Spinocerebellar Degenerations: The Ataxias and Spastic Paraplegias - 1st Edition - ISBN: 9780750675031

Spinocerebellar Degenerations: The Ataxias and Spastic Paraplegias, Volume 31

1st Edition

Blue Books of Neurology Series, Volume 31

Authors: Alexis Brice Stefan-M. Pulst
Hardcover ISBN: 9780750675031
Imprint: Butterworth-Heinemann
Published Date: 23rd May 2007
Page Count: 368

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Table of Contents


1 Cerebellum and Spinal Cord – Principles of Development, Anatomical Organization, and Functional Relevance, Jeremy D. Schmahmann


2 Acquired Cerebellar Ataxias and Differential Diagnosis, Thomas Klockgether

3 Multiple System Atrophy, Christoph Globas, Felix Geser, Gregor Wenning, and Ludger Schöls

Autosomal Dominant

4 Clinical and Genetic Aspects of Spinocerebellar Ataxias with emphasis on Polyglutamine Expansions, Giovanni Stevanin, Alexandra Durr, and Alexis Brice

5 Polyglutamine Ataxias: In Vitro and In Vivo Models, Stefan-M. Pulst and Henry Paulson

6 Mechanisms underlying Non-Coding Repeat Expansions, Katherine A. Dick, John W. Day, and Laura P.W. Ranum

Autosomal Recessive

7 Friedreich Ataxia, Massimo Pandolfo

8 Ataxia-Telangiectasia, Thomas O. Crawford and Yosef Shiloh

9 Hereditary Ataxias and Spastic Parapareses in Northeastern Canada, Nicolas Dupré, Bernard Brais, Guy A. Rouleau, and Jean-Pierre Bouchard

10 Recessive Ataxia plus Oculomotor Apraxia Syndromes, Michel Koenig and Maria-Ceu Moreira

11 Metabolic Causes of Ataxia, Anthony H.V. Schapira

12 Other Autosomal Recessive Ataxias, Giuseppe De Michele and Alessandro Filla
Spastic Paraplegias

13 Differential Diagnosis of Acquired causes of Spastic Paraplegia, Bertrand Fontaine

14 SPG4, the most frequent Hereditary Spastic Paraplegia: Clinical and Genetic Aspects
Christel Depienne, Alexis Brice, and Alexandra Durr

15 Autosomal Dominant Spastic Paraplegia: Loci/Genes other than SPG4 (spastin), Evan Reid

16 Overview on Hereditary Spastic Paraplegias, José Leal Loureiro and Paula Coutinho

17 X-linked Spastic Paraplegia, James Garbern

18 Genetic Testing for Hereditary Ataxia and Hereditary Spastic Paraplegia, Martha A. Nance

19 Recessive Spastic Paraplegias, Paula Coutinho and José Leal Loureiro


Apply the expertise of today's leading authorities — all from the forefront of research and clinical practice! This volume in the Blue Books of Neurology series provides rapid access to essential information on the clinical features, differential diagnosis, genetic testing, and management of a broad group of spinocerebellar degenerative disorders, including ataxias and spastic paraplegias. It delivers the guidance you need to accurately diagnose and manage your patients' conditions using today's most effective methods!

Key Features

  • Presents a current understanding of the normal and pathological functioning of the genes responsible for ataxias and spastic paraplegias.
  • Offers current, incisive guidance on the differential diagnosis of disorders within this group - as well as from other types of neurologic disease.
  • Discusses today's best management approaches for both hereditary and acquired spinocerebellar degenerations.


No. of pages:
© Butterworth-Heinemann 2007
23rd May 2007
Hardcover ISBN:

About the Authors

Alexis Brice

Affiliations and Expertise

Professor of Medical Genetics, University of Paris, Head of the Department of Genetics, Pitie-Salpetriere Hospital, Paris, France

Stefan-M. Pulst

Affiliations and Expertise

Carmen and Louis Warschaw Chair, Division of Neurology; Professor of Medicine and Neurobiology, UCLA School of Medicine, Los Angeles, CA, USA