Hyperkinetic Movement Disorders

Hyperkinetic Movement Disorders

Roger Kurlan, Paul Greene

Language: English

Pages: 209

ISBN: 019992564X

Format: PDF / Kindle (mobi) / ePub


Hyperkinetic Movement Disorders the latest edition to the Contemporary Neurology Series, will cover the signs, the pathophysiology, the genetics (where applicable), and the treatment options of each form of hyperkinetic movement disorder. There is a presentation of clinically-focused information regarding the full spectrum of neurological and psychiatric conditions characterized by involuntary movements.

With Parkinson's disease as the most common hypokinetic movement disorder, the book expands on other hyperkinetic movement disorders where substantial progress has been made in the understanding of the role of the basal ganglia in the pathophysiology of these hyperkinesia disorders and in motor control, muscle tone, posture, and cognitive processes. Although therapies that target pathogenesis are still lacking, effective management of hyperkinetic movement disorders demands that physicians are knowledgeable about current and novel pharmacological and surgical approaches.
Following background information about how to approach hyperkinetic movement disorders and the neural circuitry underlying them, there are individual chapters that cover tremor, dystonia, Huntington's disease (and other choreas, athetosis, ballism), Tourette's syndrome (and other tic disorders), habits, mannerisms, compulsions, stereotypies, myoclonus, drug-induced disorders, Wilson's disease, hyperkinetic movement disorders with a peripheral trigger and those of unclear origin, and psychogenic movement disorders. Chapters include sections on clinical phenomenology, etiology and pathogenesis and therapy. There are also on-line resources for clinicians and patients to refer to as well.

The experienced authors have specifically selected scientific and other published information that best helps clinicians understand, diagnose and optimally treat hyperkinetic movement disorders. The authors' approach is comprehensive yet focused and practical with an emphasis on clinical care.

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Hemifacial spasm is rare in the United States. The age-adjusted incidence was 0.78 per 100,000 per year in Olmsted County, Minnesota, between 1960 and 1984, which was 3% of the incidence of Bell’s palsy (25.2 per 100,000).7 The average prevalence was 7.4 per 100,000 for men and 14.5 per 100,000 for women, but the prevalence in women over the age of 60 was considerably higher (almost 30 per 100,000).7 The only published prevalence outside the United States is from Norway, where the average

www.tsa-usa.org Individuals with Tourette’s syndrome and others interested in learning more about the condition can find educational information on this website. www.wilsonsdisease.org The Wilson’s Disease Association provides educational information about the disorder on its website. Index f denotes figure; t denotes table abetalipoproteinemia (Bassen-Kornzweig disease), 178t, 179, 184 acanthocytosis, 72, 73, 73f, 74, 79 aceruloplasminemia, fatty acid 2-hydroxylase associated neurodegeneration

associated with that gene; (4) identify any other clinical or subclinical features associated with mutations in the gene; and (5) identify genetic and nongenetic factors contributing to penetrance. This process in dystonia is in the earliest stages. I will discuss in some detail the genes for primary dystonia with the best-known clinical characteristics. DYT1 The TOR1A gene associated with DYT1 dystonia makes a protein, torsin A, that is found in CNS neurons and is an ATPase. It is in a family

important feature of HD since George Huntington reported on the “tendency to insanity and suicide.” A wide range of psychiatric and behavioral disturbances are currently recognized.32 Mood disorders, psychosis, anxiety, obsessions, compulsions, aggression, irritability, and apathy may be prominent and disabling features of HD. Depression is the most common behavioral feature of HD, occurring in as many as half of HD patients,32,33 and a suicide rate five times more frequent than in the general

prominent exacerbation of symptoms. Patients with Parkinson’s disease (PD) often report RLS,17,18 another observation compatible with a disturbance of central dopamine neurotransmission. Pallidotomy or pallidal deep brain stimulation has relieved RLS in some patients with PD, pointing to a localization of dysfunction in RLS to the basal ganglia. Perhaps the most common aggravating condition for RLS is lumbar spinal osteoarthritis and stenosis, and these should be considered in patients who

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