Colorado PROFILES, The Colorado Clinical and Translational Sciences Institute (CCTSI)
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Timothy A. Benke

TitleProfessor
InstitutionUniversity of Colorado Denver - Anschutz Medical Campus
DepartmentSOM-PEDS

    Collapse Research 
    Collapse research activities and funding
    U24NS133991     (BENNETT, JEFFREY L)Jul 15, 2023 - Jun 30, 2028
    NIH
    University of Colorado Rocky Mountain NeuroNEXT (UNCOMON) Clinical Research Consortium.
    Role: Co-Principal Investigator

    U01NS114312     (BENKE, TIMOTHY A ;DOWNS, JENNY ;LEONARD, HELEN M;MARSH, ERIC D)Feb 15, 2021 - Jan 31, 2026
    NIH
    Multi-Site Validation of Biomarkers and Core Clinical Outcome Measures for Clinical Trials Readiness in CDKL5 Deficiency Disorder
    Role: Principal Investigator

    R21NS112770     (BENKE, TIMOTHY A)Apr 1, 2020 - Sep 30, 2021
    NIH
    Pilot studies to characterize CDKL5 inhibitors
    Role: Principal Investigator

    R21NS101288     (BENKE, TIMOTHY A)Sep 15, 2017 - Aug 31, 2021
    NIH
    Exploratory determination of the role of L-type calcium channels in mediating abnormal plasticity and behavior after early life seizures
    Role: Principal Investigator

    K12NS089417     (BROOKS-KAYAL, AMY R.)Jul 1, 2015 - Jun 30, 2020
    NIH
    Colorado Neurological Sciences Academic Development Award (NSADA)
    Role: Co-Principal Investigator

    U10NS077277     (VOLLMER, TIMOTHY LEE)Sep 30, 2011 - Dec 31, 2019
    NIH
    Rocky Mountain Network for Neuroscience Clinical Studies (RMNNCS)
    Role: Co-Principal Investigator

    R01NS076577     (BENKE, TIMOTHY A)Sep 1, 2011 - Jun 30, 2017
    NIH
    Molecular mechanisms linking early life seizures, autism and intellectual disabil
    Role: Principal Investigator

    K02NS056090     (BENKE, TIMOTHY A)May 1, 2007 - Apr 30, 2012
    NIH
    Impact of early life seizures on glutamate receptors and synaptic function
    Role: Principal Investigator

    K08NS041267     (BENKE, TIMOTHY A)Sep 6, 2001 - Aug 31, 2007
    NIH
    Impact of Early-Life Seizures on Synaptic Plasticity
    Role: Principal Investigator

    F32NS009894     (BENKE, TIMOTHY A)Dec 1, 1995
    NIH
    PHARMACOLOGICAL DENDRITIC GLUTAMATE CHANGES IN LTP
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse selected publications
    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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    1. Saldaris JM, Jacoby P, Marsh ED, Suter B, Leonard H, Olson HE, Rajaraman R, Pestana-Knight E, Weisenberg J, Price D, Drummond C, Benke TA, Demarest S, Downs J. Adapting a measure of gross motor skills for individuals with CDKL5 deficiency disorder: A psychometric study. Epilepsy Res. 2024 Feb; 200:107287. PMID: 38237219.
      View in: PubMed
    2. Neul JL, Percy AK, Benke TA, Berry-Kravis EM, Glaze DG, Peters SU, Marsh ED, An D, Bishop KM, Youakim JM. Trofinetide Treatment Demonstrates a Benefit Over Placebo for the Ability to Communicate in Rett Syndrome. Pediatr Neurol. 2024 Mar; 152:63-72. PMID: 38232652.
      View in: PubMed
    3. Rong M, Benke T, Zulfiqar Ali Q, Aledo-Serrano ?, Bayat A, Rossi A, Devinsky O, Qaiser F, Ali AS, Fasano A, Bassett AS, Andrade DM. Adult Phenotype of SYNGAP1-DEE. Neurol Genet. 2023 Dec; 9(6):e200105. PMID: 38045990.
      View in: PubMed
    4. XiangWei W, Perszyk RE, Liu N, Xu Y, Bhattacharya S, Shaulsky GH, Smith-Hicks C, Fatemi A, Fry AE, Chandler K, Wang T, Vogt J, Cohen JS, Paciorkowski AR, Poduri A, Zhang Y, Wang S, Wang Y, Zhai Q, Fang F, Leng J, Garber K, Myers SJ, Jauss RT, Park KL, Benke TA, Lemke JR, Yuan H, Jiang Y, Traynelis SF. Clinical and functional consequences of GRIA variants in patients with neurological diseases. Cell Mol Life Sci. 2023 Nov 03; 80(11):345. PMID: 37921875.
      View in: PubMed
    5. Neul JL, Benke TA, Marsh ED, Suter B, Silveira L, Fu C, Peters SU, Percy AK. Top caregiver concerns in Rett syndrome and related disorders: data from the US natural history study. J Neurodev Disord. 2023 10 13; 15(1):33. PMID: 37833681.
      View in: PubMed
    6. Fang X, Baggett LM, Caylor RC, Percy AK, Neul JL, Lane JB, Glaze DG, Benke TA, Marsh ED, Motil KJ, Barrish JO, Annese FE, Skinner SA. Parental age effects and Rett syndrome. Am J Med Genet A. 2024 Feb; 194(2):160-173. PMID: 37768187.
      View in: PubMed
    7. Daniels C, Greene C, Smith L, Pestana-Knight E, Demarest S, Zhang B, Benke TA, Poduri A, Olson HE. CDKL5 deficiency disorder and other infantile-onset genetic epilepsies. Dev Med Child Neurol. 2024 Apr; 66(4):456-468. PMID: 37771170.
      View in: PubMed
    8. Ziniel SI, Mackie A, Saldaris J, Leonard H, Jacoby P, Marsh ED, Suter B, Pestana-Knight E, Olson HE, Price D, Weisenberg J, Rajaraman R, VanderVeen G, Benke TA, Downs J, Demarest S. The development, content and response process validation of a caregiver-reported severity measure for CDKL5 deficiency disorder. Epilepsy Res. 2023 11; 197:107231. PMID: 37751639.
