Abstract

Predictive genetic testing for a neurogenetic disorder evokes strong emotions, and may lead to distress. The aim of this study is to investigate whether attachment style and emotion regulation strategies are associated with distress in persons who present for predictive testing for a neurogenetic disorder, and whether these psychological traits predict distress after receiving test results. Self-report scales were used to assess attachment insecurity (anxiety and avoidance) and maladaptive emotion regulation strategies (self-blame, rumination, catastrophizing) in adults at 50 % risk for Huntington’s Disease (HD), Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), and Hereditary Cerebral Hemorrhage With Amyloidosis - Dutch type (HCHWA-D), when they presented for predictive testing. Distress was measured before testing and twice (within 2 months and between 6 and 8 months) after receiving test results. Pearson correlations and linear regression were used to analyze whether attachment style and emotion regulation strategies indicated distress. In 98 persons at risk for HD, CADASIL, or HCHWA-D, attachment anxiety and catastrophizing were associated with distress before predictive testing. Attachment anxiety predicted distress up to 2 months after testing. Clinicians may consider looking for signs of attachment anxiety and catastrophizing in persons who present for predictive testing, to see who may be vulnerable for distress during and after testing.

Highlights

  • Predictive genetic tests are available for a number of adultonset autosomal dominant hereditary disorders, including neurogenetic disorders like Huntington’s Disease (HD), Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), and Hereditary Cerebral Hemorrhage With Amyloidosis Dutch type (HCHWA-D) (See Table 1)

  • Since the introduction of predictive testing programs, clinicians and researchers have been interested in recognizing persons who may be vulnerable for distress during and after predictive testing

  • Adult attachment style and emotion regulation strategies are suggested as psychological traits allowing clinicians to gain more insight in the psychodynamics of persons who may be vulnerable for distress during and after predictive testing

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Summary

Introduction

Predictive genetic tests are available for a number of adultonset autosomal dominant hereditary disorders, including neurogenetic disorders like Huntington’s Disease (HD), Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), and Hereditary Cerebral Hemorrhage With Amyloidosis Dutch type (HCHWA-D) (See Table 1). Asymptomatic individuals with a family history of one of these disorders can opt for predictive testing to find out whether or not they are carriers of the disease causing gene mutation. The impact of receiving a negative or positive test result is difficult to predict, and a case-by-case approach is recommended to meet the specific needs of the test applicant (Tibben 2007). In most persons at risk for HD, CADASIL, or HCHWA-D, undergoing predictive testing evokes strong emotions, as the detection of a pathogenic gene mutation has major consequences for the individual as well as for family members.

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