Cerebellar Degenerations: Clinical Neurobiology

ISBN-13: 9781461365570 / Angielski / Miękka / 2012 / 507 str.

Andreas Plaitakis

Opis

This book encompasses basic and clinical reports on the cerebellum and its primary atrophic disorders, the cerebellar degenerations. Rapid progress has been made in undestanding the organization and function of the cerebellum at the neuronal, synaptic, and molecular level. Of particular importance has been the identification of the chemical transmitters utilized by the cer- ebellar cellular systems. More than any other brain region, the cerebellum utilizes amino acids as its main excitatory and inhibitory neurotransmitters. Excitatory amino acid transmitters, in addition to serving neuronal com- munication, may also mediate trophic and toxic effects, and as such, they may playa role in neurodegenerative processes. The cerebellar degenerations were among the first human disorders with primary system atrophy to be studied clinically and pathologically. This field of clinical cerebellar sciences, no longer confined to the previously known descriptive level, is now advancing rapidly, propelled by rapid advances in neuroimaging, immunology, and molecular biology. The advent of CT, MRI, and PET has in recent years permitted the study of central nervous system alterations in living patients, thus contributing substantially to the accuracy of the diagnosis and the classification of these disorders. The nosology of cerebellar degenerations, which has been the subject of much debate for over a century, is presently a dynamic field, with new entities being recognized and old "classic ataxias" being redefined in the light of new genetic evidence.

Spis treści

Introduction. 1. The Cerebellum and its Disorders in the Dawn of the Molecular Age; A. Plaitakis. Part I: Basic Neurosciences of the Cerebellum. 2. Anatomy and Neurochemical Anatomy of the Cerebellum; J. Hamori. 3. Physiology of the Cerebellum; M. Ito. 4. Amino Acid Transmitters in the Adult and Developing Cerebellum; R. Balazs. 5. Glutamate Receptors in Mammalian Cerebellum: Alterations in Human Ataxic Disorders and Cerebellar Mutant Mice; E. Kouvelas, A. Mitsakos, F. Angelatou, P. Tsiotos, G. Voukelatou. 6. Regional and Cellular Distribution of Glutamate Dehydrogenase and Pyruvate Dehydrogenase Complex in Brain: Implications for Neurodegenerative Disorders; C. Aoki, T.A. Milner, V.M. Pickel. 7. The Purkinje Cell Degeneration Mutant, A Model to Study the Consequences on Neuronal Degeneration; B. Ghetti, L. Triarchou. Part II: Clinical Neurosciences of the Cerebellum. 8. Classification and Epidemiology of Cerebellar Degenerations; A. Plaitakis. 9. The Cerebellar Cortex and the Dentate Nucleus in Hereditary Ataxias; A.H. Koeppen, D.I. Turok. 10. Clinical Neurophysiology in Olivopontocerebellar Atrophy; S. Chokroverty. 11. Pathophysiology of Ataxia in Humans; H.C. Diener, D. Dichgans. 12. Oculomotor Abnormalities in Cerebellar Degeneration; M. Fetter, J. Dichgans. 13. Clinical and Radiologic Features of Cerebellar Degenerations; A. Plaitakis, S. Katoh, Y.P. Huang. Part III: Ethiopathogenesis of Cerebellar Disorders. 14. Glutamate Dehydrogenase Deficiency in Cerebellar Degenerations; A. Plaitakis,P. Shashidharan. 15. Mitochondrial Abnormalities in Hereditary Ataxias; S. Sorbi, J.P. Blass. 16. Cerebellar Disorders in Hexosaminidase Deficiencies; W.G. Johnson. 17. Dominant Olivopontocerebellar Atrophy Mapping to Human Chromosome 6p; M. Nances, L.J. Schut. 18. Positron Emission Tomography of Cerebellar Degeneration; S. Gilman. 19. Ataxia Telengiectasia. A Human Model of Neuro-Immune Degeneration; M. Fiorelli, M. Carbonari, M. Cherchi, C. Gaetano. 20. Paraneoplastic Cerebellar Degeneration; J.E. Hammack, J.B. Posner. Index.