title: Comparison of the clinical course of Japanese MM1-type sporadic Creutzfeldt-Jakob disease between subacute spongiform encephalopathy and panencephalopathic-type.
abstract: BACKGROUND
  Approximately half of Japanese sporadic Creutzfeldt-Jakob disease (sCJD) cases show panencephalopathic-type (PE-type) pathology, which is a rare subtype in North Americans and Europeans. Until now, the differences in the clinical course between subacute spongiform encephalopathy (SSE) cases and PE-type cases have been unclear.
  
  
  METHODS
  To investigate the clinical course of both subtypes, clinical findings from 42 Japanese MM1-type sCJD cases (20 SSE cases and 22 PE-type cases) were retrospectively evaluated by statistical analysis.
  
  
  RESULTS
  No significant differences could be found regarding age at disease onset, the period between disease onset and first observation of myoclonus, the period between disease onset and the first observation of periodic sharp-wave complexes on electroencephalogram, or the period between disease onset and progression to the akinetic mutism state - whereas total disease duration and the period between the akinetic mutism state and death were significantly longer in PE-type cases. The prolonged disease duration was induced by the extended survival period in the akinetic mutism state. There was a statistically significant difference between the two series regarding performance of tube-feeding, but no statistically significant difference regarding performance of tracheotomy or gastrostomy. None of the cases received mechanical ventilation.
  
  
  CONCLUSIONS
  We speculate that the most crucial factor of the prolonged survival period of Japanese sCJD cases, particularly in the PE-type, is that the introduction of tube-feeding in the akinetic mutism state leads to the stabilization of the patient's general condition.
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan. Electronic address: iwasaki@sc4.so-net.ne.jp.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Iwasaki
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.
authors.forename: Shinsui
authors.initials: S
authors.lastname: Tatsumi
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.
authors.forename: Maya
authors.initials: M
authors.lastname: Mimuro
authors.affiliation: Department of Neurological Science, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Tetsuyuki
authors.initials: T
authors.lastname: Kitamoto
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.
authors.forename: Mari
authors.initials: M
authors.lastname: Yoshida
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D044466:Asian People; D007562:Creutzfeldt-Jakob Syndrome / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D018450:Disease Progression; D004569:Electroencephalography / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009207:Myoclonus / Q000473:pathology*; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Akinetic mutism state; Creutzfeldt-Jakob disease; MM1-type; Myoclonus; Periodic sharp-wave complexes; Tube-feeding