Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach

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Written by a panel of world authorities, this comprehensive text is the only book of its kind, covering the full range of neuromuscular diseases seen in children. It explains how childhood neuromuscular diseases differ from those in adult patients, and it provides clinicians with all the knowledge they need to successfully diagnose and treat their pediatric patients.

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Product Details

  • ISBN-13: 9780750671903
  • Publisher: Elsevier Health Sciences
  • Publication date: 10/18/2002
  • Edition description: New Edition
  • Pages: 1344
  • Product dimensions: 8.76 (w) x 11.18 (h) x 2.31 (d)

Meet the Author

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.

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Table of Contents

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

17. Facial Palsies: Congenital and acquired
18. Upper Extremities
19. Lower Extremities

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

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