Patients with
prion diseases can live for long periods of time in a state of
akinetic mutism given appropriate management of their symptoms. To study symptom support in these cases, we performed
gastrostomies on 3 patients with V180I genetic
Creutzfeldt-Jakob disease (CJD) who had become akinetic and mute, and compared them to 14 other similar patients being fed by tube. In the 3
gastrostomy cases, there were no direct complications due to the
gastrostomy or
tube feeding, nor were there episodes of discontinuation of
tube feeding or initiation of continuous
drip infusion due to severe complications.
Antibiotics were administered for mild
infections, a complication of CJD, with 0.2% and 8.8% of the total time after
gastrostomy being used for intravenous or transluminal administration, respectively. We compared the present patient series with that of our previous report statistically, and found that patients undergoing
gastrostomy required significantly fewer discontinuations of
tube feeding than those who did not. No significant difference in
antibiotic administration was found between groups, however. It is our conclusion that
gastrostomy should be allowed for symptom support in akinetic patients with
prion disease, but adequate informed consent must be provided to the patient's family.