Description The volume summarizes the most recent knowledge in the field of motor system (upper and lower motor neuron) physiology and pathophysiology
as related to diseases of this system. It covers the various aspects of motor system disorders from the motor cortex to the spinal anterior
horn cell. Emphasis is placed on clinical disorders as they affect the human motor system and includes critical discussion of advances
in techniques for diagnosing and understanding the pathophysiology of disorders affecting the motor system, describing approaches to
characterization of disease type, location, severity and prognosis. There is discussion of the specificity and sensitivity of methodological
techniques used in diagnosis, with comparison among the various methods.
Audience
Clinical neurophysiologists and neurologists
Contents Foreword. List of Contributors. 1. Overview (A. Eisen).
I. Anatomy and Physiology of the Human Motor System.
2. Comparative anatomy of the motor system: differences in the organization of corticospinal control in different species (R. Lemon,
J. Griffiths). 3. Developmental aspects of corticospinal projections (J.A. Eyre). 4. Principles of corticospinal system organization
and function (P.D. Cheney, A. Belhaj-Saïf, M.-H. Boudrias). 5. Molecular mechanisms regulating motor neuron development and degeneration
(T.J. Kilpatrick, N. Tubridy). 6. Physiology of the motor neuron and the motor unit (C.J. Heckman, R.M. Enoka). 7. Cytopathology of the
motor neuron (P.G. Ince, S.B. Wharton). 8. Genetics of amyotrophic lateral sclerosis (M. Dewil et al.). 9. Neuroimaging in amyotrophic
lateral sclerosis (G. Comi, L. Leocani). 10. Animal models of motor neuron death (J.-P. Julien).
II. Methodology and Techniques
Applicable to Motor Neuron Diseases.
11. Needle electromyography (J.R. Daube, E.J. Sorenson). 12. Peripheral nerve conduction
studies and neuromuscular junction testing (J. Kimura). 13. Motor unit number estimates (J.M. Shefner). 14. Magnetic stimulation and
central conduction time (K.R. Mills). 15. Evoked potentials in amyotrophic lateral sclerosis (R. Dengler, K. Krampfl). 16. Triple stimulation
technique (K.M. Rosler, M.R. Registris). 17. Cortical threshold and excitability measurements (U. Ziemann). 18. Spinal and long-latency
reflexes (T.V. Ilic, G. Deuschl). 19. Single unit recordings - peristimulus time histograms (PSTHs) (M. Weber). 20. Threshold electrotonus
and the assessment of nerve excitability in amyotrophic lateral sclerosis (M.C. Kiernan, D. Burke).
III. Disorders Primarily
Affecting the Anterior Horn Cell.
21. Spinal muscular atrophies ( M. De Visser, H. Franssen). 22. Arthrogryposis multiplex
and related congenital disorders (J.G. Hall). 23. Spinobulbar muscular atrophy (Kennedy's disease) (J.-M. Gallo). 24. Late muscular sequelae
after poliomyelitis (E. St?lberg). 25. Other infections of the anterior horn cell (R. Olney). 26. Toxic motor neuronopathies and neuropathies
(S.L. Venance, M.J. Strong). 27. Motor neuron disorders with other diseases and exposures (J.D. Mitchell, A.M. Beaumont).
IV.
Amyotrophic Lateral Sclerosis and Related Conditions.
28. Classic Charcot amyotrophic lateral sclerosis (A. Eisen, C.
Krieger). 29. Electrophysiological evaluation of oropharyngeal dysphagia in ALS (C. Ertekin). 30. Electrodiagnostic assessment of respiratory
dysfunction in motor neuron disease (M. De Carvalho). 31. Assessment of autonomic function in motor neuron diseases (P.L. Oey). 32. Neurophysiology
of hereditary amyotrophic lateral sclerosis (H.G. Stewart, P.M. Andersen). 33. Autosomal recessive juvenile amyotrophic lateral sclerosis
(F. Hentati). 34. Paraneoplastic motor neuron disease (E. Nobile-Orazio, A. Cappellari). 35. Multifocal motor neuropathy (R. Kaji). 36.
Focal amyotrophies of the upper and lower limbs (A. Uncini, A. Di Muzio).
V. Disorders Primarily Affecting the Upper Motor
Neuron.
37. Primary lateral sclerosis (N. Le Forestier, V. Meininger). 38. Hereditary spastic paraplegia (H. El-Shanti).
39. Hereditary ataxias (T. Klockgether, M. Abele). 40. Konzo (K.E. Eeg-Olofsson, D. Tshala-Katumbay).
VI. Research Studies.
41. Application of clinical neurophysiology to clinical trials involving motor neuron diseases (M.B. Bromberg).
VII.
Future Advances and Applications.
42. The role of clinical neurophysiology in motor neuron diseases: where next? (M.
Swash).
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