Ataxic Disorders

Sankara H. Subramony, Professor of Neurology, McKnight Institute at the University of Florida, Gainesville, Florida, USA and Alexandra Dürr, INSERM U289 et Département de Génétique Cytogénétique et Embryologie, Hôpital de la Salpêtrière, Paris, France CONTENTS BASIC ASPECTS The cerebellum - structure and connections; Physiology of clinical dysfunction of the cerebellum; Oculomotor aspects of the hereditary cerebellar ataxias; Magnetic resonance and nuclear medicine imaging studies in ataxic disease; Neuropathology of degenerative ataxias; Approach to ataxic diseases ACQUIRED ATAXIAS Acquired ataxias, infectious and para-infectious; Ataxia in patients with brain infarcts and hemorrhages; Ataxia resulting from posterior fossa tumors of childhood and other mass lesions;  Nutritional cerebellar degeneration, with comments on its relationship to Wernicke disease and alcoholism; Immune-mediated acquired ataxias; Toxic agents causing cerebellar ataxias; Paraneoplastic cerebellar degeneration DEGENERATIVE ATAXIAS Epidemiology and population genetics of degenerative ataxias; Sporadic adult onset ataxia of unknown aetiology DEGENERATIVE ATAXIAS - MITOCHONDRIAL, AUTOSOMAL RECESSIVE AND X-LINKED Overview of autosomal recessive ataxias; Friedrich’s ataxia; Ataxia with vitamin E deficiency and abetalipoproteinemia; Ataxia-telangiectasia; Autosomal recessive cerebellar ataxias with oculomotor apraxia; Other autosomal recessive and childhood ataxias; Ataxia in mitochondrial disorders; Fragile X-associated tremor/ataxia syndrome DEGENERATIVE ATAXIAS - AUTOSOMAL DOMINANT Overview of autosomal dominant ataxias; Spinocerebellar ataxia type 1, 2; Machado-Joseph disease/spinocerebellar ataxia type 3; Spinocerebellar ataxia type 5, 6, 7; Clinical and genetic features of spinocerebellar ataxia type 8; Spinocerebellar ataxia type 10, 11, 12, 13, 14, 15, 20, 25, 28; Other spinocerebellar ataxias; Dentatorubral-pallidoluysian atrophy; Episodic ataxias 1 and 2 OTHER ISSUES Ataxias related to sensory neuropathies; Frontal lobe ataxia; Balance and gait problems in the elderly; Treatment and management issues in ataxic disease