Main description:

The latest research regarding the neurodegenerative conditions known as neuroacanthocytosis will be found in this book. Recent advances have identified the range of mutations in the causative genes. In vitro studies have identified potential protein interactions, and work on erythrocyte membranes suggests mechanisms for the generation of acanthocytes. Animal models are being generated and provide the foundation for possible therapeutic interventions.

Contents:

Forward .- Preface .- Part I: The Neuroacanthocytosis Syndromes.- Neuroacanthocytosis Syndromes - A Current Overview. Differential Diagnosis of Chorea. An Update on Hardie Neuroacanthocytosis Series. Update on McLeod Syndrome. Huntington's Disease-like 2. Neuroacanthocytosis in Japan - Review of The Literature and Cases.- Part II: Basic Research - Proteins and Erythrocytes.- The Function of Chorein. Recent studies of Kell and XK: Expression Profiles of Mouse Kell and XK mRNA. Questions of Cell Shape. Neuroacanthocytosis-related Changes In Erythrocyte Membrane Organization And Function. McLeod syndrome: A perspective from Japanese Blood Centers.- Part III: Animal Models.- A Mouse Model of Chorea-acanthocytosis. Part IV: The Structural Basis of Brain Involvement in Neuroacanthocytosis .- Functional Imaging in Neuroacanthocytosis. Volumetric Neuroimaging in Neuroacanthocytosis. Neuropathology of Chorea-acanthocytosis. The Neuropathology of McLeod Syndrome. Cerebral Involvement in McLeod Syndrome: The First Autopsy Revisited.- Part V: Clinical Aspects and Therapy.- Psychiatric morbidity in NA (title to be updated). Muscular Aspects of Chorea-acanthocytosis. Impaired Autonomic Dysfunction in Neuroacanthocytosis and Causes of Sudden Death: Analysis of a Case of Chorea-acanthocytosis with Dysautonomia. Sleep Disorders in Neuroacanthocytosis. Neurosurgery for Neuroacanthocytosis. Speech/occupational therapy in neuroacanthocytosis.- Part VI: The Way Forward.- Virtual Neuroacanthocytosis Institute - A Look Forward