Variant Creutzfeldt–Jakob disease occurring in mother and son: Figure 1

Metabolic patterns in prion diseases: an FDG PET voxel-based analysis

“…Regarding vCJD, despite the fact that only one patient is included in this series, this is the first time that a voxel-based analysis has been performed from FDG PET of this entity [16]. Findings similar to sCJD were observed in this subject.…”

“…Particularly interesting and perhaps unexpected is the thalamic involvement, probably reflecting direct damage in this deep grey matter structure. In fact, the thalamus is clearly affected by severe gliosis in other prion diseases such as FFI and vCJD [15,16]. Moreover, thalamic hyperintensities have also been observed on brain MRI in some subjects with sCJD [1,6], complicating the differential diagnosis from vCJD.…”

Neuroimagen estructural y funcional en las enfermedades priónicas humanas

“…La vECJ afecta a pacientes más jóvenes que las formas esporádicas, y la mediana de supervivencia es de 14 meses. Recientemente se ha descrito su presentación familiar 26 . En el curso clínico inicial predominan los síntomas psiquiátricos, con alteraciones en el comportamiento, cambios en el estado de ánimo, irritabilidad y aislamiento social.…”

“…En el diagnostico diferencial deben también incluirse entidades como la hipertensión intracraneal, la enfermedad por el arañazo de gato 33 , el síndrome de Alpers 34 , síndromes paraneoplásicos 35 y encefalitis postinfecciosas 36 . Existen muy pocos estudios de neuroimagen funcional en pacientes con vECJ 26 (fig. 1).…”

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

“…Both patients were 129MM homozygous, had never received a blood transfusion or tissue graft, and had lived in the same town within the Castilla-León region of Spain ( Table 1 ) ( 1 ). The region is a farming area at high risk for bovine spongiform encephalopathy (BSE); 3 of the 5 cases of vCJD reported in Spain came from this region ( 1 ). The patients had similar eating habits, which included ingestion of bovine brain.…”