BasilT.DarrasMD

Child Neurology • Boston, MA

Professor of Neurology, Boston Children's Hospital and Harvard Medical School

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Office
300 Longwood Ave
Fegan 11
Boston, MA 02115
Phone(617) 355-8235
Fax(617) 730-0279
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Education & Training

Yale University School of MedicinePost-Doctoral Fellowship, Human Genetics, 1985 - 1988
Tufts Medical CenterFellowship, Child Neurology, 1982 - 1985
Nassau University Medical CenterResidency, Pediatrics, 1979 - 1982
University of AthensClass of 1977

Certifications & Licensure

MA State Medical License 1982 - 2021
American Board of Medical Genetics and Genomics Clinical Genetics
American Board of Pediatrics Pediatrics
American Board of Psychiatry and Neurology Neurology with Special Qualification in Child Neurology

Awards, Honors, & Recognition

America's Top Doctors Castle Connolly, 2002-2014
Boston Magazine Castle Connolly, 2008-2013
Boston Herald Castle Connolly, 2005
Super Doctor SuperDoctors.com
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Clinical Trials

A Study to Assess the Efficacy and Safety of ISIS-SMN Rx in Infants With Spinal Muscular Atrophy Start of enrollment: 2014 Jul 01Shieh, P.,Day, J.,Parsons, J.,Finkel, R.,Kuntz, N.L.,Darras, B.T.,Connolly, A.,Chiriboga, C.,Pena, L.D.,Finanger, E.,Tennekoon, G.,Iannaccone, S.,Sampaio, H.,Ryan, M.,Selby, K.,Vajsar, J.,Oskoui, M.,Serva...
Prospective, Longitudinal Study of the Natural History and Functional Status of Patients With Myotubular Myopathy (MTM) Start of enrollment: 2014 Feb 01Darras, B.T.
Duchenne Muscular Dystrophy: Double-blind Randomized Trial to Find Optimum Steroid Regimen Start of enrollment: 2013 Jan 01Perry Shieh, MD, PhD, Craig McDonald, MD, Richard Finkel, MD, Nancy Kuntz, MD, Richard Barohn, MD, Kathryn Wagner, MD, PhD, Basil Darras, MD, Peter I Karachunski, MD, Leslie A. Morrison, MD, Emma Ciafalon...