      View in: PubMed
    9. Camp CR, Vlachos A, Kl?ckner C, Krey I, Banke TG, Shariatzadeh N, Ruggiero SM, Galer P, Park KL, Caccavano A, Kimmel S, Yuan X, Yuan H, Helbig I, Benke TA, Lemke JR, Pelkey KA, McBain CJ, Traynelis SF. Loss of Grin2a causes a transient delay in the electrophysiological maturation of hippocampal parvalbumin interneurons. Commun Biol. 2023 09 19; 6(1):952. PMID: 37723282.
      View in: PubMed
    10. Myers SJ, Yuan H, Perszyk RE, Zhang J, Kim S, Nocilla KA, Allen JP, Bain JM, Lemke JR, Lal D, Benke TA, Traynelis SF. Classification of missense variants in the N-methyl-d-aspartate receptor GRIN gene family as gain- or loss-of-function. Hum Mol Genet. 2023 09 16; 32(19):2857-2871. PMID: 37369021.
      View in: PubMed
    11. Percy AK, Neul JL, Benke TA, Marsh ED, Glaze DG. A review of the Rett Syndrome Behaviour Questionnaire and its utilization in the assessment of symptoms associated with Rett syndrome. Front Pediatr. 2023; 11:1229553. PMID: 37635789.
      View in: PubMed
    12. Castano A, Silvestre M, Wells CI, Sanderson JL, Ferrer CA, Ong HW, Lang Y, Richardson W, Silvaroli JA, Bashore FM, Smith JL, Genereux IM, Dempster K, Drewry DH, Pabla NS, Bullock AN, Benke TA, Ultanir SK, Axtman AD. Discovery and characterization of a specific inhibitor of serine-threonine kinase cyclin-dependent kinase-like 5 (CDKL5) demonstrates role in hippocampal CA1 physiology. Elife. 2023 07 25; 12. PMID: 37490324.
      View in: PubMed
    13. Castano A, Silvestre M, Wells CI, Sanderson JL, Ferrer CA, Ong HW, Lang Y, Richardson W, Silvaroli JA, Bashore FM, Smith JL, Genereux IM, Dempster K, Drewry DH, Pabla NS, Bullock AN, Benke TA, Ultanir S, Axtman AD. Discovery and characterization of a specific inhibitor of serine-threonine kinase cyclin dependent kinase-like 5 (CDKL5) demonstrates role in hippocampal CA1 physiology. bioRxiv. 2023 Jun 16. PMID: 37162893.
      View in: PubMed
    14. Neul JL, Percy AK, Benke TA, Berry-Kravis EM, Glaze DG, Marsh ED, Lin T, Stankovic S, Bishop KM, Youakim JM. Trofinetide for the treatment of Rett syndrome: a randomized phase 3 study. Nat Med. 2023 Jun; 29(6):1468-1475. PMID: 37291210.
      View in: PubMed
    15. Meredith FL, Vu TA, Gehrke B, Benke TA, Dondzillo A, Rennie KJ. Expression of hyperpolarization-activated current (Ih) in zonally defined vestibular calyx terminals of the crista. J Neurophysiol. 2023 06 01; 129(6):1468-1481. PMID: 37198134.
      View in: PubMed
    16. Saldaris J, Leonard H, Wong K, Jacoby P, Spence M, Marsh ED, Benke TA, Demarest S, Downs J. Validating the Communication and Symbolic Behavior Scales-Developmental Profile Infant-Toddler Checklist (CSBS-DP ITC) Beyond Infancy in the CDKL5 Deficiency Disorder. J Autism Dev Disord. 2023 May 15. PMID: 37184758.
      View in: PubMed
    17. Olson HE, Demarest S, Pestana-Knight E, Moosa AN, Zhang X, P?rez-P?rez JR, Weisenberg J, O'Connor Prange E, Marsh ED, Rajaraman RR, Suter B, Katyayan A, Haviland I, Daniels C, Zhang B, Greene C, DeLeo M, Swanson L, Love-Nichols J, Benke T, Harini C, Poduri A. Epileptic spasms in CDKL5 deficiency disorder: Delayed treatment and poor response to first-line therapies. Epilepsia. 2023 07; 64(7):1821-1832. PMID: 37114835.
      View in: PubMed
    18. Neul JL, Benke TA, Marsh ED, Suter B, Silveira L, Fu C, Peters SU, Percy AK. Top Caregiver Concerns in Rett syndrome and related disorders: data from the US Natural History Study. Res Sq. 2023 Mar 20. PMID: 36993737.
      View in: PubMed
    19. Saby JN, Peters SU, Benke TA, Standridge SM, Swanson LC, Lieberman DN, Olson HE, Key AP, Percy AK, Neul JL, Nelson CA, Roberts TPL, Marsh ED. Comparison of evoked potentials across four related developmental encephalopathies. J Neurodev Disord. 2023 03 04; 15(1):10. PMID: 36870948.
      View in: PubMed
    20. Wong K, Junaid M, Demarest S, Saldaris J, Benke TA, Marsh ED, Downs J, Leonard H. Correction to: Factors influencing the attainment of major motor milestones in CDKL5 deficiency disorder. Eur J Hum Genet. 2023 Feb; 31(2):252. PMID: 36224270.
      View in: PubMed
    21. Saldaris JM, Jacoby P, Leonard H, Benke TA, Demarest S, Marsh ED, Downs J. Psychometric properties of QI-Disability in CDKL5 Deficiency Disorder: Establishing readiness for clinical trials. Epilepsy Behav. 2023 02; 139:109069. PMID: 36634535.
      View in: PubMed
    22. Stansauk J, Fidell A, Benke T, Schaffer M, Demarest ST. Analysis of electrocardiograms in individuals with CDKL5 deficiency disorder. Am J Med Genet A. 2023 Jan; 191(1):108-111. PMID: 36372969.
      View in: PubMed
    23. Haviland I, Daniels CI, Greene CA, Drew J, Love-Nichols JA, Swanson LC, Smith L, Nie DA, Benke T, Sheidley BR, Zhang B, Poduri A, Olson HE. Genetic Diagnosis Impacts Medical Management for Pediatric Epilepsies. Pediatr Neurol. 2023 01; 138:71-80. PMID: 36403551.
