Volume 23, Number 9—September 2017
Dispatch
Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case
Table 1
Demographic and clinical features of 2 case-patients from Spain with variant CJD and reference cases from the United Kingdom*
Characteristic | Patient 1 | Patient 2 | UK cases, n = 150 |
---|---|---|---|
Patient sex | M | F | |
Case-patient age at illness onset, y | 41 | 64 | 29 (mean) |
Case-patient age at death, y | 41 | 64 | 30 (mean) |
Disease duration, mo | 9 | 7 | 14 (mean) |
Early visual symptoms | + | – | 6% |
Early unsteadiness | – | + | 11% |
No typical appearance of sporadic CJD on EEG† | + | + | 100% |
Bilateral symmetric pulvinar high signal on MRI scan of brain | Yes | Yes | 93% |
Positive tonsil biopsy result | ND | ND | 19% |
History of travel to or residence in United Kingdom | No | No | 100% |
Codon 129MM | Yes | Yes | 100%† |
Type 2B PrP | Yes | Yes | 100%† |
*CJD, Creutzfeldt-Jakob disease; EEG, electroencephalogram; MRI, magnetic resonance imaging; PrP, prion protein; ND, not done; –, negative; +, positive.
†Of those tested.
1These senior authors contributed equally to this article.
Page created: August 17, 2017
Page updated: August 17, 2017
Page reviewed: August 17, 2017
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