Neuromuscular Disorders of Infancy, Childhood, and Adolescence

Dr. H. Royden Jones was Chair of the Department of Neurology at Lahey Hospital and Medical Center, Burlington, Massachusetts; Director of the Electromyography Laboratory at Boston Children's Hospital; and Clinical Professor of Neurology at Harvard Medical School. Dr. Jones completed residencies in Internal Medicine and Neurology and a fellowship in neurological physiology at the Mayo Clinic. He served over 3 years in the United States Army as Chief of Neurology at 5th General Hospital, Bad Cannstatt, Germany. Dr. Jones was Board certified in neurology, clinical neurophysiology, and neuromuscular medicine. Upon completion of his training he joined the Lahey Clinic in 1972. In 1977 he also joined the neurology department at Boston Children's Hospital, founding the electromyography laboratory in 1979. Pediatric EMG became his major clinical research interest. Dr. Jones was co-editor of three major textbooks on childhood clinical neurophysiology and neuromuscular disorders. He was a co-founder of the biennial International Paediatric EMG Conference based at Great Ormond Street Children's Hospital, London, England. Recognized as one of the top neurologists in the U.S., Dr. Jones was an author and editor of several Netter publications including two editions of Netter's Neurology, The Netter Collection of Medical Illustrations: Nervous System, Volume 7, Part I (Brain) and Part II (Spinal Cord and Peripheral Motor and Sensory Systems), 2nd Editions (volumes in the Netter Green Book Collection). Dr. Jones authored and edited several other Netter publications and contributed over 200 peer-reviewed papers and book chapters. Dr. Jones served 8 years as a director of the American Board of Psychiatry and Neurology, becoming Chair of its Neurology Council in 2004. In 2007 he received the Distinguished Physician Award from the American Association of Neuromuscular and Electrodiagnostic Medicine. Lahey Clinic's Medical Staff Association recognized Dr. Jones in 2010 with its highest honor-the Frank Lahey Award for commitment to the values of Dr. Frank Lahey: respect, teamwork, excellence, commitment to personal best." Dr. Jones was named Outstanding Teacher in Pediatric Neurology 2012 - 2013 by the Department of Neurology, Boston Children's Hospital, Harvard Medical School. He also received an award in recognition of his many years of dedicated teaching at Tufts University School of Medicine. Dr. Basil T. Darras is Associate Neurologist-in-Chief and Chief of the Division of Clinical Neurology at Boston Children's Hospital, and the Joseph J. Volpe Professor of Neurology at Harvard Medical School. Dr. Darras is a pediatric neurologist with advanced training and certification in human genetics and neuromuscular medicine. At Boston Children's he serves as Director of the Neuromuscular Program, which includes one of the oldest and most active muscular dystrophy clinics in the country and where he specializes in the care of children with neuromuscular conditions originating from inherited or acquired conditions of the motor unit. He has published over 140 original reports in peer-reviewed journals and over 70 chapters, reviews and editorials; his major publications and specific research interests have focused on the molecular genetics, diagnostics, and therapeutics of Duchenne and Becker muscular dystrophies and spinal muscular atrophy, and on defining the indications for new diagnostic methodologies in the evaluation of children with pediatric neuromuscular diseases. Basic research, carried out in collaboration with scientists in the research laboratories of Boston Children's and other institutions, focuses on the description of novel mutations in patients with neuromuscular diseases and the application of new technologies to explore the pathogenesis and treatment of congenital myopathies and muscular dystrophies. Dr. Darras is site PI for the consortium conducting Pediatric Neuromuscular Clinical Research in Spinal Muscular Atrophy, and site PI/PD for the NeuroNEXT NINDS Program. He is currently involved in multiple clinical trials of novel genetic interventions for the treatment of spinal muscular atrophy and Duchenne muscular dystrophy.

I. Clinical and Laboratory Approach to the Infant and Child with a Neuromuscular Problem 1.Introduction: Historical Perspectives 2.Genetics 3.EMG 4.Muscle Biopsy 5.Nerve Biopsy II.Infantile Hypotonia and Arthrogryposis 6.Neonatal Hypotonia 7.Arthrogryposis III.Anterior Horn Cell Disorders 8.Spinal Muscular Atrophies 9.Juvenile Muscular Atrophy of Unilateral Upper Extremity (Hirayama Disease) 10. Juvenile ALS vs. Other Motor Neuron Disorders 11.Infantile Poliomyelitis and Other Enteropathic Motor Neuron Diseases IV.Radiculopathies and Plexopathies 12.Radiculopathies 13.Plexopathies Rabies Peripheral Nerve Disorders 14.Vaccine Associated Poliomyelitis 15.Tetanus 16.Rabies Mononeuropathies 17.Facial Palsies: Congenital and acquired 18.Upper Extremities 19.Lower Extremities Polyneuropathies 20.Overview of the Neuropathies 21.Congenital and Early Infantile Neuropathies 22.Hereditary Charcot - Marie - Tooth and Other Genetic Neuropathies 23.Guillain - Barre Syndrome 24.Acute Moter Axonal Neuropathy in Childhood 25.Tick Paralysis 26.Chronic Inflammatory Demyelinating and Recurrent Neuropathies 27.Peripheral Neuropathy in Inherited Metabolic Disease 28.Neuropathies Secondary to Systemic Disorders 29.Childhood Hansen's Disease 30.Toxic Neuropathies 31.Autonomic Neuropathies V.Neuromuscular Junction Disorders 32.Infantile Botulism 33.Congential Miyasthenia Gravis 34.Acquired Miyasthenia Gravis in Childhood VI.Myopathies 35.Clinical Syndromes of Congenital Myopathies 36.Congenital Muscular Dystrophies 37.Dystrophinopathies 38.Facioscapulohumeral Dystrophy, Scapuloperoneal Syndromes and Distal Myopathies 39.The Limb Girdle Muscular Dystrophies 40.Emery - Dreifuss Muscular Dystrophy 41.Myopathies of Systemic Disease 42.Channelopathies Affecting Skeletal Muscle in Childhood 43.Glycogen Storage Diseases of Muscle 44.Lipid Storage Muscular Disorders 45.Myoadenylate Deaminase Deficiency 46.Metabolic : Mitochondrial 47.Juvenile Dermatomyositis and other Inflammatory Myopathies in Children 48.Other Inflammatory Myopathies: Viral Trichinosis, and Pyomyositis 49.Rigid Spine Syndromes VII.Special Clinical Problems 50.Neuromuscular Problems of the Critically III Neonate and Child 51.HyperCKemia and Rhabdomyolysis 52.Malignant Hyperthermia 53.Continuous Muscle Fiber Activity Including the Stiff Person 54.Disorder of the Ocular Motor Cranial Nerves and Extraocular Muscles 55.Neurogenic Dysphagia in Newborns and Infants 56.Friedreich's Ataxis, Vitamin E. Deficiency 57.Dominantly Inherited Spinocerebellar Syndromes 58.Reflex Sympathetic Dystrophy VIII.General Therapeutic Principles 59.Autoimmune Pharmacotherapeutic Intervention in Children 60.Androgen and Corticosteroid Therapy of Muscular Dystrophies: New Therapeutic Modalities 61.The Principles and Practice of Molecular Therapies 62.Intensive Care Management 63.Orthopedic Treatment 64.Rehabilitation of the Pediatric Patient with a Neuromuscular Disease 65.Bioethical Issues