      View in: PubMed
    24. Sanderson JL, Freund RK, Castano AM, Benke TA, Dell'Acqua ML. The CaV1.2 G406R mutation decreases synaptic inhibition and alters L-type Ca2+ channel-dependent LTP at hippocampal synapses in a mouse model of Timothy Syndrome. Neuropharmacology. 2022 Dec 01; 220:109271. PMID: 36162529.
      View in: PubMed
    25. Wong K, Junaid M, Demarest S, Saldaris J, Benke TA, Marsh ED, Downs J, Leonard H. Factors influencing the attainment of major motor milestones in CDKL5 deficiency disorder. Eur J Hum Genet. 2023 02; 31(2):169-178. PMID: 35978140.
      View in: PubMed
    26. Leonard H, Whitehouse A, Jacoby P, Benke T, Demarest S, Saldaris J, Wong K, Reddihough D, Williams K, Downs J. Quality of life beyond diagnosis in intellectual disability - Latent profiling. Res Dev Disabil. 2022 Oct; 129:104322. PMID: 35939908.
      View in: PubMed
    27. Saby JN, Mulcahey PJ, Zavez AE, Peters SU, Standridge SM, Swanson LC, Lieberman DN, Olson HE, Key AP, Percy AK, Neul JL, Nelson CA, Roberts TPL, Benke TA, Marsh ED. Electrophysiological biomarkers of brain function in CDKL5 deficiency disorder. Brain Commun. 2022; 4(4):fcac197. PMID: 35974796.
      View in: PubMed
    28. Amin S, Monaghan M, Aledo-Serrano A, Bahi-Buisson N, Chin RF, Clarke AJ, Cross JH, Demarest S, Devinsky O, Downs J, Pestana Knight EM, Olson H, Partridge CA, Stuart G, Trivisano M, Zuberi S, Benke TA. International Consensus Recommendations for the Assessment and Management of Individuals With CDKL5 Deficiency Disorder. Front Neurol. 2022; 13:874695. PMID: 35795799.
      View in: PubMed
    29. Buchanan CB, Stallworth JL, Joy AE, Dixon RE, Scott AE, Beisang AA, Benke TA, Glaze DG, Haas RH, Heydemann PT, Jones MD, Lane JB, Lieberman DN, Marsh ED, Neul JL, Peters SU, Ryther RC, Skinner SA, Standridge SM, Kaufmann WE, Percy AK. Anxiety-like behavior and anxiolytic treatment in the Rett syndrome natural history study. J Neurodev Disord. 2022 05 14; 14(1):31. PMID: 35568815.
      View in: PubMed
    30. Knight EMP, Amin S, Bahi-Buisson N, Benke TA, Cross JH, Demarest ST, Olson HE, Specchio N, Fleming TR, Aimetti AA, Gasior M, Devinsky O. Safety and efficacy of ganaxolone in patients with CDKL5 deficiency disorder: results from the double-blind phase of a randomised, placebo-controlled, phase 3 trial. Lancet Neurol. 2022 05; 21(5):417-427. PMID: 35429480.
      View in: PubMed
    31. Leonard H, Downs J, Benke TA, Swanson L, Olson H, Demarest S. CDKL5 deficiency disorder: clinical features, diagnosis, and management. Lancet Neurol. 2022 06; 21(6):563-576. PMID: 35483386.
      View in: PubMed
    32. Saldaris J, Leonard H, Jacoby P, Marsh ED, Benke TA, Demarest S, Downs J. Initial Validation and Reliability of the CDKL5 Deficiency Disorder Hand Function Scale (CDD-Hand). J Child Neurol. 2022 05; 37(6):541-547. PMID: 35422141.
      View in: PubMed
    33. Downs J, Jacoby P, Saldaris J, Leonard H, Benke T, Marsh E, Demarest S. Negative impact of insomnia and daytime sleepiness on quality of life in individuals with the cyclin-dependent kinase-like 5 deficiency disorder. J Sleep Res. 2022 10; 31(5):e13600. PMID: 35415902.
      View in: PubMed
    34. Fang X, Butler KM, Abidi F, Gass J, Beisang A, Feyma T, Ryther RC, Standridge S, Heydemann P, Jones M, Haas R, Lieberman DN, Marsh ED, Benke TA, Skinner S, Neul JL, Percy AK, Friez MJ, Caylor RC. Analysis of X-inactivation status in a Rett syndrome natural history study cohort. Mol Genet Genomic Med. 2022 05; 10(5):e1917. PMID: 35318820.
      View in: PubMed
    35. Leonard H, Gold W, Samaco R, Sahin M, Benke T, Downs J. Improving clinical trial readiness to accelerate development of new therapeutics for Rett syndrome. Orphanet J Rare Dis. 2022 03 04; 17(1):108. PMID: 35246185.
      View in: PubMed
    36. Demarest S, Marsh R, Treat L, Fisher MP, Dempsey A, Junaid M, Downs J, Leonard H, Benke T, Morris MA. The Lived Experience of Parents' Receiving the Diagnosis of CDKL5 Deficiency Disorder for Their Child. J Child Neurol. 2022 05; 37(6):451-460. PMID: 35196159.
      View in: PubMed
    37. Neul JL, Percy AK, Benke TA, Berry-Kravis EM, Glaze DG, Peters SU, Jones NE, Youakim JM. Design and outcome measures of LAVENDER, a phase 3 study of trofinetide for Rett syndrome. Contemp Clin Trials. 2022 03; 114:106704. PMID: 35149233.
      View in: PubMed
    38. Jacoby P, Whitehouse A, Leonard H, Saldaris J, Demarest S, Benke T, Downs J. Devising a Missing Data Rule for a Quality of Life Questionnaire-A Simulation Study. J Dev Behav Pediatr. 2022 08 01; 43(6):e414-e418. PMID: 35075044.
