Basil Darras

Basil Darras, MD

Associate Neurologist-in-chief; Chief-Division of Clinical Neurology, Director-Neuromuscular Center

Professor of Neurology, Harvard Medical School

  • Contact: 617-355-2751

  • Fax: 617-730-0285

"I strive to maintain enthusiasm, optimism, and rigorous science as I design and lead studies to translate the genetic origins of neuromuscular disease into effective treatments for the infants and children in my care."

Medical Services

Specialties

  • Electromyography (EMG)
  • Neuromuscular Disorders
  • Spinal Muscular Atrophy
  • Cerebral Palsy
  • Child Neurology

Departments

  • Neurology

Languages

  • English
  • Greek

Programs

  • Neuromuscular Program
  • Spinal Muscular Atrophy Program
To schedule an appointment: Call 617-355-2751 or Request an Appointment
Basil Darras

During my childhood in Greece, my parents instilled in me intellectual curiosity, the ambition to excel, and personal values focused on service. 

My approach to care reflects my interest in the genetic basis of neurological disease and my desire to practice on the forefront of clinical care and research.

I was born and raised in Greece and was strongly influenced by a number of doctors in my family. One of my uncles would let me watch him treat patients and help him give immunizations in schools.

I earned my country's highest marks on nationwide tests to be admitted to medical school, and graduated from Athens University School of Medicine at the top of my class. But I became frustrated by the system that controlled access to further training in pediatrics (I was required to wait three to four years), so I came to the U.S. for my residency. I had planned to return to Greece, but stayed in the U.S. due to the enormous opportunities here to do research and care for patients at the highest possible level.

I am motivated to find cures for children with neuromuscular disorders. I have trained and published extensively as a basic scientist, but was not fulfilled by researching and diagnosing patients alone without helping to improve their lives and outcomes. As a result, I've become very active in clinical research and am excited to be practicing and researching at a time in the field when powerful new resources are at our disposal.

Experience and Education

Education

Medical Degree

University of Athens Medical School, 1977

Athens, GREECE

Internship

University of Athens Hospital, 1979

Athens, GREECE

Residency

Nassau County Medical Center, Clinical Campus SUNY at Stony Brook, 1982

Stony Brook, NY

Residency

Tufts Medical School - New England Medical Center, 1985

Boston, MA

Fellowship

Yale University School of Medicine, 1988

New Haven, CT

Certifications

  • Pediatrics, Child Neurology, Medical Genetics

  • Neuromuscular Medicine

Research

My research efforts encompass both clinical and basic projects focused on molecular genetics, diagnostics and therapeutics for neuromuscular diseases in children.

My basic science work has led to the publication of several important papers in the investigation of DMD (Duchenne muscular dystrophy). Colleagues and I described for the first time in a 1987 Nature publication the phenomenon of germ-line mosaicism in DMD, in which a female with negative blood-based DNA testing for carrier status may still be a carrier and thus be at risk for transmitting the disease to her children. Identifying this biological phenomenon in DMD has had important implications for the genetic counseling of DMD families. 

I am committed to basic research and collaborate with scientists in the Enders and CLS research laboratories of Boston Children's Hospital as well as other institutions on the description of novel mutations in patients with neuromuscular diseases and the application of new technologies to explore the pathogenesis of congenital myopathies and muscular dystrophies.

My clinical research focuses on DMD and SMA (spinal muscular atrophy). I have led several pivotal clinical trials to test mutation specific treatments for these diseases. A series of clinical trials of an antisense oligonucleotide given via spinal tap for SMA has shown encouraging results. I lead one of a few centers in the United States currently testing this treatment and other treatments for SMA and DMD.

My clinical studies have also investigated arthrogryposis multiplex congenita (AMC), chronic inflammatory demyelinating neuropathy (CIDP), and the development of diagnostic algorithms for evaluating children with metabolic myopathies and other neuromuscular diseases.

Remarkable progress in the field of neuromuscular diseases has introduced new genetic tests and has changed the indications for utilization of certain diagnostic procedures in the evaluation of children with presumed disorders of the motor unit. In articles and chapters published in the last few years, I have attempted to define the indications for the new diagnostic tests and re-delineate the role of traditional procedures such as electromyography and muscle biopsy.

I am proud to have written the only major, comprehensive textbook on pediatric neuromuscular disorders, Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician’s Approach, the most comprehensive text in pediatric neuromuscular diseases ever published, with more than a thousand copies sold world-wide in the first few months after the release of the second edition in 2015. I am the author of over 140 peer-reviewed publications and over 80 book chapters, including:

