis type 1. Semin Pediatr Neurol. 2006 Mar; 13(1):2-7. PMID: 16818170.
      Citations: 78     Fields:    Translation:Humans
    20. Lee MC, Frank JI, Kahana M, Tonsgard JH, Frim DM. Decompressive hemicraniectomy in a 6-year-old male after unilateral hemispheric stroke. Case report and review. Pediatr Neurosurg. 2003 Apr; 38(4):181-5. PMID: 12646736.
      Citations: 10     Fields:    Translation:Humans
    21. Miller MB, Tonsgard JH, Soltani K. Late-onset neurofibromatosis in a liver transplant recipient. Int J Dermatol. 2000 May; 39(5):376-9. PMID: 10849133.
      Citations:    Fields:    Translation:Humans
    22. Nadig M, Munshi I, Short MP, Tonsgard JH, Sullivan C, Frim DM. A child with neurofibromatosis-1 and a lumbar epidural arteriovenous malformation. J Child Neurol. 2000 Apr; 15(4):273-5. PMID: 10805199.
      Citations: 2     Fields:    Translation:Humans
    23. Stevenson DA, Birch PH, Friedman JM, Viskochil DH, Balestrazzi P, Boni S, Buske A, Korf BR, Niimura M, Pivnick EK, Schorry EK, Short MP, Tenconi R, Tonsgard JH, Carey JC. Descriptive analysis of tibial pseudarthrosis in patients with neurofibromatosis 1. Am J Med Genet. 1999 Jun 11; 84(5):413-9. PMID: 10360395.
      Citations: 36     Fields:    Translation:Humans
    24. Tonsgard JH, Kwak SM, Short MP, Dachman AH. CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions. Neurology. 1998 Jun; 50(6):1755-60. PMID: 9633723.
      Citations: 45     Fields:    Translation:Humans
    25. Alexander JJ, Snyder A, Tonsgard JH. Omega-oxidation of monocarboxylic acids in rat brain. Neurochem Res. 1998 Feb; 23(2):227-33. PMID: 9475518.
      Citations: 5     Fields:    Translation:AnimalsCells
    26. Tonsgard JH, Yelavarthi KK, Cushner S, Short MP, Lindgren V. Do NF1 gene deletions result in a characteristic phenotype? Am J Med Genet. 1997 Nov 28; 73(1):80-6. PMID: 9375928.
      Citations: 17     Fields:    Translation:Humans
    27. Alexander JJ, Banerjee P, Dawson G, Tonsgard JH. Hyperammonemia increases serotonin 1A receptor expression in both rat hippocampus and a transfected hippocampal cell line, HN2-5. J Neurosci Res. 1995 May 01; 41(1):105-10. PMID: 7674372.
      Citations: 1     Fields:    Translation:AnimalsCells
    28. Kossak BD, Schmidt-Sommerfeld E, Schoeller DA, Rinaldo P, Penn D, Tonsgard JH. Impaired fatty acid oxidation in children on valproic acid and the effect of L-carnitine. Neurology. 1993 Nov; 43(11):2362-8. PMID: 8232957.
      Citations: 5     Fields:    Translation:HumansCells
    29. Tonsgard JH, Oesterle CS. The ophthalmologic presentation of NF-2 in childhood. J Pediatr Ophthalmol Strabismus. 1993 Sep-Oct; 30(5):327-30. PMID: 8254451.
      Citations:    Fields:    Translation:HumansCells
    30. Kundu RK, Getz GS, Tonsgard JH. Induction of (omega-1)-oxidation of monocarboxylic acids by acetylsalicylic acid. J Lipid Res. 1993 Jul; 34(7):1187-99. PMID: 8371066.
      Citations: 4     Fields:    Translation:AnimalsCells
    31. Taravath S, Tonsgard JH. Cerebral malformations in Carpenter syndrome. Pediatr Neurol. 1993 May-Jun; 9(3):230-4. PMID: 8352858.
      Citations: 7     Fields:    Translation:Humans
    32. Aoyama T, Uchida Y, Kelley RI, Marble M, Hofman K, Tonsgard JH, Rhead WJ, Hashimoto T. A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase. Biochem Biophys Res Commun. 1993 Mar 31; 191(3):1369-72. PMID: 8466512.
