Table 1

Clinical characteristics of subjects affected by Cree encephalitis

Patient (see fig 1)Age at presentation (months)Birth OFC (gestation in weeks)Postnatal OFC (age in months)Brain calcificationWhite matter hypodensitiesCSF WCC/mm3 (age in months)†IFN-α IU/l in CSF/serum* (age in months)
OFC = occipitofrontal circumference. WCC = white cell count. BG = basal ganglia. WM = white matter. PV = periventricular. DN = dentate nuclei. NK = not known. NA = not analysed.
†Abnormal ≥5 cells/mm3.
*Normal levels <2 IU/l.
XI.8135 cm (40)41 cm (27)PVYes 9 (1);100 / 20 (1);
2 (12)100 / 25 (12)
XII.5Birth35.5 cm (40)39.5 cm (8)PVYes 9 (24)NA
XII.7133 cm (40)<2nd centile (2)BGYes47 (2)NA
XII.9BirthNKNKBG, WMYes25 (5)NA
XII.12935.5 cm (40)46 cm (20)BG, WMYes 5 (9)12/50 (9)
XIII.8233 cm (40)43 cm (48)BG, DNYes11 (48)NA
XIV.3134.5 cm (39)38 cm (3)BG, WM, PVYes 4 (3)50/NA (3)