      View in: PubMed
    39. Motil KJ, Geerts S, Annese F, Neul JL, Benke T, Marsh E, Lieberman D, Skinner SA, Glaze DG, Heydemann P, Beisang A, Standridge S, Ryther R, Lane JB, Edwards L, Percy AK. Anthropometric Measures Correspond with Functional Motor Outcomes in Females with Rett Syndrome. J Pediatr. 2022 05; 244:169-177.e3. PMID: 35063470.
      View in: PubMed
    40. Bigelow LJ, Fiset C, Jarvis JHM, Macleod S, W?hr M, Benke TA, Bernard PB. Early-life seizures modify behavioral response to ultrasonic vocalization playback in adult rats. Epilepsy Behav. 2022 02; 127:108494. PMID: 34954511.
      View in: PubMed
    41. Demarest S, Calhoun J, Eschbach K, Yu HC, Mirsky D, Angione K, Shaikh TH, Carvill GL, Benke TA. Whole-exome sequencing and adrenocorticotropic hormone therapy in individuals with infantile spasms. Dev Med Child Neurol. 2022 05; 64(5):633-640. PMID: 35830182.
      View in: PubMed
    42. Brock D, Fidell A, Thomas J, Juarez-Colunga E, Benke TA, Demarest S. Cerebral Visual Impairment in CDKL5 Deficiency Disorder Correlates With Developmental Achievement. J Child Neurol. 2021 10; 36(11):974-980. PMID: 34547934.
      View in: PubMed
    43. Brock DC, Demarest S, Benke TA. Clinical Trial Design for Disease-Modifying Therapies for Genetic Epilepsies. Neurotherapeutics. 2021 07; 18(3):1445-1457. PMID: 34595733.
      View in: PubMed
    44. Benke TA, Park K, Krey I, Camp CR, Song R, Ramsey AJ, Yuan H, Traynelis SF, Lemke J. Clinical and therapeutic significance of genetic variation in the GRIN gene family encoding NMDARs. Neuropharmacology. 2021 11 01; 199:108805. PMID: 34560056.
      View in: PubMed
    45. Olson HE, Daniels CI, Haviland I, Swanson LC, Greene CA, Denny AMM, Demarest ST, Pestana-Knight E, Zhang X, Moosa AN, Fidell A, Weisenberg JL, Suter B, Fu C, Neul JL, Percy AK, Marsh ED, Benke TA, Poduri A. Current neurologic treatment and emerging therapies in CDKL5 deficiency disorder. J Neurodev Disord. 2021 09 16; 13(1):40. PMID: 34530725.
      View in: PubMed
    46. Saldaris J, Weisenberg J, Pestana-Knight E, Marsh ED, Suter B, Rajaraman R, Heidary G, Olson HE, Devinsky O, Price D, Jacoby P, Leonard H, Benke TA, Demarest S, Downs J. Content Validation of Clinician-Reported Items for a Severity Measure for CDKL5 Deficiency Disorder. J Child Neurol. 2021 10; 36(11):998-1006. PMID: 34378447.
      View in: PubMed
    47. Olson HE, Costantini JG, Swanson LC, Kaufmann WE, Benke TA, Fulton AB, Hansen R, Poduri A, Heidary G. Cerebral visual impairment in CDKL5 deficiency disorder: vision as an outcome measure. Dev Med Child Neurol. 2021 11; 63(11):1308-1315. PMID: 34028805.
      View in: PubMed
    48. Aledo-Serrano ?, G?mez-Iglesias P, Toledano R, Garcia-Pe?as JJ, Garcia-Morales I, Anciones C, Soto-Insuga V, Benke TA, Del Pino I, Gil-Nagel A. Sodium channel blockers for the treatment of epilepsy in CDKL5 deficiency disorder: Findings from a multicenter cohort. Epilepsy Behav. 2021 05; 118:107946. PMID: 33848848.
      View in: PubMed
    49. Saby JN, Benke TA, Peters SU, Standridge SM, Matsuzaki J, Cutri-French C, Swanson LC, Lieberman DN, Key AP, Percy AK, Neul JL, Nelson CA, Roberts TPL, Marsh ED. Multisite Study of Evoked Potentials in Rett Syndrome. Ann Neurol. 2021 04; 89(4):790-802. PMID: 33480039.
      View in: PubMed
    50. Peters SU, Fu C, Marsh ED, Benke TA, Suter B, Skinner SA, Lieberman DN, Standridge S, Jones M, Beisang A, Feyma T, Heydeman P, Ryther R, Glaze DG, Percy AK, Neul JL. Phenotypic features in MECP2 duplication syndrome: Effects of age. Am J Med Genet A. 2021 02; 185(2):362-369. PMID: 33170557.
      View in: PubMed
    51. Raspa M, Bann CM, Gwaltney A, Benke TA, Fu C, Glaze DG, Haas R, Heydemann P, Jones M, Kaufmann WE, Lieberman D, Marsh E, Peters S, Ryther R, Standridge S, Skinner SA, Percy AK, Neul JL. A Psychometric Evaluation of the Motor-Behavioral Assessment Scale for Use as an Outcome Measure in Rett Syndrome Clinical Trials. Am J Intellect Dev Disabil. 2020 11 01; 125(6):493-509. PMID: 33211820.
      View in: PubMed
    52. MacKay CI, Wong K, Demarest ST, Benke TA, Downs J, Leonard H. Exploring genotype-phenotype relationships in the CDKL5 deficiency disorder using an international dataset. Clin Genet. 2021 01; 99(1):157-165. PMID: 33047306.
      View in: PubMed
    53. Fu C, Armstrong D, Marsh E, Lieberman D, Motil K, Witt R, Standridge S, Lane J, Dinkel T, Jones M, Hale K, Suter B, Glaze D, Neul J, Percy A, Benke T. Multisystem comorbidities in classic Rett syndrome: a scoping review. BMJ Paediatr Open. 2020; 4(1):e000731. PMID: 33024833.
      View in: PubMed
    54. Fu C, Armstrong D, Marsh E, Lieberman D, Motil K, Witt R, Standridge S, Nues P, Lane J, Dinkel T, Coenraads M, von Hehn J, Jones M, Hale K, Suter B, Glaze D, Neul J, Percy A, Benke T. Consensus guidelines on managing Rett syndrome across the lifespan. BMJ Paediatr Open. 2020; 4(1):e000717. PMID: 32984552.
