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Other idiopathic choreic syndromes 

Other idiopathic choreic syndromes
Chapter:
Other idiopathic choreic syndromes
Author(s):

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

DOI:
10.1093/med/9780192619112.003.0667
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date: 29 May 2020

Chorea is the major neurological feature of several idiopathic disorders which may be inherited. The commonest cause of inherited chorea is Huntington’s disease and this has been dealt with separately in Chapter 20. Other inherited chorea disorders include benign hereditary chorea, paroxysmal kinesiogenic chorea, choreoacanthocytosis, and ataxia-telangiectasia. The latter two conditions are associated with systemic abnormalities which reflect the generalized nature of the inherited defect. Those infrequently producing chorea are only referenced. There are also the so-called Huntington’s disease look like syndromes and these have also been dealt with in Chapter 21. There are, furthermore, a number of other inherited disorders in which there is a recognized associated biochemical abnormality in the blood. In most of these, chorea can occur but dystonia is usually the major movement disorder. These are covered in Chapters 40 and 41 in the section on dystonic syndromes. This distinction is rather artificial and as the underlying causes of the idiopathic disorders are known, some may be more appropriately included in the symptomatic groups. Benign hereditary chorea and paroxysmal kinesiogenic chorea stand apart from the others in that they are not associated with additional major signs and do not show gradual progression with development of widespread ‘degeneration’ of the nervous system but may even improve with age. They are perhaps the closest choreic anaology to the primary or idiopathic dystonias. Paroxysmal kinesiogenic chorea is dealt with under ‘Paroxysmal movement disorders’, Chapter 49. Spontaneous oro-facial chorea, which may be present in the elderly, is an idiopathic disorder. Although it is of uncertain cause and included in Table 21.1, it is unlike the other disorders in being acquired. Because of this and its relation to tardive dyskinesia it is discussed with the latter condition in Chapter 23 under the ‘Symptomatic choreic syndromes’.

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