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Items: 1 to 50 of 100

1.

Phase 1 Study of Edasalonexent (CAT-1004), an Oral NF-κB Inhibitor, in Pediatric Patients with Duchenne Muscular Dystrophy.

Finanger E, Vandenborne K, Finkel RS, Lee Sweeney H, Tennekoon G, Yum S, Mancini M, Bista P, Nichols A, Liu H, Fretzen A, Donovan JM.

J Neuromuscul Dis. 2019;6(1):43-54. doi: 10.3233/JND-180341.

2.

Ambulatory function in spinal muscular atrophy: Age-related patterns of progression.

Montes J, McDermott MP, Mirek E, Mazzone ES, Main M, Glanzman AM, Duong T, Young SD, Salazar R, Pasternak A, Gee R, De Sanctis R, Coratti G, Forcina N, Fanelli L, Ramsey D, Milev E, Civitello M, Pane M, Pera MC, Scoto M, Day JW, Tennekoon G, Finkel RS, Darras BT, Muntoni F, De Vivo DC, Mercuri E.

PLoS One. 2018 Jun 26;13(6):e0199657. doi: 10.1371/journal.pone.0199657. eCollection 2018.

3.

Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy: Implications for Motor Function.

Salazar R, Montes J, Dunaway Young S, McDermott MP, Martens W, Pasternak A, Quigley J, Mirek E, Glanzman AM, Civitello M, Gee R, Duong T, Mazzone ES, Main M, Mayhew A, Ramsey D, Muni Lofra R, Coratti G, Fanelli L, De Sanctis R, Forcina N, Chiriboga C, Darras BT, Tennekoon GI, Scoto M, Day JW, Finkel R, Muntoni F, Mercuri E, De Vivo DC.

Pediatr Phys Ther. 2018 Jul;30(3):209-215. doi: 10.1097/PEP.0000000000000515.

PMID:
29924070
4.

Evaluator Training and Reliability for SMA Global Nusinersen Trials1.

Glanzman AM, Mazzone ES, Young SD, Gee R, Rose K, Mayhew A, Nelson L, Yun C, Alexander K, Darras BT, Zolkipli-Cunningham Z, Tennekoon G, Day JW, Finkel RS, Mercuri E, De Vivo DC, Baldwin R, Bishop KM, Montes J.

J Neuromuscul Dis. 2018;5(2):159-166. doi: 10.3233/JND-180301.

5.

Longitudinal timed function tests in Duchenne muscular dystrophy: ImagingDMD cohort natural history.

Arora H, Willcocks RJ, Lott DJ, Harrington AT, Senesac CR, Zilke KL, Daniels MJ, Xu D, Tennekoon GI, Finanger EL, Russman BS, Finkel RS, Triplett WT, Byrne BJ, Walter GA, Sweeney HL, Vandenborne K.

Muscle Nerve. 2018 Nov;58(5):631-638. doi: 10.1002/mus.26161. Epub 2018 Jul 24.

PMID:
29742798
6.

Two-Year Longitudinal Changes in Lower Limb Strength and Its Relation to Loss in Function in a Large Cohort of Patients With Duchenne Muscular Dystrophy.

Batra A, Harrington A, Lott DJ, Willcocks R, Senesac CR, McGehee W, Xu D, Mathur S, Daniels MJ, Rooney WD, Forbes SC, Triplett W, Deol JK, Arpan I, Bendixen R, Finkel R, Finanger E, Tennekoon G, Byrne B, Russman B, Sweeney HL, Walter G, Vandenborne K.

Am J Phys Med Rehabil. 2018 Oct;97(10):734-740. doi: 10.1097/PHM.0000000000000957.

PMID:
29734234
7.

Skeletal muscle magnetic resonance biomarkers correlate with function and sentinel events in Duchenne muscular dystrophy.

