Synopses & Reviews
This book encompasses basic and clinical reports on the cerebellum and its primary atrophic disorders, the cerebellar degenerations. Rapid progress has been made in understanding 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 cerebellar 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 communication, may also mediate trophic and toxic effects and, as such, they may play a role in neurodegenerative processes. This volume will be useful to clinicians who care for patients with cerebellar disorders as well as to the basic and the clinical investigators whose work concerns the cerebellum. Knowledge of advances occurring in basic sciences will help the clinical investigator to develop new approaches and therapeutic strategies for these presently intractable human disorders. Also the basic investigator will benefit from the study of human disorders by identifying new research leads provided by these intractable -- yet fascinating -- afflictions.
Table of Contents
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.