      View in: PubMed
    55. Hallgr?msson B, Aponte JD, Katz DC, Bannister JJ, Riccardi SL, Mahasuwan N, McInnes BL, Ferrara TM, Lipman DM, Neves AB, Spitzmacher JAJ, Larson JR, Bellus GA, Pham AM, Aboujaoude E, Benke TA, Chatfield KC, Davis SM, Elias ER, Enzenauer RW, French BM, Pickler LL, Shieh JTC, Slavotinek A, Harrop AR, Innes AM, McCandless SE, McCourt EA, Meeks NJL, Tartaglia NR, Tsai AC, Wyse JPH, Bernstein JA, Sanchez-Lara PA, Forkert ND, Bernier FP, Spritz RA, Klein OD. Automated syndrome diagnosis by three-dimensional facial imaging. Genet Med. 2020 10; 22(10):1682-1693. PMID: 32475986.
      View in: PubMed
    56. Benke TA, Kind PC. Proof-of-concept for a gene replacement approach to CDKL5 deficiency disorder. Brain. 2020 03 01; 143(3):716-718. PMID: 32203572.
      View in: PubMed
    57. O'Leary H, Vanderlinden L, Southard L, Castano A, Saba LM, Benke TA. Transcriptome analysis of rat dorsal hippocampal CA1 after an early life seizure induced by kainic acid. Epilepsy Res. 2020 03; 161:106283. PMID: 32062370.
      View in: PubMed
    58. Demarest ST, Olson HE, Moss A, Pestana-Knight E, Zhang X, Parikh S, Swanson LC, Riley KD, Bazin GA, Angione K, Niestroj LM, Lal D, Juarez-Colunga E, Benke TA. CDKL5 deficiency disorder: Relationship between genotype, epilepsy, cortical visual impairment, and development. Epilepsia. 2019 08; 60(8):1733-1742. PMID: 31313283.
      View in: PubMed
    59. Demarest S, Pestana-Knight EM, Olson HE, Downs J, Marsh ED, Kaufmann WE, Partridge CA, Leonard H, Gwadry-Sridhar F, Frame KE, Cross JH, Chin RFM, Parikh S, Panzer A, Weisenberg J, Utley K, Jaksha A, Amin S, Khwaja O, Devinsky O, Neul JL, Percy AK, Benke TA. Severity Assessment in CDKL5 Deficiency Disorder. Pediatr Neurol. 2019 08; 97:38-42. PMID: 31147226.
      View in: PubMed
    60. Peters SU, Fu C, Suter B, Marsh E, Benke TA, Skinner SA, Lieberman DN, Standridge S, Jones M, Beisang A, Feyma T, Heydeman P, Ryther R, Kaufmann WE, Glaze DG, Neul JL, Percy AK. Characterizing the phenotypic effect of Xq28 duplication size in MECP2 duplication syndrome. Clin Genet. 2019 05; 95(5):575-581. PMID: 30788845.
      View in: PubMed
    61. Olson HE, Demarest ST, Pestana-Knight EM, Swanson LC, Iqbal S, Lal D, Leonard H, Cross JH, Devinsky O, Benke TA. Cyclin-Dependent Kinase-Like 5 Deficiency Disorder: Clinical Review. Pediatr Neurol. 2019 08; 97:18-25. PMID: 30928302.
      View in: PubMed
    62. Daniels D, Knupp K, Benke T, Wolter-Warmerdam K, Moran M, Hickey F. Infantile Spasms in Children With Down Syndrome: Identification and Treatment Response. Glob Pediatr Health. 2019; 6:2333794X18821939. PMID: 30671494.
      View in: PubMed
    63. Neul JL, Benke TA, Marsh ED, Skinner SA, Merritt J, Lieberman DN, Standridge S, Feyma T, Heydemann P, Peters S, Ryther R, Jones M, Suter B, Kaufmann WE, Glaze DG, Percy AK. The array of clinical phenotypes of males with mutations in Methyl-CpG binding protein 2. Am J Med Genet B Neuropsychiatr Genet. 2019 01; 180(1):55-67. PMID: 30536762.
      View in: PubMed
    64. Zamarian L, Delazer M, Ehling R, Pertl MT, Bsteh G, Wenter J, Glatzl S, Brenneis C, Benke T, Berger T. Improvement of medical judgments by numerical training in patients with multiple sclerosis. Eur J Neurol. 2019 01; 26(1):106-112. PMID: 30117230.
      View in: PubMed
    65. Benke T, Traynelis SF. AMPA-Type Glutamate Receptor Conductance Changes and Plasticity: Still a Lot of Noise. Neurochem Res. 2019 Mar; 44(3):539-548. PMID: 29476449.
      View in: PubMed
    66. Hector RD, Kalscheuer VM, Hennig F, Leonard H, Downs J, Clarke A, Benke TA, Armstrong J, Pineda M, Bailey MES, Cobb SR. CDKL5 variants: Improving our understanding of a rare neurologic disorder. Neurol Genet. 2017 Dec; 3(6):e200. PMID: 29264392.
      View in: PubMed
    67. Kirk ME, Meredith FL, Benke TA, Rennie KJ. AMPA receptor-mediated rapid EPSCs in vestibular calyx afferents. J Neurophysiol. 2017 06 01; 117(6):2312-2323. PMID: 28298303.
      View in: PubMed
    68. Larson A, Weisfeld-Adams JD, Benke TA, Bonnen PE. Cerebrotendinous Xanthomatosis Presenting with Infantile Spasms and Intellectual Disability. JIMD Rep. 2017; 35:1-5. PMID: 27858369.
      View in: PubMed
    69. Goodell DJ, Benke TA, Bayer KU. Developmental restoration of LTP deficits in heterozygous CaMKIIa KO mice. J Neurophysiol. 2016 11 01; 116(5):2140-2151. PMID: 27535377.
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    70. Barcomb K, Hell JW, Benke TA, Bayer KU. The CaMKII/GluN2B Protein Interaction Maintains Synaptic Strength. J Biol Chem. 2016 07 29; 291(31):16082-9. PMID: 27246855.
