Chorea: Causes and Management by Federico E. Micheli, Peter A. LeWitt

By Federico E. Micheli, Peter A. LeWitt

Chorea: explanations and Management presents a complete and well timed replace for the big variety of neurological stipulations, either inherited and bought, which lead to this universal hyperkinetic circulation disorder.

This e-book describes intimately the newest scientific and etiological information about chorea. administration suggestions, pathophysiology, and linked clinical and psychiatric difficulties linked to chorea also are addressed. the 19 chapters are contributed through internationally-recognized authors operating on the leading edge of study within the particular issues associated with chorea.

Chorea: motives and Management is geared toward an viewers of neurologists, psychiatrists, neuropsychologists, experts in clinical genetics, medical and easy researchers in neurosciences, and generalist scientific physicians with an curiosity in circulation disorders.

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Cardoso F. Sydenham’s chorea. Handb Clin Neurol. 2011;100:221–9. Suggested Reading Inzelberg R, Weinberger M, Gak E. Benign hereditary chorea: an update. Parkinsonism Relat Disord. 2011;17:301–7. Chapter 3 Chorea-Acanthocytosis Andreas Hermann Abstract Neuroacanthocytosis (NA) syndromes are a group of rare disorders displaying neurodegeneration and misshaped spiky red blood cells (acanthocytes). NA syndromes include chorea-acanthocytosis (ChAc), McLeod syndrome (MLS), Huntington’s disease-like 2, and pantothenate kinase-associated neurodegeneration (PKAN) with ChAc as the prototype of this disease family.

Creatine kinase is elevated in serum, and blood acanthocytosis of 7–50 % is seen in almost all patients. Until now, rather anything is known about the pathophysiology of the disease, and thus the treatment is still mainly symptomatic. Epidemiology There are no real epidemiology data on the prevalence or incidence of ChAc. Approximately 1,000 patients are estimated worldwide. ChAc is not restricted to a specific ethnical background. However, higher prevalence was found in Japan and within the French-Canadian populations most likely due to a founder effect [6].

Recently two Japanese families were described with adult-onset autosomal dominant chorea with caudate atrophy, responsive to haloperidol and with a benign evolution. 3. Besides choreic movements, dysarthria, hypotonia, and mild dementing features in some were found. The mean age of onset was 53 years. The term “benign hereditary chorea type 2” was coined for this entity [53]. Huntington disease is an autosomal dominant disorder featured by chorea, behavioral symptoms, and cognitive decline. Besides this, other movement disorders such as parkinsonism, dystonia, myoclonus, tics, and ataxia can arise.

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