Genetic testing is gaining importance in the diagnosis of a rare genetic CNS diseases. Increasingly, asymptomatic individuals want to know the risk of developing the disease, even if they have no family history of the condition. Further, since no preventive lifestyle changes or medicines exist, such worries cannot be offset by adopting preventive actions. Additionally, risk data is often estimated based on data from families affected by the condition and the risk for individuals without family history is unknown, even if they are carriers of the pathogenic gene variant. Therefore, genetic testing of such individuals is ethically questionable. Further, the information about genetic status may lead to significant worry for the individual and the family about the likely development of the disease in the future and it can also increase the actual disease risk. Patients who experience anxiety may require psychological counseling based on e.g. cognitive-behavioral therapy. Also, clinical geneticists should be able to convey genetic information in a way that minimizes the risk of negative mindset formation as this can increase the actual disease risk. Therefore, the regulatory approval of genetic testing may need to be expanded to ascertain that individuals for whom the risk cannot be estimated are not tested. Further, the industry should be encouraged to develop counseling strategies that can mitigate the negative psychological effects of genetic testing using their technologies.