Abstract | BACKGROUND: 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. CONCLUSION: 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.
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Authors | Yasushi Iwasaki, Shinsui Tatsumi, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida |
Journal | Clinical neurology and neurosurgery
(Clin Neurol Neurosurg)
Vol. 121
Pg. 59-63
(Jun 2014)
ISSN: 1872-6968 [Electronic] Netherlands |
PMID | 24793477
(Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | Copyright © 2014 Elsevier B.V. All rights reserved. |
Topics |
- Aged
- Aged, 80 and over
- Asian People
- Creutzfeldt-Jakob Syndrome
(mortality, pathology, therapy)
- Disease Progression
- Electroencephalography
(methods)
- Female
- Humans
- Male
- Middle Aged
- Myoclonus
(pathology)
- Retrospective Studies
- Treatment Outcome
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