Kunkel LM, Hejtmancik JF, Caskey CT, Speer A, Monaco AP, Middlesworth W, Colletti CA, Bertelson C, Müller U, Bresnan M, Shapiro F, Tantravahi U, Speer J, Latt SA, Bartlett R, Pericak-Vance MA, Roses AD, Thompson MW, Ray PN, Worton RG, Fischbeck KH, Gallano P, Coulon M, Duros C, Boue J, Junien C, Chelly J, Hamard G, Jeanpierre M, Lambert M, Kaplan JC, Emery A, Dorkins H, McGlade S, Davies KE, Boehm C, Arveiler B, Lemaire C, Morgan GJ, Denton MJ, Amos J, Bobrow M, Benham F, Boswinkel E, Cole C, Dubowitz V, Hart K, Hodgson S, Johnson L, Walker A, Roncuzzi L, Ferlini A, Nobile C, Romeo G, Wilcox DE, Affara NA, Ferguson-Smith MA, Lindolf M, Kaariainen H, de la Chapelle A, Ionasescu V, Searby C, Ionasescu R, Bakker E, van Ommen GJ, Pearson PL, Greenberg CR, Hamerton JL, Wrogemann K, Doherty RA, Polakowska R, Hyser C, Quirk S, Thomas N, Harper JF, Darras BT, Francke U. Analysis of deletions in DNA from patients with Becker and Duchenne muscular dystrophy. Nature 1986;322:73-77.

Darras BT, Harper JF, Francke U. Prenatal diagnosis and detection of carriers in Duchenne's muscular dystrophy. N Engl J Med 1987;316:985-992.

Darras BT, Francke U. A partial deletion of the muscular dystrophy gene transmitted twice by an unaffected male. Nature 1987;329:556-558.

Darras BT, Koenig M, Kunkel LM, Francke U. Direct method for prenatal diagnosis and carrier detection in Duchenne’s/Becker’s muscular dystrophy using the entire Dystrophin cDNA. Am J Med Genet 1988;29:713-726.

Darras BT. Molecular genetics of Duchenne and Becker muscular dystrophy. J Pediatr 1990;117:1-15.

Darras BT, Jones HR. Diagnosis of pediatric neuromuscular disorders in the era of DNA analysis. Pediatr Neurol 2000;23(4):289-300.

Wu JY, Kuban K, Allred EN, Darras BT. Association of Duchenne muscular dystrophy with autism spectrum disorder. J Child Neurol 2005;20:790-795.

Markowitz JA, Singh P, Darras BT. Spinal muscular atrophy: a clinical and research update. Pediatr Neurol 2012;46(1):1-12.

Singh P, Liew WK, Darras BT. Current advances in drug development in spinal muscular atrophy. Curr Opin Pediatr 2013 Dec;25(6):682-8.

Darras BT, Markowitz JA, Monani UR, De Vivo DC. Spinal muscular atrophies. In: Darras BT, Jones HR Jr, Ryan MM, De Vivo DC, editors. Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician’s Approach. Second edition. San Diego: Academic Press, 2015.

Darras BT, Menache-Starobinski CC, Hinton V, Kunkel LM. Dystrophinopathies. In: Darras BT, Jones HR Jr, Ryan MM, De Vivo DC, editors. Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician’s Approach. Second edition. San Diego: Academic Press, 2015.


Darras BT, Jones HR Jr, Ryan MM, De Vivo DC, editors. Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician’s Approach. Second edition. San Diego: Academic Press, 2015.

Professional History

I focus in my practice on treating children with neuromuscular diseases, which present as problems of motor development and function.

I am Associate neurologist-in-chief at Boston Children's Hospital and hold the Joseph J. Volpe Chair in Neurology at Harvard Medical School. I am the Chief of the division of clinical neurology in the Department of Neurology at the hospital. For 11 years, I ran the hospital's neurology residency training program and I was twice voted teacher of the year by neurology residents.
  
My special focus is in the care of children with neuromuscular conditions originating from inherited or acquired conditions of the motor unit. These include patients with complex muscle diseases like muscular dystrophies and congenital or metabolic myopathies, neuromuscular transmission defects, neuropathies, motor neuronopathies, and also inflammatory muscle or nerve conditions. Further, I see patients with general neurological problems such seizures when I attend on the inpatient neurology services.
   
Following medical school in Greece and residencies in pediatrics and child neurology at the State University of New York at Stony Brook and Tufts Medical Center, respectively. I completed a post-doctoral fellowship at Yale in genetics, which gives me a unique lens through which I approach problems of childhood motor development.

When I was training in neurology, I gravitated toward neuromuscular cases. The fact that many have a genetic basis appealed to me. I felt that there was a higher probability of finding a treatment if we could understand the pathogenesis of the diseases.

At Boston Children's Hospital, I am proud to be involved with two innovative multi-disciplinary clinical programs. I direct the Neuromuscular Program, which provides diagnostic evaluation and therapeutic services for children with neuromuscular diseases and is one largest of its kind in the country; and the Spinal Muscular Atrophy (SMA) Clinical Research Program, which seeks to improve medical care of children with SMA and discover new treatments for this devastating motor neuron disease.

Through my clinical and research experience, as well as my hospital leadership roles, I have influenced care in the field of pediatric neuromuscular diseases both nationally and internationally.

To schedule an appointment: Call 617-355-2751 or Request an Appointment

Locations

The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”
- Sandra L. Fenwick, President and CEO

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944

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