Publications & Presentations

PubMed

Respiratory trajectories in type 2 and non-ambulant 3 Spinal muscular atrophy in the iSMAC cohort study
F. Trucco, Deborah Ridout, Mariacristina Scoto, Giorgia Coratti, Marion Main, Robert Muni Lofra, Anna Mayhew, Jacqueline Montes, Marika Pane, Valeria Sansone, Emilio A...> ;Neurology. 2021 Jan 26
Yeo and Darras: Extraneuronal Phenotypes of Spinal Muscular Atrophy.
Crystal Jing Jing Yeo, Basil T. Darras> ;Annals of Neurology. 2021 Jan 1
Clinical Variability in Spinal Muscular Atrophy Type III.
Giorgia Coratti, Sonia Messina, Simona Lucibello, Maria Carmela Pera, Jacqueline Montes, Amy Pasternak, Francesca Bovis, Jessica Exposito Escudero, Elena S. Mazzone, A...> ;Annals of Neurology. 2020 Dec 1
Medical management of muscle weakness in Duchenne muscular dystrophy.
Sarah R Rivera, Sumit K Jhamb, Hoda Z. Abdel-Hamid, Gyula Acsadi, John Brandsema, Emma Ciafaloni, Basil T. Darras, Susan T. Iannaccone, Chamindra G. Konersman, Nancy L...> ;PLOS ONE. 2020 Oct 19
Defining the clinical, molecular and imaging spectrum of adaptor protein complex 4-associated hereditary spastic paraplegia.
Ebrahimi-Fakhari, D., Teinert, J., Behne, R., Wimmer, M., D'Amore, A., Eberhardt, K., Brechmann, B., Ziegler, M., Jensen, D. M., Nagabhyrava, P., Geisel, G., Carmody, ...> ;Brain : a journal of neurology. 2020 Sep 27
Gain and loss of abilities in type II SMA: A 12-month natural history study.
Coratti, G., Lucibello, S., Pera, M. C., Duong, T., Lofra, R. M., Civitello, M., D'Amico, A., Goemans, N., Darras, B. T., Bruno, C., Sansone, V. A., Day, J., Osorio, A...> ;Neuromuscular disorders : NMD. 2020 Sep 8
Meta-analyses of ataluren randomized controlled trials in nonsense mutation Duchenne muscular dystrophy.
Craig Campbell, Richard J. Barohn, Enrico Bertini, Brigitte Chabrol, Giacomo P. Comi, Basil T. Darras, Richard S. Finkel, Kevin M. Flanigan, Nathalie Goemans, Susan T....> ;Journal of Comparative Effectiveness Research. 2020 Aug 27
Seven-Year Experience From the National Institute of Neurological Disorders and Stroke-Supported Network for Excellence in Neuroscience Clinical Trials.
Merit Cudkowicz, Marianne Chase, Christopher S. Coffey, Dixie Ecklund, Brenda Thornell, Codrin Lungu, Katy Mahoney, Laurie Gutmann, Jeremy M. Shefner, Kevin J. Staley,...> ;JAMA Neurology. 2020 Jun 1
Longitudinal natural history of type I spinal muscular atrophy: a critical review.
Mercuri, E., Lucibello, S., Perulli, M., Coratti, G., de Sanctis, R., Pera, M. C., Pane, M., Montes, J., de Vivo, D. C., Darras, B. T., Kolb, S. J., Finkel, R. S.> ;Orphanet journal of rare diseases. 2020 Apr 7
Scoliosis Surgery Significantly Impacts Motor Abilities in Higher-functioning Individuals with Spinal Muscular Atrophy1.
Young, S. D., Montes, J., Salazar, R., Glanzman, A. M., Pasternak, A., Mirek, E., Martens, W., Finkel, R. S., Darras, B. T., De Vivo, D. C.> ;Journal of neuromuscular diseases. 2020 Feb 23
Revised Recommendations for the Treatment of Infants Diagnosed with Spinal Muscular Atrophy Via Newborn Screening Who Have 4 Copies of SMN2.
Glascock, J., Sampson, J., Connolly, A. M., Darras, B. T., Day, J. W., Finkel, R., Howell, R. R., Klinger, K. W., Kuntz, N., Prior, T., Shieh, P. B., Crawford, T. O., ...> ;Journal of neuromuscular diseases. 2020 Feb 3
Overturning the Paradigm of Spinal Muscular Atrophy as just a Motor Neuron Disease
Crystal Jing Jing Yeo, Basil T. Darras> ;Pediatric Neurology. 2020 Jan 22
