Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case

Abstract

We investigated transmission characteristics of variant Creutzfeldt-Jakob disease in a mother and son from Spain. Despite differences in patient age and disease manifestations, we found the same strain properties in these patients as in UK vCJD cases. A single strain of agent appears to be responsible for all vCJD cases to date.

Keywords: BSE; Spain; TSE; United Kingdom; mouse study; prions; strain; transmissible spongiform encephalopathy; vCJD; variant Creutzfeldt-Jakob disease.

Figure 1

Comparison of vCJD incubation periods in wild-type mice from inoculation of brain tissue homogenates from 2 patients from Spain (son, patient 1; mother, patient 2) with vCJD and a reference patient from the United Kingdom. Results show similar incubation period ranking. Incubation periods were calculated in mice showing clinical and pathologic signs of transmissible spongiform encephalopathy disease. There was a single positive case in VM mice from patient 2. Data show mean incubation period ± SEM. vCJD, variant Creutzfeldt-Jakob disease.

Figure 2

Vacuolation profile of vCJD in wild-type mice from inoculation of brain tissue homogenates from 2 patients from Spain (son, patient 1; mother, patient 2) with vCJD and a reference patient from the United Kingdom. A) RIII mice; B) C57BL/6J mice; C) VM mice. Profiles show similarities in vacuolar pathology intensity and distribution in wild-type mouse brains. Data show mean ± SEM of clinical and pathologic positive mice, with the exception of VM mice arising from the Spain patients, which also include pathologic positive only mice (n≥6 per group). G1–G9, gray matter scoring regions: G1, medulla; G2, cerebellum; G3, superior colliculus; G4, hypothalamus; G5, thalamus; G6, hippocampus; G7, septum; G8, retrosplenial and adjacent motor cortex; G9, cingulate and adjacent motor cortex. W1–W3, white matter scoring regions: W1, cerebellar white matter; W2, mesencephalic tegmentum; W3, pyramidal tract. vCJD, variant Creutzfeldt-Jakob disease.