- •Fifty-one Japanese MM1-type sCJD patients were examined retrospectively.
- •Background, clinical course, and disease management were assessed.
- •Tube-fed patients (68.6%) differed significantly from those who were not tube fed (31.4%).
- •Disease duration was associated with some but not all factors examined.
- •The most crucial factor was tube feeding once akinetic mutism was reached.
Although Japanese cases of sporadic Creutzfeldt–Jakob disease (sCJD) generally involve longer survival periods compared to those from other countries, details regarding the factors influencing survival are unclear. To determine the influence of certain factors on survival, we retrospectively assessed 51 Japanese MM1-type sCJD patients with respect to background, clinical course, and disease management. No significant differences were found between men and women, tracheotomy and nontracheotomy patients, or patients treated in public and other types of hospitals. Although the survival period of tube-fed patients was significantly longer than that of patients who were not tube fed, survival of patients fed via a nasal tube did not differ significantly from that of gastrostomy-fed patients. The proportion of tube-fed patients was 68.6% (35/51). Disease duration was not significantly associated with age or year of onset. However, it was associated with time from onset to first recognition of myoclonus, first recognition of periodic sharp-wave complexes on electroencephalogram, and progression to the akinetic mutism state. Mechanical ventilation was not performed for any patient. Because the total disease duration increased in cases with a slowly progressive clinical course as a natural outcome, we concluded that the most crucial factor contributing to the prolonged survival of Japanese sCJD patients was tube feeding once the akinetic mutism state had been reached.
Abbreviations:EEG (electroencephalogram), sCJD (sporadic Creutzfeldt–Jakob disease), PSWC (periodic sharp-wave complex)
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Published online: June 30, 2015
Accepted: June 28, 2015
Received in revised form: June 13, 2015
Received: December 27, 2014
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.