Barnard AM, Willcocks RJ, Finanger EL, Daniels MJ, Triplett WT, Rooney WD, Lott DJ, Forbes SC, Wang DJ, Senesac CR, Harrington AT, Finkel RS, Russman BS, Byrne BJ, Tennekoon GI, Walter GA, Sweeney HL, Vandenborne K.

PLoS One. 2018 Mar 19;13(3):e0194283. doi: 10.1371/journal.pone.0194283. eCollection 2018.

8.

Use of the Wilmington Robotic Exoskeleton to Improve Upper Extremity Function in Patients With Duchenne Muscular Dystrophy.

Estilow T, Glanzman AM, Powers K, Moll A, Flickinger J, Medne L, Tennekoon G, Yum SW.

Am J Occup Ther. 2018 Mar/Apr;72(2):7202345010p1-7202345010p5. doi: 10.5014/ajot.2018.022939.

PMID:
29426391
9.

Revised Hammersmith Scale for spinal muscular atrophy: A SMA specific clinical outcome assessment tool.

Ramsey D, Scoto M, Mayhew A, Main M, Mazzone ES, Montes J, de Sanctis R, Dunaway Young S, Salazar R, Glanzman AM, Pasternak A, Quigley J, Mirek E, Duong T, Gee R, Civitello M, Tennekoon G, Pane M, Pera MC, Bushby K, Day J, Darras BT, De Vivo D, Finkel R, Mercuri E, Muntoni F.

PLoS One. 2017 Feb 21;12(2):e0172346. doi: 10.1371/journal.pone.0172346. eCollection 2017.

10.

Revised upper limb module for spinal muscular atrophy: Development of a new module.

Mazzone ES, Mayhew A, Montes J, Ramsey D, Fanelli L, Young SD, Salazar R, De Sanctis R, Pasternak A, Glanzman A, Coratti G, Civitello M, Forcina N, Gee R, Duong T, Pane M, Scoto M, Pera MC, Messina S, Tennekoon G, Day JW, Darras BT, De Vivo DC, Finkel R, Muntoni F, Mercuri E.

Muscle Nerve. 2017 Jun;55(6):869-874. doi: 10.1002/mus.25430. Epub 2017 Feb 6.

PMID:
27701745
11.

Rasch analysis of the Pediatric Evaluation of Disability Inventory-computer adaptive test (PEDI-CAT) item bank for children and young adults with spinal muscular atrophy.

Pasternak A, Sideridis G, Fragala-Pinkham M, Glanzman AM, Montes J, Dunaway S, Salazar R, Quigley J, Pandya S, O'Riley S, Greenwood J, Chiriboga C, Finkel R, Tennekoon G, Martens WB, McDermott MP, Fournier HS, Madabusi L, Harrington T, Cruz RE, LaMarca NM, Videon NM, Vivo DC, Darras BT; Muscle Study Group (MSG) and the Pediatric Neuromuscular Clinical Research Network for Spinal Muscular Atrophy (PNCRN).

Muscle Nerve. 2016 Dec;54(6):1097-1107. doi: 10.1002/mus.25164. Epub 2016 Oct 6.

PMID:
27121348
12.

Physical therapy services received by individuals with spinal muscular atrophy (SMA).

Dunaway S, Montes J, McDermott MP, Martens W, Neisen A, Glanzman AM, Pasternak A, Riley S, Sproule D, Chiriboga C, Finkel R, Tennekoon G, Darras B, De Vivo D, Pandya S.

J Pediatr Rehabil Med. 2016;9(1):35-44. doi: 10.3233/PRM-160360.

PMID:
26966799
13.

Multicenter prospective longitudinal study of magnetic resonance biomarkers in a large duchenne muscular dystrophy cohort.

Willcocks RJ, Rooney WD, Triplett WT, Forbes SC, Lott DJ, Senesac CR, Daniels MJ, Wang DJ, Harrington AT, Tennekoon GI, Russman BS, Finanger EL, Byrne BJ, Finkel RS, Walter GA, Sweeney HL, Vandenborne K.

Ann Neurol. 2016 Apr;79(4):535-47. doi: 10.1002/ana.24599. Epub 2016 Feb 19.

