The role of psychiatry in genetic prediction programmes for Huntington's disease

Abstract

Psychiatrists are becoming increasingly involved in the assessment of candidates for genetic testing for Huntington's disease (HD) in which their role ranges from diagnosing conventional psychiatric disorder to identifying ‘new odd behaviours'. They are also expected to predict dysfunctional response to test results (e.g. self-harm). This paper describes the psychiatric component of the Cambridge HD Clinic and its assessment protocols. Practical concepts, useful to the understanding of the clinical problems that may be encountered, are also discussed.

Psychiatric symptoms in neurologically asymptomatic Huntington's disease gene carriers: a comparison with gene negative at risk subjects

“…The fact that our study was controlled and blind allowed qualitatively to compare the psychiatric morbidity shown by both gene positive and gene negative subjects (19). Particularly interesting was the finding that the two groups differed in terms of their factor structure.…”

“…All the neuropsychiatric assessments were carried out on the same day as the predictive test and by trained psychiatrists using a standardized computerized protocol (19) that included:…”

“…Emotional disturbance and change of personality may also precede the onset of neurological symptoms (6, 10, 18) and this can lead to up to 50% of misdiagnosis (5, 6, 18). Being brought up in a family disturbed by the presence of an HD sufferer may give rise to maladaptive responses (19), hence it is to be expected that gene‐negative subjects will show a higher rate of psychiatric complaints than normal controls.…”

“…1. compared psychiatric profiles of two at risk groups (HD gene carriers and non‐carriers) mapped by means of a computerized battery (19). This comparison also controlled for the fact that being brought up in a HD home or the approaching predictive test may by themselves also cause psychological perturbation;…”