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    71. Goldman AM, LaFrance WC, Benke T, Asato M, Drane D, Pack A, Syed T, Doss R, Lhatoo S, Fureman B, Dingledine R. 2014 Epilepsy Benchmarks Area IV: Limit or Prevent Adverse Consequence of Seizures and Their Treatment Across The Lifespan. Epilepsy Curr. 2016 May-Jun; 16(3):198-205. PMID: 27330453.
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    72. O'Leary H, Bernard PB, Castano AM, Benke TA. Enhanced long term potentiation and decreased AMPA receptor desensitization in the acute period following a single kainate induced early life seizure. Neurobiol Dis. 2016 Mar; 87:134-44. PMID: 26706598.
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    73. Benke TA. GettING in Touch With What Drives Your Inner Funky: Sources of CA1 Gamma Oscillations. Epilepsy Curr. 2015 Sep-Oct; 15(5):271-3. PMID: 26448733.
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    74. Stafstrom CE, Benke TA. Autism and Epilepsy: Exploring the Relationship Using Experimental Models. Epilepsy Curr. 2015 Jul-Aug; 15(4):206-10. PMID: 26316869.
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    75. Bernard PB, Castano AM, Beitzel CS, Carlson VB, Benke TA. Behavioral changes following a single episode of early-life seizures support the latent development of an autistic phenotype. Epilepsy Behav. 2015 Mar; 44:78-85. PMID: 25659043.
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    76. Bernard PB, Benke TA. Early life seizures: evidence for chronic deficits linked to autism and intellectual disability across species and models. Exp Neurol. 2015 Jan; 263:72-8. PMID: 25284323.
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    77. Benke TA. What you seize is what you get: do we yet understand epilepsy in rett syndrome? Epilepsy Curr. 2014 Sep; 14(5):283-5. PMID: 25346641.
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    78. Barcomb K, Buard I, Coultrap SJ, Kulbe JR, O'Leary H, Benke TA, Bayer KU. Autonomous CaMKII requires further stimulation by Ca2+/calmodulin for enhancing synaptic strength. FASEB J. 2014 Aug; 28(8):3810-9. PMID: 24843070.
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    79. Benke T. What the dentate gyrus and the millennial in your basement have in common. Epilepsy Curr. 2014 May; 14(3):152-4. PMID: 24940163.
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    80. Bernard PB, Castano AM, Bayer KU, Benke TA. Necessary, but not sufficient: insights into the mechanisms of mGluR mediated long-term depression from a rat model of early life seizures. Neuropharmacology. 2014 Sep; 84:1-12. PMID: 24780380.
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    81. Benke T. Benchmark IV Progressing Nicely: Rational Pharmacotherapy May Address Cognitive Decline in Epilepsy. Epilepsy Curr. 2014 Mar; 14(2):90-2. PMID: 24872788.
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    82. Bernard PB, Castano AM, O'Leary H, Simpson K, Browning MD, Benke TA. Phosphorylation of FMRP and alterations of FMRP complex underlie enhanced mLTD in adult rats triggered by early life seizures. Neurobiol Dis. 2013 Nov; 59:1-17. PMID: 23831253.
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    83. Thyssen A, Mitchell M, Qvarnstrom Y, Rao S, Benke TA, Glod? MP. Eosinophilic meningitis in a previously healthy 13-year-old child. Pediatr Infect Dis J. 2013 Feb; 32(2):194, 198. PMID: 23328824.
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    84. Benke T. O brother, wherefore are thou? Calcium-permeable AMPA receptors make an appearance in adult status epilepticus. Epilepsy Curr. 2013 Jan; 13(1):32-4. PMID: 23447737.
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    85. Meredith FL, Benke TA, Rennie KJ. Hyperpolarization-activated current (I(h)) in vestibular calyx terminals: characterization and role in shaping postsynaptic events. J Assoc Res Otolaryngol. 2012 Dec; 13(6):745-58. PMID: 22825486.
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    86. Boada R, Hutaff-Lee C, Schrader A, Weitzenkamp D, Benke TA, Goldson EJ, Costa AC. Antagonism of NMDA receptors as a potential treatment for Down syndrome: a pilot randomized controlled trial. Transl Psychiatry. 2012 Jul 17; 2:e141. PMID: 22806212.
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    87. Liu X, Li F, Stubblefield EA, Blanchard B, Richards TL, Larson GA, He Y, Huang Q, Tan AC, Zhang D, Benke TA, Sladek JR, Zahniser NR, Li CY. Direct reprogramming of human fibroblasts into dopaminergic neuron-like cells. Cell Res. 2012 Feb; 22(2):321-32. PMID: 22105488.
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    88. Wray CD, Benke TA. Effect of price increase of adrenocorticotropic hormone on treatment practices of infantile spasms. Pediatr Neurol. 2010 Sep; 43(3):163-6. PMID: 20691936.
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    89. Stubblefield EA, Benke TA. Distinct AMPA-type glutamatergic synapses in developing rat CA1 hippocampus. J Neurophysiol. 2010 Oct; 104(4):1899-912. PMID: 20685930.
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    90. van der Knaap MS, Lai V, K?hler W, Salih MA, Fonseca MJ, Benke TA, Wilson C, Jayakar P, Aine MR, Dom L, Lynch B, K?lm?nchey R, Pietsch P, Errami A, Scheper GC. Megalencephalic leukoencephalopathy with cysts without MLC1 defect. Ann Neurol. 2010 Jun; 67(6):834-7. PMID: 20517947.
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    91. Bernard TJ, Fenton LZ, Apkon SD, Boada R, Wilkening GN, Wilkinson CC, Soep JB, Miyamoto SD, Tripputi M, Armstrong-Wells J, Benke TA, Manco-Johnson MJ, Goldenberg NA. Biomarkers of hypercoagulability and inflammation in childhood-onset arterial ischemic stroke. J Pediatr. 2010 Apr; 156(4):651-6. PMID: 20022340.
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    92. Robertson HR, Gibson ES, Benke TA, Dell'Acqua ML. Regulation of postsynaptic structure and function by an A-kinase anchoring protein-membrane-associated guanylate kinase scaffolding complex. J Neurosci. 2009 Jun 17; 29(24):7929-43. PMID: 19535604.