14.

Association of a Novel ACTA1 Mutation With a Dominant Progressive Scapuloperoneal Myopathy in an Extended Family.

Zukosky K, Meilleur K, Traynor BJ, Dastgir J, Medne L, Devoto M, Collins J, Rooney J, Zou Y, Yang ML, Gibbs JR, Meier M, Stetefeld J, Finkel RS, Schessl J, Elman L, Felice K, Ferguson TA, Ceyhan-Birsoy O, Beggs AH, Tennekoon G, Johnson JO, Bönnemann CG.

JAMA Neurol. 2015 Jun;72(6):689-98. doi: 10.1001/jamaneurol.2015.37. Erratum in: JAMA Neurol. 2015 Aug;72(8):950.

15.

Spinal muscular atrophy functional composite score: A functional measure in spinal muscular atrophy.

Montes J, Glanzman AM, Mazzone ES, Martens WB, Dunaway S, Pasternak A, Riley SO, Quigley J, Pandya S, De Vivo DC, Kaufmann P, Chiriboga CA, Finkel RS, Tennekoon GI, Darras BT, Pane M, Mercuri E, Mcdermott MP; Pediatric Neuromuscular Clinical Research Network, Muscle Study Group, SMA Europe.

Muscle Nerve. 2015 Dec;52(6):942-7. doi: 10.1002/mus.24670. Epub 2015 Oct 10.

PMID:
25846132
16.

Spectrum of neuropathophysiology in spinal muscular atrophy type I.

Harding BN, Kariya S, Monani UR, Chung WK, Benton M, Yum SW, Tennekoon G, Finkel RS.

J Neuropathol Exp Neurol. 2015 Jan;74(1):15-24. doi: 10.1097/NEN.0000000000000144.

17.

Magnetic resonance imaging and spectroscopy assessment of lower extremity skeletal muscles in boys with Duchenne muscular dystrophy: a multicenter cross sectional study.

Forbes SC, Willcocks RJ, Triplett WT, Rooney WD, Lott DJ, Wang DJ, Pollaro J, Senesac CR, Daniels MJ, Finkel RS, Russman BS, Byrne BJ, Finanger EL, Tennekoon GI, Walter GA, Sweeney HL, Vandenborne K.

PLoS One. 2014 Sep 9;9(9):e106435. doi: 10.1371/journal.pone.0106435. eCollection 2014. Erratum in: PLoS One. 2014;9(10):e111822.

18.

Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS.

Arpan I, Willcocks RJ, Forbes SC, Finkel RS, Lott DJ, Rooney WD, Triplett WT, Senesac CR, Daniels MJ, Byrne BJ, Finanger EL, Russman BS, Wang DJ, Tennekoon GI, Walter GA, Sweeney HL, Vandenborne K.

Neurology. 2014 Sep 9;83(11):974-80. doi: 10.1212/WNL.0000000000000775. Epub 2014 Aug 6.

19.

Sildenafil does not improve cardiomyopathy in Duchenne/Becker muscular dystrophy.

Leung DG, Herzka DA, Thompson WR, He B, Bibat G, Tennekoon G, Russell SD, Schuleri KH, Lardo AC, Kass DA, Thompson RE, Judge DP, Wagner KR.

Ann Neurol. 2014 Oct;76(4):541-9. doi: 10.1002/ana.24214. Epub 2014 Jul 10.

20.

Severe congenital RYR1-associated myopathy: the expanding clinicopathologic and genetic spectrum.

Bharucha-Goebel DX, Santi M, Medne L, Zukosky K, Dastgir J, Shieh PB, Winder T, Tennekoon G, Finkel RS, Dowling JJ, Monnier N, Bönnemann CG.

Neurology. 2013 Apr 23;80(17):1584-9. doi: 10.1212/WNL.0b013e3182900380. Epub 2013 Apr 3. Erratum in: Neurology. 2013 May 28;80(22):2081. Zukosky, Kristin [corrected to Zukosky, Kristen].