Response to "The Spectrum of Neuromuscular Disorders Admitted to a Pediatric Intensive Care Unit Is Broader Than Anticipated".
Harrar, D. B., Darras, B. T., Ghosh, P. S.> ;Journal of child neurology. 2020 Jan 4
Electrical impedance myography for reducing sample size in Duchenne muscular dystrophy trials.
Melanie L. Leitner, Kush Kapur, Basil T. Darras, Michele L. Yang, B. Wong, Laura Dalle Pazze, Julaine Florence, Martin Buck, Laura Freedman, Jose L. Bohorquez, Seward ...> ;Annals of clinical and translational neurology. 2020 Jan 1
The Value of Imaging and Composition-Based Biomarkers in Duchenne Muscular Dystrophy Clinical Trials
Stephen M. Chrzanowski, Basil T. Darras, Seward B. Rutkove> ;Neurotherapeutics. 2020 Jan 1
A novel homozygous splice-site mutation in the SPTBN4 gene causes axonal neuropathy without intellectual disability.
Häusler, M. G., Begemann, M., Lidov, H. G., Kurth, I., Darras, B. T., Elbracht, M.> ;European journal of medical genetics. 2019 Dec 21
Deflazacort vs prednisone treatment for Duchenne muscular dystrophy: A meta-analysis of disease progression rates in recent multicenter clinical trials.
McDonald, C. M., Sajeev, G., Yao, Z., McDonnell, E., Elfring, G., Souza, M., Peltz, S. W., Darras, B. T., Shieh, P. B., Cox, D. A., Landry, J., Signorovitch, J.> ;Muscle & nerve. 2019 Oct 11
Acute Neuromuscular Disorders in the Pediatric Intensive Care Unit.
Harrar, D. B., Darras, B. T., Ghosh, P. S.> ;Journal of child neurology. 2019 Sep 11
An Integrated Safety Analysis of Infants and Children with Symptomatic Spinal Muscular Atrophy (SMA) Treated with Nusinersen in Seven Clinical Trials.
Basil T. Darras, Michelle A. Farrar, Eugenio Mercuri, Richard S. Finkel, R. Foster, Steven G. Hughes, I. Bhan, Wildon Farwell, Sarah Gheuens> ;CNS Drugs. 2019 Sep 1
Nusinersen improves walking distance and reduces fatigue in later-onset spinal muscular atrophy.
Montes, J., Young, S. D., Mazzone, E. S., Pasternak, A., Glanzman, A. M., Finkel, R. S., Darras, B. T., Muntoni, F., Mercuri, E., De Vivo, D. C., Bishop, K. M., Schnei...> ;Muscle & nerve. 2019 Jul 13
Urine mRNA to identify a novel pseudoexon causing dystrophinopathy.
Layal Antoury, Ningyan Hu, Basil T. Darras, Thurman M. Wheeler> ;Annals of clinical and translational neurology. 2019 May 17
Homozygous <i>TRPV4</i> mutation causes congenital distal spinal muscular atrophy and arthrogryposis.
Velilla, J., Marchetti, M. M., Toth-Petroczy, A., Grosgogeat, C., Bennett, A. H., Carmichael, N., Estrella, E., Darras, B. T., Frank, N. Y., Krier, J., Gaudet, R., Gup...> ;Neurology. Genetics. 2019 May 2
Nusinersen in later-onset spinal muscular atrophy: Long-term results from the phase 1/2 studies.
Darras, B. T., Chiriboga, C. A., Iannaccone, S. T., Swoboda, K. J., Montes, J., Mignon, L., Xia, S., Bennett, C. F., Bishop, K. M., Shefner, J. M., Green, A. M., Sun, ...> ;Neurology. 2019 Apr 26
Neurofilament as a potential biomarker for spinal muscular atrophy
Basil T. Darras, Thomas O. Crawford, Richard S. Finkel, Eugenio Mercuri, Darryl C. De Vivo, Maryam Oskoui, Eduardo F. Tizzano, Monique M. Ryan, Francesco Muntoni, Guol...> ;Annals of clinical and translational neurology. 2019 Apr 17
X-linked myotubular myopathy: A prospective international natural history study
M. Annoussamy, C. Lilien, Teresa Gidaro, E. Gargaun, V. Chê, Ulrike Schara, Andrea Gangfuß, Adele D'Amico, James J. Dowling, Basil T. Darras, A. Daron, A. Hernandez, C...> ;Neurology. 2019 Apr 16
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Other