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    93. Zamarian L, Bodner T, Revkin SK, Benke T, Boesch S, Donnemiller E, Delazer M. Numerical deficits in a single case of basal ganglia dysfunction. Neurocase. 2009 Oct; 15(5):390-404. PMID: 19370479.
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    94. Delazer M, Sinz H, Zamarian L, Stockner H, Seppi K, Wenning GK, Benke T, Poewe W. Decision making under risk and under ambiguity in Parkinson's disease. Neuropsychologia. 2009 Jul; 47(8-9):1901-8. PMID: 19428422.
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    95. Oskarsson B, Zawadzki L, Benke T, Quan D. Neuromuscular hyperexcitability associated with acetylcholine receptor antibodies in a child. J Child Neurol. 2009 Jan; 24(1):90-2. PMID: 18936193.
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    96. Cornejo BJ, Mesches MH, Benke TA. A single early-life seizure impairs short-term memory but does not alter spatial learning, recognition memory, or anxiety. Epilepsy Behav. 2008 Nov; 13(4):585-92. PMID: 18678283.
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    97. Haag A, Knake S, Hamer HM, Boesebeck F, Freitag H, Schulz R, Baum P, Helmstaedter C, Wellmer J, Urbach H, Hopp P, Mayer T, Hufnagel A, Jokeit H, Lerche H, Uttner I, Meencke HJ, Meierkord H, Pauli E, Runge U, Saar J, Trinka E, Benke T, Vulliemoz S, Wiegand G, Stephani U, Wieser HG, Rating D, Werhahn K, Noachtar S, Schulze-Bonhage A, Wagner K, Alpherts WC, Boas Wv, Rosenow F. The Wada test in Austrian, Dutch, German, and Swiss epilepsy centers from 2000 to 2005: a review of 1421 procedures. Epilepsy Behav. 2008 Jul; 13(1):83-9. PMID: 18358786.
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    98. Stafstrom CE, Benke TA. Early-life seizures and cognitive impairment: a spiny problem? Epilepsy Curr. 2008 Jan-Feb; 8(1):27-8. PMID: 18265887.
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    99. Bernard TJ, deVeber GA, Benke TA. Athletic participation after acute ischemic childhood stroke: a survey of pediatric stroke experts. J Child Neurol. 2007 Aug; 22(8):1050-3. PMID: 17761660.
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    100. Cornejo BJ, Mesches MH, Coultrap S, Browning MD, Benke TA. A single episode of neonatal seizures permanently alters glutamatergic synapses. Ann Neurol. 2007 May; 61(5):411-26. PMID: 17323345.
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    101. Hall DA, Parsons J, Benke T. Paroxysmal nonkinesigenic dystonia and celiac disease. Mov Disord. 2007 Apr 15; 22(5):708-10. PMID: 17377923.
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    102. Jones J, Stubblefield EA, Benke TA, Staley KJ. Desynchronization of glutamate release prolongs synchronous CA3 network activity. J Neurophysiol. 2007 May; 97(5):3812-8. PMID: 17344368.
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    103. Delazer M, Sinz H, Zamarian L, Benke T. Decision-making with explicit and stable rules in mild Alzheimer's disease. Neuropsychologia. 2007 Apr 09; 45(8):1632-41. PMID: 17328931.
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    104. Peralta C, Stampfer-Kountchev M, Karner E, K?llensperger M, Geser F, Wolf E, Seppi K, Benke T, Poewe W, Wenning GK. Orthostatic hypotension and attention in Parkinson's disease with and without dementia. J Neural Transm (Vienna). 2007; 114(5):585-8. PMID: 17195917.
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    105. Visani P, Schmutzhard E, Trinka E, Pfausler B, Benke T. Subcortical deficit pattern after brain abscess: a neuropsychological study. Eur J Neurol. 2006 Jun; 13(6):599-603. PMID: 16796583.
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    106. Karner E, Jenner C, Donnemiller E, Delazer M, Benke T. [The clinical syndrome of posterior cortical atrophy]. Nervenarzt. 2006 Feb; 77(2):208-14. PMID: 16205876.
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    107. Dzhala VI, Talos DM, Sdrulla DA, Brumback AC, Mathews GC, Benke TA, Delpire E, Jensen FE, Staley KJ. NKCC1 transporter facilitates seizures in the developing brain. Nat Med. 2005 Nov; 11(11):1205-13. PMID: 16227993.
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    108. Benke T, K?yl? B, Delazer M, Trinka E, Kemmler G. Cholinergic treatment of amnesia following basal forebrain lesion due to aneurysm rupture--an open-label pilot study. Eur J Neurol. 2005 Oct; 12(10):791-6. PMID: 16190917.
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    109. Schnee ME, Lawton DM, Furness DN, Benke TA, Ricci AJ. Auditory hair cell-afferent fiber synapses are specialized to operate at their best frequencies. Neuron. 2005 Jul 21; 47(2):243-54. PMID: 16039566.
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    110. Dobesberger J, Walser G, Embacher N, Unterberger I, Luef G, Bauer G, Benke T, Bartha L, Ortler M, Trinka E. Gyratory seizures revisited: a video-EEG study. Neurology. 2005 Jun 14; 64(11):1884-7. PMID: 15955938.
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    111. Bartha L, Benke T, Bauer G, Trinka E. Interictal language functions in temporal lobe epilepsy. J Neurol Neurosurg Psychiatry. 2005 Jun; 76(6):808-14. PMID: 15897504.
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    112. Bannister NJ, Benke TA, Mellor J, Scott H, G?rdal E, Crabtree JW, Isaac JT. Developmental changes in AMPA and kainate receptor-mediated quantal transmission at thalamocortical synapses in the barrel cortex. J Neurosci. 2005 May 25; 25(21):5259-71. PMID: 15917466.
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    113. Benke T, Chemelli A, Lottersberger C, Waldenberger P, Karner E, Trinka E. Transient global amnesia triggered by the intracarotid amobarbital procedure. Epilepsy Behav. 2005 Mar; 6(2):274-8. PMID: 15710318.
