prof. dr. L.H. van den Berg
prof. dr. L.H. van den Berg
- Neurology & Neurosurgery
Leonard van den Berg did a fellowship at the Neurological Institute at Columbia University in New York and obtained his PhD degree in 1995 in Utrecht. He has been professor of Experimental Neurology since 2005 and leads a research group focused on translational research into ALS and other diseases of motor neurons. His research has been focused on the search for effective treatment for patients with motor neuron diseases and motor neuropathies by delineating the biological and molecular pathways that initiate and/or drive motor neuron degeneration. These efforts are based on clinical, lifestyle, environmental,genetic and imaging data obtained from large population-based case–control studies. Part of the research activity has been focused on characterizing the phenotype, diagnosis, pathology and treatment options for multifocal motor neuropathy, which is an important treatable ALS mimic. He is principal investigator of the largest, prospective population-based case-control study in ALS (PAN) to provide class I level of evidence of both environmental/lifestyle and genetic factors that determine risk and outcome of ALS and related motor neuron disorders. His research group has strong bioinformatic expertise in genome-wide association studies, copy number variation, gene expression pathways and other systems biology approaches. He is founder and director of The Netherlands ALS Center, which aims to improve the diagnosis, treatment/care and scientific research for ALS in The Netherlands. He promotes international collaborations on ALS research as chairman of the European Network to find the Cure for ALS (ENCALS)) and as coordinator of awarded EU grants (FP7 Euro-MOTOR (systems biology), JPND SOPHIA (biomarkers)).He promotes international collaborations on ALS research as chairman of the European Network for the Cure of ALS (ENCALS)), as coordinator of awarded EU grants (FP7 Euro-MOTOR(systems biology), JPND SOPHIA (biomarkers)) and as initiator of the worldwide ALS genetics research Project MinE.
Prof. Leonard van den Berg has regular interaction with pharmaceutical and other industrial partners. He has not received personal fees or other personal benefits.
UMCU has received major funding by Julius Clinical for participating in clinical studies sponsored by Amylyx and Ferrer.
UMCU has received funding by Apellis and Orphazyme for providing outcome measure training during clinical trials.
UMCU received minor funding (€200-10,000 per industrial partner) for consultation and invited lectures by Neuropath, Orphazyme, Avexis, BrainEver, Amylyx, Argenx, Calico Life Sciences, Ferrer, Biogen, QurAlis, Brainstorm Cell Therapeutics, Arrowhead, Egeen, Corcept, Sanofi, Kadimastem, Medscape, Takeda, Ionis, RRD, Uniqure and Novartis.
UMC Utrecht participation in following clinical trials (all payments are received by the UMC Utrecht)
UMC Utrecht executes the investigator initiated study: Safety and Efficacy of Tauroursodeoxycholic (TUDCA) as add-on Treatment in Patients Affected by Amyotrophic Lateral Sclerosis (ALS). Van den Berg is local principal investigator. The UMC Utrecht sponsors this trial (2019-)
UMC Utrecht executes the industry initiated study: An Extension Study to Assess the Long-Term Safety, Tolerability, Pharmacokinetics, and Effect on Disease Progression of BIIB078 Administered to Previously Treated Adults With C9ORF72-Associated Amyotrophic Lateral Sclerosis. Van den Berg is local principal investigator. (2020-)
UMC Utrecht executes the industry initiated study: A Phase 1 Multiple-Ascending-Dose Study to Assess the Safety, Tolerability, and Pharmacokinetics of BIIB105 Administered Intrathecally to Adults With Amyotrophic Lateral Sclerosis With or Without Poly-CAG Expansion in the Ataxin-2 Gene. Van den Berg is local principal investigator. (2020-)
UMC Utrecht executes the industry initiated study: A Multicenter, Randomized, Double-blind, Placebo-controlled, Phase 1b/2a Study of WVE-004 Administered Intrathecally to Patients With C9orf72-associated Amyotrophic Lateral Sclerosis (ALS) or Frontotemporal Dementia (FTD). Van den Berg is local principal investigator. (2021-)
UMC Utrecht executes the industry initiated study: A Randomized, Double-Blind, Placebo-Controlled, Phase 2 Study to Assess Efficacy, Long Term Safety and Tolerability of RT001 in Subjects With Amyotrophic Lateral Sclerosis. Van den Berg is local principal investigator. (2021-)
UMC Utrecht executes the industry initiated study: A Multi-arm, Adaptive, Group-sequential trial NETwork to evaluate lithium efficacy in patients with Amyotrophic Lateral Sclerosis (ALS). This trial is sponsored by the UMC Utrecht. Van den Berg is local principal investigator. (2021-) TRICALS and the UMC Utrecht sponsor this trial.
