Fig. 1

Histology and immunohistochemistry of ballooned neurons in cortex of the cingulum. a View of cortex in the cingulum showing several ballooned neurons, in Case 3 (HPS, ×200). b High power view showing one ballooned neuron with inclusion-like appearance, in Case 1 (HPS). c Low power view of the cingulum in Case 3 showing greater number of ballooned neurons in layers 4–6 of the cerebral cortex, without topographical pattern (αB-crystallin, ×50). d High power view of αB-crystallin showing strong positivity in ballooned neurons and their projections as well as positive neuropil thread, in Case 1 (αB-crystallin, ×400). e In the cortex of the cingulum, in Case 1, αB-crystallin positive ballooned neurons, one with cleft and positive rim of astrocytic processes; positive neuropil threads are also seen (αB-crystallin, ×600). f Positivity of ballooned neurons with NF200, in Case 1 (NF200, ×600)