Phosphofructokinase deficiency (glycogen storage disease VII, Tarui disease)
Darras BT, Craigen WJ
http://www.uptodate.com/contents/phosphofructokinase-deficiency-glycogen-storage-disease-vii-tarui-d
UpToDate, Wolters Kluwer Health - 2012-10-01
Phosphoglycerate kinase deficiency and phosphoglycerate mutase deficiency
Darras BT, Craigen WJ
http://www.uptodate.com/contents/phosphoglycerate-kinase-deficiency-and-phosphoglycerate-mutase-defi
UpToDate, Wolters Kluwer Health - 2012-10-23
Phosphorylase b kinase deficiency
Darras BT, Craigen WJ
http://www.uptodate.com/contents/phosphorylase-b-kinase-deficiency
UpToDate, Wolters Kluwer Health - 2013-03-25
Approach to the metabolic myopathies
Darras BT
http://www.uptodate.com/contents/approach-to-the-metabolic-myopathies
UpToDate, Wolters Kluwer Health - 2013-03-05
Causes of metabolic myopathies
Darras BT
http://www.uptodate.com/contents/causes-of-metabolic-myopathies
UpToDate, Wolters Kluwer Health - 2012-06-02
Clinical features and diagnosis of Duchenne and Becker muscular dystrophy
Darras BT
http://www.uptodate.com/contents/clinical-features-and-diagnosis-of-duchenne-and-becker-muscular-dys
UpToDate, Wolters Kluwer Health - 2012-09-30
Emery-Dreifuss muscular dystrophy
Darras BT
http://www.uptodate.com/contents/emery-dreifuss-muscular-dystrophy
UpToDate, Wolters Kluwer Health - 2013-02-28
Energy metabolism in muscle
Darras BT
http://www.uptodate.com/contents/energy-metabolism-in-muscle
UpToDate, Wolters Kluwer Health - 2012-11-01
Facioscapulohumeral muscular dystrophy
Darras BT
http://www.uptodate.com/contents/facioscapulohumeral-muscular-dystrophy
UpToDate, Wolters Kluwer Health - 2012-11-26
Limb-girdle muscular dystrophy
Darras BT
http://www.uptodate.com/contents/limb-girdle-muscular-dystrophy
UpToDate, Wolters Kluwer Health - 2013-01-23
Myotonic dystrophy: Etiology, clinical features, and diagnosis
Darras BT, Chad DA
http://www.uptodate.com/contents/myotonic-dystrophy-etiology-clinical-features-and-diagnosis
UpToDate, Wolters Kluwer Health - 2012-10-25
Myotonic dystrophy: Prognosis and management
Darras BT, Chad DA
http://www.uptodate.com/contents/myotonic-dystrophy-prognosis-and-management
UpToDate, Wolters Kluwer Health - 2012-10-26
Oculopharyngeal, distal, and congenital muscular dystrophies
Darras BT
http://www.uptodate.com/contents/oculopharyngeal-distal-and-congenital-muscular-dystrophies
UpToDate, Wolters Kluwer Health - 2012-10-17
Overview of muscular dystrophies
Darras BT
http://www.uptodate.com/contents/overview-of-muscular-dystrophies-beyond-the-basics
UpToDate, Wolters Kluwer Health - 2012-05-27
Treatment of Duchenne and Becker muscular dystrophy
Darras BT
http://www.uptodate.com/contents/treatment-of-duchenne-and-becker-muscular-dystrophy
UpToDate, Wolters Kluwer Health - 2013-03-20
Glycogen branching enzyme deficiency (glycogen storage disease IV, Andersen disease)
Darras BT, Craigen WJ
http://www.uptodate.com/contents/glycogen-branching-enzyme-deficiency-glycogen-storage-disease-iv-an
UpToDate, Wolters Kluwer Health - 2013-02-13
Glycogen debrancher deficiency (glycogen storage disease III)
Darras BT, Craigen WJ
http://www.uptodate.com/contents/glycogen-debrancher-deficiency-glycogen-storage-disease-iii
UpToDate, Wolters Kluwer Health - 2013-01-18
Lactate dehydrogenase deficiency
Darras BT, Craigen WJ
http://www.uptodate.com/contents/lactate-dehydrogenase-deficiency
UpToDate, Wolters Kluwer Health - 2012-10-23
Lysosomal acid maltase deficiency (glycogen storage disease II, Pompe disease)
Darras BT, Craigen WJ
http://www.uptodate.com/contents/lysosomal-acid-maltase-deficiency-glycogen-storage-disease-ii-pompe
UpToDate, Wolters Kluwer Health - 2012-12-14
Lysosome-associated membrane protein 2 deficiency (glycogen storage disease IIb, Danon disease)
Darras BT, Craigen WJ
http://www.uptodate.com/contents/lysosome-associated-membrane-protein-2-deficiency-glycogen-storage-
UpToDate, Wolters Kluwer Health - 2012-08-15
Muscle phosphorylase deficiency (glycogen storage disease V, McArdle disease)
Darras BT, Craigen WJ
http://www.uptodate.com/contents/muscle-phosphorylase-deficiency-glycogen-storage-disease-v-mcardle-
UpToDate, Wolters Kluwer Health - 2012-10-01
Other disorders of glycogen metabolism: GLUT2 deficiency and aldolase A deficiency
Darras BT, Craigen WJ
http://www.uptodate.com/contents/other-disorders-of-glycogen-metabolism-glut2-deficiency-and-aldolas
UpToDate, Wolters Kluwer Health - 2012-11-13
Overview of inherited disorders of glucose and glycogen metabolism
Darras BT, Craigen WJ
http://www.uptodate.com/contents/overview-of-inherited-disorders-of-glucose-and-glycogen-metabolism
UpToDate, Wolters Kluwer Health - 2012-11-13
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Press Mentions

PTC Therapeutics to Host A Conference Call to Review Part 2 of the SUNFISH Clinical Trial Data at the SMA Europe ConferenceFebruary 03, 2020 22:16
Biogen Advances Spinal Muscular Atrophy (SMA) Clinical Research with New Study Evaluating a Higher Dose of SPINRAZA (Nusinersen) and Additional Data in a Broad Range of PatientsSeptember 18, 2019 23:05

Grant Support

Ninds Next: Children'S Hospital Boston Clinical Research SiteNational Institute Of Neurological Disorders And Stroke2011
Electrical Impedance Myography And Quantitative Ultrasound In Duchenne Muscular DNational Institute Of Arthritis And Musculoskeletal And Skin Diseases2011
Clinical Study Of Spinal Muscular AtrophyNational Center For Research Resources2006–2007
Dichloroacetate In Mitochondrial DiseaseNational Center For Research Resources2004–2006
Molecular Studies Of Sodium Channels In Skeletal MuscleNational Institute Of Neurological Disorders And Stroke1989–1993