Cerebellar Degenerations: Clinical Neurobiology

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.

Contenu

1. The cerebellum and its disorders in the dawn of the molecular ag.- I. Basic Neurosciences of the Cerebellum.- 2. Anatomy and neurochemical anatomy of the cerebellu.- 3. Physiology of the cerebellu.- 4. Amino acid transmitters in the adult and developing cerebellu.- 5. Glutamate receptors in mammalian cerebellum: Alterations in human ataxic disorders and cerebellar mutant mic.- 6. Regional and cellular distribution of glutamate dehydrogenase and pyruvate dehydrogenase complex in brain: Implications for neurodegenerative disorder.- 7. The Purkinje cell degeneration mutant: a model to study the consequences of neuronal degeneratio.- II. Clinical Neurosciences of the Cerebellum.- 8. Classification and epidemiology of cerebellar degeneration.- 9. The cerebellar cortex and the dentate nucleus in hereditary ataxi.- 10. Clinical neurophysiology in olivopontocerebellar atroph.- 11. Pathophysiology of ataxia in human.- 12. Oculomotor abnormalities in cerebellar degeneratio.- 13. Clinical and radiologic features of cerebellar degeneratio.- III. Etiopathogenesis of Cerebellar Disorders.- 14. Glutamate dehydrogenase deficiency in cerebellar degeneration.- 15. Mitochondrial abnormalities in hereditary ataxia.- 16. Cerebellar disorder in the hexosaminidase deficiencie.- 17. Dominant olivopontocerebellar atrophy mapping to human chromosome 6.- 18. Positron emission tomography studies of cerebellar degeneratio.- 19. Ataxia telengiectasia: A human model of neuroimmune degeneratio.- 20. Paraneoplastic cerebellar degeneratio.