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Behavioural complaints in participants who underwent predictive testing for Huntington’s disease
  1. M-N W Witjes-Ané1,
  2. A H Zwinderman2,
  3. A Tibben1,3,
  4. G-J B van Ommen4,
  5. R A C Roos1
  1. 1Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands
  2. 2Department of Medical Statistics, Leiden University Medical Centre, Leiden, The Netherlands
  3. 3Departments of Clinical Genetics and Neurology, Leiden University Medical Centre, Leiden, The Netherlands
  4. 4Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands.
  1. Correspondence to:
 Dr M-N W Witjes-Ané, Leiden University Medical Centre, Department of Neurology-Neuropsychology, J3-R, PO Box 9600, 2300 RC Leiden, The Netherlands;

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Changes in mood and behaviour form the most variable symptoms of the clinical characteristics of Huntington’s disease (HD).1 Although the diagnosis is usually based on motor signs, behavioural changes occur as a first manifestation of HD in up to half of the patients.2,3 Irritable behaviour, aggression, and depression are most commonly seen in the first phase of the disease.4 Anxiety, obsessions, and apathy are also extremely common in HD. In certain families, major affective disorders may appear as early as 20 years before the onset of chorea and dementia.5 However, with regard to the manifestation of psychiatric signs, there is a known difficulty in distinguishing between an intrinsic and a reactive pattern. The action of the disease is often intertwined with the reaction to the disease in diagnosed patients but also in “asymptomatic” (that is, absence of motor signs) subjects carrying the expansion of the CAG triplet repeat (henceforth referred to as “carriers” compared to “non-carriers”). As far as we are aware, only two studies have been reported regarding psychiatric symptoms in asymptomatic carriers compared to non-carriers.6,7 A controlled psychiatric study reported by Shiwach and Norbury7 showed that there was no significant increase in affective disorder in the former group. However, the whole predictive tested group showed a higher prevalence of psychiatric episodes than their partners. According to the authors it is, therefore, not plausible that depression is an early sign of HD in asymptomatic carriers. Depression and feelings of helplessness are indeed usually seen as a consequence of stressful events related to HD, like predictive testing in both carriers and non-carriers, even years after the predictive test result. Many studies have been reported on mood changes as a reactive pattern in both carriers and non-carriers,8,9 but behavioural changes as …

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