Highlights
- •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.
Abstract
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)Keywords
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Article info
Publication history
Published online: June 30, 2015
Accepted:
June 28,
2015
Received in revised form:
June 13,
2015
Received:
December 27,
2014
Identification
Copyright
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