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    114. Rennie KJ, Streeter MA, Benke TA, Moritz AT. Modeling channel properties in vestibular calyx terminals. Biomed Sci Instrum. 2005; 41:358-63. PMID: 15850132.
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    115. L?thi A, Wikstr?m MA, Palmer MJ, Matthews P, Benke TA, Isaac JT, Collingridge GL. Bi-directional modulation of AMPA receptor unitary conductance by synaptic activity. BMC Neurosci. 2004 Nov 11; 5:44. PMID: 15538948.
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    116. Lyle P, Benke T, Forno E, Branum J, Parker S. Evaluation of encephalitis in the toddler: what part of negative don't you understand? Curr Opin Pediatr. 2004 Oct; 16(5):567-70. PMID: 15367852.
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    117. Benke T, Karner E, Seppi K, Delazer M, Marksteiner J, Donnemiller E. Subacute dementia and imaging correlates in a case of Fahr's disease. J Neurol Neurosurg Psychiatry. 2004 Aug; 75(8):1163-5. PMID: 15258221.
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    118. Benke TA, Swann J. The tetanus toxin model of chronic epilepsy. Adv Exp Med Biol. 2004; 548:226-38. PMID: 15250597.
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    119. Trinka E, Walser G, Unterberger I, Luef G, Benke T, Bartha L, Ortler M, Bauer G. Peri-ictal water drinking lateralizes seizure onset to the nondominant temporal lobe. Neurology. 2003 Mar 11; 60(5):873-6. PMID: 12629252.
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    120. Burtscher J, Bartha L, Twerdy K, Eisner W, Benke T. Effect of endoscopic third ventriculostomy on neuropsychological outcome in late onset idiopathic aqueduct stenosis: a prospective study. J Neurol Neurosurg Psychiatry. 2003 Feb; 74(2):222-5. PMID: 12531955.
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    121. Benke TA, L?thi A, Palmer MJ, Wikstr?m MA, Anderson WW, Isaac JT, Collingridge GL. Mathematical modelling of non-stationary fluctuation analysis for studying channel properties of synaptic AMPA receptors. J Physiol. 2001 Dec 01; 537(Pt 2):407-20. PMID: 11731574.
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    122. Trinka E, Unterberger I, Luef G, Benke T, Berger T, Bauer G. Acute aphasia in multiple sclerosis. Arch Neurol. 2001 Jan; 58(1):133-4. PMID: 11176950.
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    123. L?thi A, Chittajallu R, Duprat F, Palmer MJ, Benke TA, Kidd FL, Henley JM, Isaac JT, Collingridge GL. Hippocampal LTD expression involves a pool of AMPARs regulated by the NSF-GluR2 interaction. Neuron. 1999 Oct; 24(2):389-99. PMID: 10571232.
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    124. Voller B, Benke T, Benedetto K, Schnider P, Auff E, Aichner F. Neuropsychological, MRI and EEG findings after very mild traumatic brain injury. Brain Inj. 1999 Oct; 13(10):821-7. PMID: 10576466.
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    125. Isaac JT, L?thi A, Palmer MJ, Anderson WW, Benke TA, Collingridge GL. An investigation of the expression mechanism of LTP of AMPA receptor-mediated synaptic transmission at hippocampal CA1 synapses using failures analysis and dendritic recordings. Neuropharmacology. 1998 Oct-Nov; 37(10-11):1399-410. PMID: 9849675.
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    126. Benke TA, L?thi A, Isaac JT, Collingridge GL. Modulation of AMPA receptor unitary conductance by synaptic activity. Nature. 1998 Jun 25; 393(6687):793-7. PMID: 9655394.
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    127. Benke T, Hochleitner M, Bauer G. Aura phenomena during syncope. Eur Neurol. 1997; 37(1):28-32. PMID: 9018029.
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    128. Bresink I, Benke TA, Collett VJ, Seal AJ, Parsons CG, Henley JM, Collingridge GL. Effects of memantine on recombinant rat NMDA receptors expressed in HEK 293 cells. Br J Pharmacol. 1996 Sep; 119(2):195-204. PMID: 8886398.
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    129. Wilkemeyer MF, Smith KL, Zarei MM, Benke TA, Swann JW, Angelides KJ, Eisensmith RC. Adenovirus-mediated gene transfer into dissociated and explant cultures of rat hippocampal neurons. J Neurosci Res. 1996 Jan 15; 43(2):161-74. PMID: 8820964.
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    130. Benke T, Gasse T, Hittmair-Delazer M, Schmutzhard E. Lyme encephalopathy: long-term neuropsychological deficits years after acute neuroborreliosis. Acta Neurol Scand. 1995 May; 91(5):353-7. PMID: 7639064.
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    131. Berek K, Wagner M, Chemelli AP, Aichner F, Benke T. Hemispheric disconnection in Marchiafava-Bignami disease: clinical, neuropsychological and MRI findings. J Neurol Sci. 1994 May; 123(1-2):2-5. PMID: 8064315.
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    132. Benke TA, Jones OT, Collingridge GL, Angelides KJ. N-Methyl-D-aspartate receptors are clustered and immobilized on dendrites of living cortical neurons. Proc Natl Acad Sci U S A. 1993 Aug 15; 90(16):7819-23. PMID: 7689230.
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    133. Poewe W, Berger W, Benke T, Schelosky L. High-speed memory scanning in Parkinson's disease: adverse effects of levodopa. Ann Neurol. 1991 Jun; 29(6):670-3. PMID: 1892368.
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    134. Schelosky L, Benke T, Poewe WH. Effects of treatment with trihexyphenidyl on cognitive function in early Parkinson's disease. J Neural Transm Suppl. 1991; 33:125-32. PMID: 1753241.
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    135. Kertesz A, Benke T. Sex equality in intrahemispheric language organization. Brain Lang. 1989 Oct; 37(3):401-8. PMID: 2478252.
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    136. Kertesz A, Black SE, Tokar G, Benke T, Carr T, Nicholson L. Periventricular and subcortical hyperintensities on magnetic resonance imaging. 'Rims, caps, and unidentified bright objects'. Arch Neurol. 1988 Apr; 45(4):404-8. PMID: 3355395.
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