Research Article| Volume 357, ISSUE 1-2, P63-68, October 15, 2015

Download started.


Factors influencing the survival period in Japanese patients with sporadic Creutzfeldt–Jakob disease


      • 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.


      EEG (electroencephalogram), sCJD (sporadic Creutzfeldt–Jakob disease), PSWC (periodic sharp-wave complex)


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Bito S.
        • Matsumura S.
        • Singer M.K.
        • Meredith L.S.
        • Fukuhara S.
        • Wenger N.S.
        Acculturation and end-of-life decision making: comparison of Japanese and Japanese-American focus groups.
        Bioethics. 2007; 21: 251-262
        • Cali I.
        • Castellani R.
        • Yuan J.
        • Al-Shekhlee A.
        • Cohen M.L.
        • Xiao X.
        • Moleres F.J.
        • Parchi P.
        • Zou W.Q.
        • Gambetti P.
        Classification of sporadic Creutzfeldt–Jakob disease revisited.
        Brain. 2006; 129: 2266-2277
        • Iwasaki Y.
        • Mimuro M.
        • Yoshida M.
        • Kitamoto T.
        • Hashizume Y.
        Survival to akinetic mutism state in Japanese cases of MM1-type sporadic Creutzfeldt–Jakob disease is similar to Caucasians.
        Eur. J. Neurol. 2011; 18: 999-1002
        • Iwasaki Y.
        • Mori K.
        • Ito M.
        Investigation of the clinical course and treatment of prion disease patients in the akinetic mutism state in Japan.
        Rinsho Shinkeigaku. 2012; 52 (in Japanese with English abstract): 314-319
        • Iwasaki Y.
        • Tatsumi S.
        • Mimuro M.
        • Kitamoto T.
        • Hashizume Y.
        • Yoshida M.
        Relation between clinical findings and progression of cerebral cortical pathology in MM1-type sporadic Creutzfeldt–Jakob disease: proposed staging of cerebral cortical pathology.
        J. Neurol. Sci. 2014; 341: 97-104
        • Iwasaki Y.
        • Tatsumi S.
        • Mimuro M.
        • Kitamoto T.
        • Yoshida M.
        Comparison of the clinical course of Japanese MM1-type sporadic Creutzfeldt–Jakob disease between subacute spongiform encephalopathy and panencephalopathic-type.
        Clin. Neurol. Neurosurg. 2014; 121: 59-63
        • Iwasaki Y.
        • Yoshida M.
        • Hashizume Y.
        • Kitamoto T.
        • Sobue G.
        Clinicopathologic characteristics of sporadic Japanese Creutzfeldt–Jakob disease classified according to prion protein gene polymorphism and prion protein type.
        Acta Neuropathol. 2006; 112: 561-571
        • Jansen C.
        • Head M.W.
        • Rozemuller A.J.M.
        • Ironside J.W.
        Panencephalopathic Creutzfeldt–Jakob disease in the Netherlands and the UK: clinical and pathological characteristics of nine patients.
        Neuropathol. Appl. Neurobiol. 2009; 35: 272-282
        • Nagoshi K.
        • Sadakane A.
        • Nakamura Y.
        • Yamada M.
        • Mizusawa H.
        Duration of prion disease is longer in Japan than in other countries.
        J. Epidemiol. 2011; 21: 255-262
        • Parchi P.
        • Giese A.
        • Capellari S.
        • Brown P.
        • Schulz-Schaeffer W.
        • Windl O.
        • Zerr I.
        • Budka H.
        • Kopp N.
        • Piccardo P.
        • Poser S.
        • Rojiani A.
        • Streichemberger N.
        • Julien J.
        • Vital C.
        • Ghetti B.
        • Gambetti P.
        • Kretzschmar H.
        Classification of sporadic Creutzfeldt–Jakob disease based on molecular and phenotypic analysis of 300 subjects.
        Ann. Neurol. 1999; 46: 224-233
        • Smyth A.
        • Riedl M.
        • Kimura R.
        • Olick R.
        • Siegler M.
        End of life decisions in amyotrophic lateral sclerosis: a cross-cultural perspective.
        J. Neurol. Sci. 1997; 152: S93-S96