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Huntington's disease 

Huntington's disease
Chapter:
Huntington's disease
Author(s):

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

DOI:
10.1093/med/9780192619112.003.0644
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date: 23 September 2018

As it is an inherited disorder Huntington’s disease is theoretically present from conception. It is the first clinical manifestation, however, that betrays the presence of the fatal gene. This may appear at any time between childhood and old age. The most obvious sign of Huntington’s disease once the disorder has become manifest is the choreic movement disturbance. It is thus not surprising that the majority of surveys report that the commonest presenting features are neurological and usually choreic (Hayden 1981). Many clinicians, however, believe that the initial manifestations of the disorder are almost invariably psychiatric. These consist of subtle personality change with behavioural problems, irritability, moodiness, impaired judgement, impulsiveness, distractibility, impersistence at tasks, withdrawal, impoverished relationships with others, and a tendency to concrete ideation. A variety of scoring scales exist to rate the severity of Huntington’s disease. The management of a patient with Huntington’s disease involves more than just treating the patient. It requires extensive and ongoing contact with the affected family. Only limited therapy is available for Huntington’s disease. While it is possible to alleviate some of the symptoms, no drug has yet been developed which can successfully alter the underlying disease process, which continues unabated, resulting in progressive physical and mental deterioration leading to premature death. Overall there are only a limited number of trials and most treatment reports consist of small series or case reports (Bonelli et al. 2004). In most cases, research was carried out with regard to motor impairment, particularly chorea, even though this may be one of the least disabling symptoms. Treatment for depression, psychosis, and dementia remain less well studied (Bonelli et al. 2004).

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