Gastrostomy in patients with prion disease

Abstract

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.

Identification of intracerebral hemorrhage in the early‐phase of MM1+2C‐type sporadic Creutzfeldt–Jakob disease: A case report

“…Furthermore, the Japanese social environment and traditional ethical values encourage patients with end-stage neurological disorders to receive intensive, life-sustaining treatments. 12 The cause of ICH in the present case was unclear. Although a physician did not notice hypertension in the patient initially, his blood pressure was high after admission.…”

Serial evaluation of swallowing function in a long-term survivor of V180I genetic Creutzfeldt-Jakob disease

“…To better understand dysphagia in V180I gCJD patients, we reviewed articles that described nutritional administration methods for ten patients including the current patient (Table 1) [6,8,12,[14][15][16][17]. Six of the ten patients (60%) could orally intake food after deteriorating to the state of akinetic mutism.…”

“… Case No. Author (y) Onset of Age (yrs) sex Polymorphism at the codon 129 in the PRNP gene Oral intake or Tube-feeding (Duration between onset and starting tube-fed) (m) Duration of oral intake after onset (m) Duration between onset and akinetic mutism (m) Duration of oral intake after deteriorating akinetic mutism (m) Cause of death (Duration between onset and death) (m) MRI findings Autopsy finding of brainstem Evaluation of brainstem function Evaluation of swallowing function 1 Iwasaki (2019) [ 14 ] 87 F M/M Oral intake 22 18 4 Alive Cerebral hyperintensity Brainstem preserved - N.D. N.D. 2 Iwasaki (2018) [ 15 ] 86 F M/M Oral intake 10 - N.D. General weakness (10) Cerebral hyperintensity No apparent atrophy N.D. N.D. 3 Iwasaki (2017) [ 16 ] 78 F M/M Gastrostomy (30) 30 16 14 Respiratory failure (33) Cerebral hyperintensity N.D. N.D. ...…”

Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System

“…When clinicians create the gastrostomy, they can pass deep into the stomach and abdominal walls. Therefore, clinicians treat the gastrostomy carefully at the time of its creation [14].…”