      Citations: 49     Fields:    Translation:HumansCells
    33. Maselli RA, Burnett ME, Tonsgard JH. In vitro microelectrode study of neuromuscular transmission in a case of botulism. Muscle Nerve. 1992 Mar; 15(3):273-6. PMID: 1313544.
      Citations: 1     Fields:    Translation:HumansCells
    34. Kundu RK, Tonsgard JH, Getz GS. Induction of omega-oxidation of monocarboxylic acids in rats by acetylsalicylic acid. J Clin Invest. 1991 Dec; 88(6):1865-72. PMID: 1752948; PMCID: PMC295754.
      Citations: 3     Fields:    Translation:AnimalsCells
    35. Tonsgard JH, Meredith SC. Characterization of the binding sites for dicarboxylic acids on bovine serum albumin. Biochem J. 1991 Jun 15; 276 ( Pt 3):569-75. PMID: 2064600; PMCID: PMC1151043.
      Citations: 3     Fields:    Translation:Cells
    36. Tonsgard JH, Stephens JK, Rhead WJ, Penn D, Horwitz AL, Kirschner BS, Whitington PF, Berger S, Tripp ME. Defect in fatty acid oxidation: laboratory and pathologic findings in a patient. Pediatr Neurol. 1991 Mar-Apr; 7(2):125-30. PMID: 2059253.
      Citations: 2     Fields:    Translation:HumansCells
    37. Tonsgard JH, Tung B, Kornafel KS, Getz GS. Environmentally induced differential amplification of mitochondrial populations. Biochem J. 1990 Sep 01; 270(2):511-8. PMID: 2144730; PMCID: PMC1131752.
      Citations:    Fields:    Translation:AnimalsCells
    38. Hammond DN, Lee HJ, Tonsgard JH, Wainer BH. Development and characterization of clonal cell lines derived from septal cholinergic neurons. Brain Res. 1990 Apr 02; 512(2):190-200. PMID: 2354356.
      Citations: 9     Fields:    Translation:AnimalsCells
    39. Tonsgard JH. Effect of Reye's syndrome serum on the ultrastructure of isolated liver mitochondria. Lab Invest. 1989 Apr; 60(4):568-73. PMID: 2496274.
      Citations:    Fields:    Translation:HumansCells
    40. Tonsgard JH, Mendelson SA, Meredith SC. Binding of straight-chain saturated dicarboxylic acids to albumin. J Clin Invest. 1988 Nov; 82(5):1567-73. PMID: 3183053; PMCID: PMC442724.
      Citations: 3     Fields:    Translation:HumansCells
    41. Tonsgard JH, Harwicke N, Levine SC. Kluver-Bucy syndrome in children. Pediatr Neurol. 1987 May-Jun; 3(3):162-5. PMID: 3508062.
      Citations: 3     Fields:    Translation:Humans
    42. Hammond DN, Wainer BH, Tonsgard JH, Heller A. Neuronal properties of clonal hybrid cell lines derived from central cholinergic neurons. Science. 1986 Dec 05; 234(4781):1237-40. PMID: 3775382.
      Citations: 17     Fields:    Translation:AnimalsCells
    43. Tonsgard JH. Serum dicarboxylic acids in patients with Reye syndrome. J Pediatr. 1986 Sep; 109(3):440-5. PMID: 3746531.
      Citations: 13     Fields:    Translation:Humans
    44. Tonsgard JH, Getz GS. Effect of Reye's syndrome serum on isolated chinchilla liver mitochondria. J Clin Invest. 1985 Aug; 76(2):816-25. PMID: 4031073; PMCID: PMC423909.
      Citations: 15     Fields:    Translation:HumansAnimalsCells
    45. Tonsgard JH. Urinary dicarboxylic acids in Reye syndrome. J Pediatr. 1985 Jul; 107(1):79-84. PMID: 4009343.
      Citations: 6     Fields:    Translation:Humans
    46. Tonsgard JH, Huttenlocher PR, Thisted RA. Lactic acidemia in Reye's syndrome. Pediatrics. 1982 Jan; 69(1):64-9. PMID: 7054762.
      Citations: 3     Fields:    Translation:Humans
    47. Tonsgard JH, Huttenlocher PR. Salicylates and Reye's syndrome. Pediatrics. 1981 Nov; 68(5):747-8. PMID: 7312481.
      Citations: 3     Fields:    Translation:Humans
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