21.

Juvenile-onset motor neuron disease caused by novel mutations in β-hexosaminidase.

Pierson TM, Torres PA, Zeng BJ, Glanzman AM, Adams D, Finkel RS, Mahuran DJ, Pastores GM, Tennekoon GI, Kolodny EH.

Mol Genet Metab. 2013 Jan;108(1):65-9. doi: 10.1016/j.ymgme.2012.10.023. Epub 2012 Nov 2.

22.

Unanswered questions in Friedreich ataxia.

Lynch DR, Deutsch EC, Wilson RB, Tennekoon G.

J Child Neurol. 2012 Sep;27(9):1223-9. doi: 10.1177/0883073812453498. Epub 2012 Jul 25. Review.

23.

Pediatric optic neuritis: brain MRI abnormalities and risk of multiple sclerosis.

Bonhomme GR, Waldman AT, Balcer LJ, Daniels AB, Tennekoon GI, Forman S, Galetta SL, Liu GT.

Neurology. 2009 Mar 10;72(10):881-5. doi: 10.1212/01.wnl.0000344163.65326.48.

PMID:
19273821
24.

Umbilical cord blood transplantation for juvenile metachromatic leukodystrophy.

Pierson TM, Bonnemann CG, Finkel RS, Bunin N, Tennekoon GI.

Ann Neurol. 2008 Nov;64(5):583-7. doi: 10.1002/ana.21522.

25.

Mega-corpus callosum, polymicrogyria, and psychomotor retardation: confirmation of a syndromic entity.

Pierson TM, Zimmerman RA, Tennekoon GI, Bönnemann CG.

Neuropediatrics. 2008 Apr;39(2):123-7. doi: 10.1055/s-2008-1081218.

PMID:
18671190
26.

Exon skipping mutations in collagen VI are common and are predictive for severity and inheritance.

Lampe AK, Zou Y, Sudano D, O'Brien KK, Hicks D, Laval SH, Charlton R, Jimenez-Mallebrera C, Zhang RZ, Finkel RS, Tennekoon G, Schreiber G, van der Knaap MS, Marks H, Straub V, Flanigan KM, Chu ML, Muntoni F, Bushby KM, Bönnemann CG.

Hum Mutat. 2008 Jun;29(6):809-22. doi: 10.1002/humu.20704.

PMID:
18366090
27.

Hemosiderin-laden macrophages in the cerebrospinal fluid of a neonate after traumatic lumbar puncture.

Wusthoff CJ, Abend NS, Tennekoon G.

Pediatr Infect Dis J. 2008 Jan;27(1):83-4.

PMID:
18162950
28.

Childhood multiple sclerosis: a review.

Waldman A, O'Connor E, Tennekoon G.

Ment Retard Dev Disabil Res Rev. 2006;12(2):147-56. Review.

PMID:
16807911
29.

Central nervous system hyperexcitability associated with glutamate dehydrogenase gain of function mutations.

Raizen DM, Brooks-Kayal A, Steinkrauss L, Tennekoon GI, Stanley CA, Kelly A.

J Pediatr. 2005 Mar;146(3):388-94.

PMID:
15756227
30.

Notch and Schwann cell transformation.

Li Y, Rao PK, Wen R, Song Y, Muir D, Wallace P, van Horne SJ, Tennekoon GI, Kadesch T.

Oncogene. 2004 Feb 5;23(5):1146-52.

PMID:
14762442
31.

Lysophosphatidic acid promotes survival and differentiation of rat Schwann cells.

Li Y, Gonzalez MI, Meinkoth JL, Field J, Kazanietz MG, Tennekoon GI.

J Biol Chem. 2003 Mar 14;278(11):9585-91. Epub 2003 Jan 10.

32.
33.

Neurofibromatosis type 1: II. Answers from animal models.

Lakkis MM, Tennekoon GI.

J Neurosci Res. 2001 Aug 1;65(3):191-4. Review. No abstract available.

