Fig 6.

Total brain volume measurements in patients with INCL. Total brain volumes (dominated by the cerebral volume) were initially lower than normal and decreased dramatically with time. The equation for the best curve fit is shown. We did not detect a difference between INCL in boys and girls (P = .96). The normal curve reflects total brain volumes measured on 23 healthy volunteers 1.1–9.6 years of age participating in other studies at our institution; for the healthy volunteers, we did not find a statistical difference between boys and girls (P = .80) (dark line = mean, light lines = ±2SD). For comparison, the onset ages of major symptoms (mean and 95% CI) observed in our patients are plotted below the volume curve. A indicates developmental regression; B, cerebral atrophy noted in clinical MR imaging report; C, myoclonic jerks and seizures; D, loss of vision; E, deceleration of head growth; F, isoelectric visual-evoked potentials; and G, isoelectric EEG or electroretinogram.