Movement Disorders

Genetics and Models

Edited by

  • Mark LeDoux, Department of Neurology, University of Tennessee Health Science Center, Memphis, TN, USA; Department of Anatomy & Neurobiology, University of Tennessee Health Science Center, Memphis, TN, USA
  • Mark LeDoux, Department of Neurology, University of Tennessee Health Science Center, Memphis, TN, USA; Department of Anatomy & Neurobiology, University of Tennessee Health Science Center, Memphis, TN, USA

The use of animal models is a key aspect of scientific research in numerous fields of medicine. This book vigorously examines the important contributions and application of animal models to the understanding of human movement disorders and will serve as an essential resource for basic neuroscientists engaged in movement disorders research. Academic clinicians, translational researchers and basic scientists are brought together to connect experimental findings made in different animal models to the clinical features, pathophysiology and treatment of human movement disorders. A vital feature of this book is an accompanying DVD with video clips of human movement disorders and their corresponding animal models. The book is divided into sections on Parkinson disease, Huntington disease, dystonia, tremor, paroxysmal movement disorders, ataxia, myoclonus, restless legs syndrome, drug-induced movement disorders, multiple system atrophy, progressive supranuclear palsy/corticobasal degeneration and spasticity. This book serves as an essential resource for both clinicians interested in the science being generated with animal models and basic scientists studying the pathogenesis of particular movement disorders.
View full description


General neurologists, neurologists with subspecialty interest in movement disorders, neuroscientists, rehabilitation physicians, psychiatrists, and academic veterinarians with an interest in animal models.


Book information

  • Published: December 2004
  • ISBN: 978-0-12-088382-0


"The book is a timely review of animal models of movement disorders...The text is an excellent resource for neurologists and researchers specializing in movement disorders. In addition, this book will undoubtedly be a useful reference source for general neurologists, neurology residents, and basic science fellow and students." - Zbigniew K. Wszolek, Department of Neurology, Mayo Clinic Jacksonville in PARKINSONISM AND RELATED DISORDERS (September 2005) "...this new textbook provides a comprehensive treatment of animal models used to study human movement disorders and should serve the neurologist, researcher, and other interested scientists very well." --Dr. A.S. Fix, Central Product Safety, The Proctor and Gamble Co, Miami Valley Innovation Center, Cincinnati, OH, in VETERINARY PATHOLOGY (43:5) 2006

Table of Contents

Table of Contents


List of Contributors

Section A: Scientific Foundations

A1: Classification and Clinical Features of Movement Disorders

A2: Animal Models and the Science of Movement Disorders

A3: Generation of Transgenic and Gene-Targeted Mouse Models of Movement Disorders

A4: Genetics of Spontaneous Mutations in Mice

A5: Assessment of Movement Disorders in Rodents

A6: Response Dynamics: Measurement of the Force and Rhythm of Motor Responses in Laboratory Animals

A7: Behavior in Drosophila: Analysis and Control

A8: Use of C. elegans to Model Human Movement Disorders

Section B: Parkinson Disease

B1: The Phenotypic Spectrum of Parkinson Disease

B2: MPTP-Induced Nigrostriatal Injury in Nonhuman Primates

B3: From Man to Mouse: The MPTP Model of Parkinson Disease

B4: Rotenone Rat and Other Neurotoxin Models of Parkinson Disease

B5: Drosophila Models of Parkinson Disease

B6: Phenotypical Characterization of Genetic Mouse Models of Parkinson Disease

B7: Utility of 6-Hydroxydopamine Lesioned Rats in the Preclinical Screening of Novel Treatments for Parkinson Disease

B8: Motor Complications in Primate Models of Parkinson Disease

B9: C. elegans Models of Parkinson Disease

Section C: Dystonia

C1: Clinical Features and Classification of the Human Dystonias

C2: The Genetically Dystonic Rat

C3: Animal Models of Benign Essential Blepharospasm and Hemifacial Spasm

C4: Mouse Models of Dystonia

C5: The Owl Monkey Model of Focal Dystonia

C6: DYT1 Transgenic Mouse

C7: The hph-1 Mouse

Section D: Huntington Disease

D1: Clinical and Pathological Characteristics of Huntington Disease

D2: Transgenic Rodent Models of Huntington Disease

D3: Knock-in and Knock-out Models of Huntington Disease

D4: Drosophila Models of Huntington Disease

Section E: Tremor Disorders

E1: Neurophysiologic Characterization of Tremor

E2: Essential Tremor

E3: Harmaline Tremor

E4: GABAA Receptor a1 Subunit Knockout Mice: A Novel Model of Essential Tremor

E5: Production and Physiological Study of Holmes Tremor in Monkeys

E6: The Campus Syndrome in Pietrain Pig

Section F: Myoclonus

F1: Pathophysiology, Neurophysiology, and Pharmacology of Human Myoclonus

F2: Post-Hypoxic Myoclonus in Rodents

F3: Baboon Model of Myoclonus

Section G: Tic Disorders

G1: Tourette Syndrome

G2: Animal Models of Tourette Syndrome

Section H: Paroxysmal Movement Disorders

H1: Paroxysmal Dyskinesias in Humans

H2: The Genetically Dystonic Hamster: An Animal Model of Paroxysmal Dystonia

H3: Mouse Models of Hyperekplexia

H4: Bovine Hyperekplexia

H5: Movement Disorders in Drosophila Mutants of Potassium Channels and Biogenic Amine Pathways

Section I: Progressive Supranuclear Palsy and Corticobasal Ganglionic Degeneration

I1: Progressive Supranuclear Palsy and Corticobasal Degeneration

I2: Genetic Susceptibility and Animal Modeling of PSP

I3: Rodent Models of Tauopathies

Section J: Multiple System Atrophy

J1: Clinical Spectrum and Pathological Features of Multiple System Atrophy

J2: Double-Lesion Animal Models of Multiple System Atrophy

J3: A Mouse Model for Multiple System Atrophy

Section K: Ataxias

K1: Clinical and Pathological Features of Hereditary Ataxias

K2: Acquired Ataxias

K3: Animal Models of Spinocerebellar Ataxia Type 1 (SCA1)

K4: Spinocerebellar Ataxia Type 2 (SCA2)

K5: SCA7 Mouse Models

K6: Animal Models of Friedreich Ataxia

K7: Animal Oculomotor Data Illuminate Cerebellum-Related Eye Movement Disorders

Section L: Spasticity

L1: Spasticity

L2: Hereditary Spastic Paraplegia: Clinical Features and Animal Models

L3: The Spastic Rat with Sacral Spinal Cord Injury

L4: Rat Spinal Cord Contusion Model of Spasticity

Section M: Drug-induced Movement Disorders

M1: Drug-Induced Movement Disorders

M2: Neuroleptic-Induced Acute Dystonia and Tardive Dyskinesia in Primates

M3: Motor Effects of Typical and Atypical Antipsychotic Drugs in Rodents

M4: Animal Models of Drug-Induced Akathisia

Section N: Restless legs Syndrome

N1: Clinical Features and Animal Models of Restless Legs Syndrome and Periodic Limb Movement