Table. Demographic and clinical features of case-patients with variant CJD from the Netherlands, France, Italy, and United States and 2 reference case-patients from the United Kingdom*.
| Characteristic | The Netherlands | France | Italy | United States | United Kingdom |
|
|---|---|---|---|---|---|---|
| 1 | 2 | |||||
| Case-patient sex | F | F | F | F | M | M |
| Case-patient age at illness onset, y | 24 | 36 | 25 | 22 | 24 | 35 |
| Case-patient age at death, y | 26 | 37 | 27 | 24 | 25 | 36 |
| Disease duration, mo | 19 | 14 | 27 | 32 | 14 | 12 |
| Early psychiatric symptoms | Yes | Yes | Yes | Yes | Yes | Yes |
| Persistent painful sensory symptoms | Yes | No | Yes | No | Yes | No |
| Ataxia | Yes | Yes | Yes | Yes | Yes | Yes |
| Myoclonus, dystonia, or chorea | Yes | Yes | Yes | Yes | Yes | Yes |
| Dementia | Yes | Yes | Yes | Yes | Yes | Yes |
| No typical appearance of sporadic CJD on EEG | Yes | Yes | No | Yes | Yes | Yes |
| Bilateral symmetric pulvinar high signal on MRI scan of brain | Yes | No | Yes | Yes | Yes | No |
| Positive tonsil biopsy result | ND | Yes | Yes | Yes | ND | ND |
| Treatment | No specific treatment | No specific treatment | Quinacrine | Quinacrine | No specific treatment | No specific treatment |
| History of travel to or residence in the United Kingdom | No | No | No | Yes† | Yes | Yes |
| Codon 129MM | Yes | Yes | Yes | Yes | Yes | Yes |
| Type 2B PrP | Yes | Yes | Yes | Yes | Yes | Yes |
*CJD, Creutzfeldt-Jakob disease; EEG, electroencephalogram; MRI, magnetic resonance imaging; ND, not done; PrP, prion protein. †Born in United Kingdom in 1979, moved to United States in 1992.