This book is a review of basic physiology, pathophysiology, clinical symptoms and diagnostic methods with emphasis on electrophysiology in disorders of muscle and neuromuscular junction, including fatigue.
The book is divided in three main parts. The first 7 chapters concern basic physiology, morphology and genetics within the field. The new classification system of muscle disorders is presented. The next 9 chapters cover to-date aspects of electrophysiological methods used for diagnosis and understanding of muscle disorders. This section includes EMG concepts, quantitative EMG methods, tests of neuromuscular transmission (SFEMG and repetitive nerve stimulation), surface EMG, nerve conduction methods, motor unit number estimation (MUNE), fatigue tests, respiratory system electrophysiology and a chapter on pediatric EMG. The last section contains 15 chapters on individual disorders of the muscle and neuromuscular junction. They represent reviews of various conditions within a main area such as limb-girdle myopathies, inflammatory myopathies, dystrophinopathies, distal myopathies, channelopathies, congenital myopathies, myasthenic disorders, muscle cramps, FSH, mitochondrial myopathies, critical illness myopathy and neuropathy, metabolic myopathies and toxic muscle diseases. Each chapter covers clinical aspects, and the specific values for EMG, nerve conduction studies, repetitive stimulation, SFEMG, macro-EMG, MUP quantitation, fatigue testing and MUNE. In general, the readers will find references on the electrodiagnostic findings in each type of condition in the chapter, both current and historical.
Since each chapter should be self-contained as much as possible, some overlap of topics is inevitable.
The strength of the volume lies in its coverage of the field from basic science to clinical aspects. Its main focus is the place of electrophysiological methods. This broad presentation of the various topics is aimed at stimulating the reader to efficiently use electrophysiology in diagnosis and future development in the field.
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Section I. Overview.
2. Skeletal muscle: structure and function (D. Burke, S.C. Gandevia). 3. Structure and function of the neuromuscular junction (J.F. Howard Jr.). 4. Nerve-muscle interactions (T. Lømo). 5. Pathology and pathogenesis of muscle diseases (H. Kalimo). 6. Genetic aspects of diagnosis (N. Dahl). 7. Ultrasound examination of muscle (J.H. Van der Hoeven). 8. Effects of aging on motor unit structure and function (L. Larsson).
Section II. Electrophysiological Methods.
9. Electromyographic methods (E. Stålberg, J. Daube). 10. Single fiber electromyography (J.V. Trontelj). 11. Methods for the quantification of conventional needle EMG (E. Stalberg). 12. Multichannel surface EMG (D.F. Stegeman, C. Houtman et al.). 13. Neurography - motor and sensory nerve conduction studies (B. Falck). 14. Repetitive nerve stimulation (D.B. Sanders). 15. Motor unit number estimation in disorders of muscle and neuromuscular junction (R.K. Olney). 16. Muscle fatigue (K.R. Mills). 17. Clinical neurophysiology of respiration (C.F. Bolton, U. Zifko). 18. Pediatric electromyography and neurography (H. Royden Jones Jr., B.T. Darras, K.E. Eeg-Olofsson).
Section III. Muscle Diseases.
19. Limb girdle muscular dystrophy (P.R.W. Fawcett, P.F. Chinnery). 20. Inflammatory myopathies (H.R. Briemberg, A.A. Amato). 21. Dystrophinopathies (K.E. Eeg-Olofsson). 22. Distal myopathies (L. Edström, T. Ansved). 23. Skeletal muscle channelopathies: myotonias, periodic paralyses and malignant hyperthermia (F. Lehmann-Horn, H. Lerche, K. Jurkat-Rott). 24. Congenital muscular disorders (M. Fardeau, D.I. Rubin). 25. Neuromuscular junction disorders (D.B. Sanders). 26. Involuntary muscle contraction of peripheral origin (T. Torbergsen). 27. Facioscapulohumeral muscular dystrophy (P.R.W. Fawcett, P.F. Chinnery). 28. Mitochondrial diseases (E. Ciafaloni, E. Arnaudo). 29. Critical care myopathies (S.J. Bird). 30. Muscle weakness in critical illness (C.F. Bolton). 31. Metabolic myopathies (C.L. Gooch, S. DiMauro). 32. Toxic myopathies (D.I. Rubin).
Section IV. Future Directions.
33. The future role of clinical neurophysiology in muscle disease (E. Stalberg, J. Daube).