PMID:
11494353
34.

Neuregulin signaling through a PI3K/Akt/Bad pathway in Schwann cell survival.

Li Y, Tennekoon GI, Birnbaum M, Marchionni MA, Rutkowski JL.

Mol Cell Neurosci. 2001 Apr;17(4):761-7.

PMID:
11312610
35.

Neurofibromatosis type 1. I. General overview.

Lakkis MM, Tennekoon GI.

J Neurosci Res. 2000 Dec 15;62(6):755-63. Review. No abstract available.

PMID:
11107159
36.

Regulation of glucose transport in cultured Schwann cells.

Magnani P, Thomas TP, Tennekoon G, DeVries GH, Greene DA, Brosius FC 3rd.

J Peripher Nerv Syst. 1998;3(1):28-36.

PMID:
10959235
37.

Myelin and disorders that affect the formation and maintenance of this sheath.

Porter BE, Tennekoon G.

Ment Retard Dev Disabil Res Rev. 2000;6(1):47-58. Review. No abstract available.

PMID:
10899797
38.

Reciprocal Id expression and myelin gene regulation in Schwann cells.

Thatikunta P, Qin W, Christy BA, Tennekoon GI, Rutkowski JL.

Mol Cell Neurosci. 1999 Dec;14(6):519-28.

PMID:
10656257
39.

Progressive spinal muscular atrophies.

Strober JB, Tennekoon GI.

J Child Neurol. 1999 Nov;14(11):691-5. Review.

PMID:
10593543
40.

Signals for proinflammatory cytokine secretion by human Schwann cells.

Rutkowski JL, Tuite GF, Lincoln PM, Boyer PJ, Tennekoon GI, Kunkel SL.

J Neuroimmunol. 1999 Nov 1;101(1):47-60.

PMID:
10580813
41.

Schwann cell-conditioned medium promotes neuroblastoma survival and differentiation.

Kwiatkowski JL, Rutkowski JL, Yamashiro DJ, Tennekoon GI, Brodeur GM.

Cancer Res. 1998 Oct 15;58(20):4602-6.

42.

A role for Pak protein kinases in Schwann cell transformation.

Tang Y, Marwaha S, Rutkowski JL, Tennekoon GI, Phillips PC, Field J.

Proc Natl Acad Sci U S A. 1998 Apr 28;95(9):5139-44.

43.

Characterization of insulin-like growth factor-I and its receptor and binding proteins in transected nerves and cultured Schwann cells.

Cheng HL, Randolph A, Yee D, Delafontaine P, Tennekoon G, Feldman EL.

J Neurochem. 1996 Feb;66(2):525-36.

44.

The zinc finger transcription factor Zif268/Egr-1 is essential for Schwann cell expression of the p75 NGF receptor.

Nikam SS, Tennekoon GI, Christy BA, Yoshino JE, Rutkowski JL.

Mol Cell Neurosci. 1995 Aug;6(4):337-48.

PMID:
8846003
45.
46.

Purification and expansion of human Schwann cells in vitro.

Rutkowski JL, Kirk CJ, Lerner MA, Tennekoon GI.

Nat Med. 1995 Jan;1(1):80-3.

PMID:
7584959
47.

Partial structure and mapping of the human myelin P2 protein gene.

Narayanan V, Ripepi B, Jabs EW, Hawkins A, Griffin C, Tennekoon G.

J Neurochem. 1994 Dec;63(6):2010-3.

48.

Sources of human Schwann cells and the influence of donor age.

Boyer PJ, Tuite GF, Dauser RC, Muraszko KM, Tennekoon GI, Rutkowski JL.

Exp Neurol. 1994 Nov;130(1):53-5.

49.

Advances in understanding the development of the nervous system.

Singer HS, Chiu AY, Meiri KF, Morell P, Nelson PG, Tennekoon G.

Curr Opin Neurol. 1994 Apr;7(2):153-9. Review.

PMID:
8019661

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