UMC Utrecht executes the industry initiated study: A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Multicenter Study to Evaluate the Efficacy and Safety of Pegcetacoplan in Subjects With Amyotrophic Lateral Sclerosis (ALS). Van den Berg is local principal investigator. (2021-)
UMC Utrecht executes the industry initiated study: A Phase 1b, Multicenter, Randomized, Placebo-Controlled, Double-Blind Study, Followed by an Open-Label Extension, to Determine the Safety, Pharmacokinetics, and Pharmacodynamics of DNL343 in Participants With Amyotrophic Lateral Sclerosis. Van den Berg is local principal investigator. (2021-)
UMC Utrecht executes the investigator initiated study study: Targeting Metabolic Flexibility in ALS (MetFlex); Safety and Tolerability of Trimetazidine for the Treatment of ALS. Van den Berg is local principal investigator. The UMC Utrecht sponsors this trial (2021-)
Neuromuscular Disease, Motor Neuron Disease
Most recent key publications
1: Walhout R, Westeneng HJ, Verstraete E, Hendrikse J, Veldink JH, van den Heuvel MP*, van den Berg LH*. Cortical thickness in ALS: towards a marker for upper motor neuron involvement. J Neurol Neurosurg Psychiatry 2015;86:288-94.
2: Diekstra FP, Van Deerlin VM, van Swieten JC, Al-Chalabi A, Ludolph AC, Weishaupt JH, Hardiman O, Landers JE, Brown RH Jr, van Es MA, Pasterkamp RJ, Koppers M, Andersen PM, Estrada K, Rivadeneira F, Hofman A, Uitterlinden AG, van Damme P, Melki J, Meininger V, Shatunov A, Shaw CE, Leigh PN, Shaw PJ, Morrison KE, Fogh I, Chiò A, Traynor BJ, Czell D, Weber M, Heutink P, de Bakker PI, Silani V, Robberecht W, Veldink JH*, van den Berg LH*. C9orf72 and UNC13A are shared risk loci for amyotrophic lateral sclerosis and frontotemporal dementia: A genome-wide meta-analysis. Ann Neurol 2014;76:120-33.
3: Van den Berg LH. Therapy of amyotrophic lateral sclerosis remains a challenge. Lancet Neurol 2014;13:1062-3
4: Vlam L, van der Pol WL, Cats EA, Straver DC, Piepers S, Franssen H, van den Berg LH. Multifocal motor neuropathy: diagnosis, pathogenesis and treatment strategies. Nature Rev Neurol 2011;8:48-58.
5: Hardiman O, van den Berg LH, Kiernan MC. Clinical diagnosis and management of ALS. Nat Rev Neurol. 2011 Oct 11;7(11):639-49
Chair section Motor Neuron Diseases, European Reference Network - EURO-NMD
Chair Executive Board, European Network for the Cure of ALS (ENCALS)
Chair Executive Board, Treatment Research Initiative to Cure ALS (TRICALS)
Director, Netherlands Neuromuscular Centre
Fellowship and Awards
2015 'Winkler award' from the Dutch Association for Neurology recognizing the largest scientific contribution to neurological science over the past 5 years.
2013 'Forbes Norris award' for high-impact ALS research results in trials, epidemiology and care combined with a major role in the delivery of evidence-based multidisciplinary ALS care.
2011 ‘Sheila Essey Award’ from the American Academy of Neurology (‘This award recognizes an individual who has made significant research contributions in the search for the cause, prevention of, and cure for amyotrophic lateral sclerosis’)
2011-2016 Personal grant ZonMw (VICI ). ‘Amyotrophic lateral sclerosis: disease or syndrome?’
2008-2013 Prinses Beatrix Fonds Neuromuscular Research Prize. ‘The identification and validation of biomarkers for amyotrophic lateral sclerosis’.
2002-2007 Personal grant ZonMw (VIDI subsidie). ‘Genetic susceptibility factors in patients with amyotrophic lateral sclerosis’.
1997-2002 Personal grant (KNAW-onderzoeker). ‘Elucidation of the molecular basis of the cross-reactive immune response to Campylobacter jejuni and peripheral nerve tissue in patients with the Guillain-Barre syndrome using phage antibody display technology’.
Research Output (639)
Genge Angela, van den Berg Leonard H, Frick Glen, Han Steve, Abikoff Cori, Simmons Adam, Lin Qun, Patra Kaushik, Kupperman Erik, Berry James D 11 Sep 2023, In: JAMA Neurology. 80 , p. 1089-1097 9 p.
van Eenennaam Remko M, Rave Neele, Kruithof Willeke J, Kruitwagen-van Reenen Esther T, van den Berg Leonard H, Visser-Meily Johanna A, Beelen Anita Sep 2023, In: PLoS ONE. 18 , p. 1-22 22 p.
Kruithof Willeke J, Kruitwagen-van Reenen Esther, van Eenennaam Remko M, Ronda Maaike C M, Lamers Marjon J, Visser-Meily Johanna M A, Beelen Anita, van den Berg Leonard H 5 Aug 2023, In: Lancet (London, England). 402 , p. 484 1 p.
van Eijk Ruben P A, van den Berg Leonard H, Roes Kit C B, Tian Lu, Lai Tze L, Nelson Lorene M, Li Chenyu, Scowcroft Anna, Garcia-Segovia Jesus, Lu Ying 8 Jul 2023, In: Clinical Pharmacology and Therapeutics. 114 , p. 883-892 10 p.
van Eenennaam Remko M, Kruithof Willeke, Beelen Anita, Bakker Leonhard A, van Eijk Ruben P A, Maessen Maud, Baardman Joost F, Visser-Meily Johanna M A, Veldink Jan H, van den Berg Leonard H Jul 2023, In: The Lancet. Neurology. 22 , p. 591-601 11 p.
de Jongh Adriaan D, van Eijk Ruben P A, Bakker Leonhard A, Bunte Tommy M, Beelen Anita, van der Meijden Conny, van Es Michael A, Visser-Meily Johanna, Kruitwagen Esther T, Veldink Jan H, van den Berg Leonard H 13 Jun 2023, In: Neurology. 101 , p. E602-E612
Shefner Jeremy M, Al-Chalabi Ammar, Andrews Jinsy A, Chio Adriano, De Carvalho Mamede, Cockroft Bettina M, Corcia Philippe, Couratier Philippe, Cudkowicz Merit E, Genge Angela, Hardiman Orla, Heiman-Patterson Terry, Henderson Robert D, Ingre Caroline, Jackson Carlayne E, Johnston Wendy, Lechtzin Noah, Ludolph Albert, Maragakis Nicholas J, Miller Timothy M, Mora Pardina Jesus S, Petri Susanne, Simmons Zachary, Van Den Berg Leonard H, Zinman Lorne, Kupfer Stuart, Malik Fady I, Meng Lisa, Simkins Tyrell J, Wei Jenny, Wolff Andrew A, Rudnicki Stacy A 30 May 2023, In: Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 24 , p. 523-534 12 p.
McFarlane Robert, Galvin Miriam, Heverin Mark, Mac Domhnaill Éanna, Murray Deirdre, Meldrum Dara, Bede Peter, Bolger Anthony, Hederman Lucy, Impey Sinéad, Stephens Gaye, O'Meara Ciara, Wade Vincent, Al-Chalabi Ammar, Chiò Adriano, Corcia Phillippe, van Damme Philip, Ingre Caroline, McDermott Christopher, Povedanos Monica, van den Berg Leonard, Hardiman Orla 23 May 2023, In: Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 24 , p. 389-393 5 p.
van Unnik Jordi W J, Nikolakopoulos Stavros, Eijkemans Marinus J C, Gonzalez-Bermejo Jésus, Bruneteau Gaelle, Morelot-Panzini Capucine, van den Berg Leonard H, Cudkowicz Merit E, McDermott Christopher J, Similowski Thomas, van Eijk Ruben P A 21 Apr 2023, In: Neurology. 100 , p. e2398-e2408
Van Daele Sien Hilde, Moisse Matthieu, van Vugt Joke J F A, Zwamborn Ramona A J, van der Spek Rick, van Rheenen Wouter, Van Eijk Kristel, Kenna Kevin, Corcia Philippe, Vourc'h Patrick, Couratier Philippe, Hardiman Orla, McLaughin Russell, Gotkine Marc, Drory Vivian, Ticozzi Nicola, Silani Vincenzo, Ratti Antonia, de Carvalho Mamede, Mora Pardina Jesús S, Povedano Monica, Andersen Peter M, Weber Markus, Başak Nazli A, Shaw Chris, Shaw Pamela J, Morrison Karen E, Landers John E, Glass Jonathan D, van Es Michael, van den Berg Leonard H, Al-Chalabi Ammar, Veldink Jan, Van Damme Philip 12 Apr 2023, In: Brain : a journal of neurology. 146 , p. 3760-3769 10 p.