Figure 4.

The cerebellar fastigial nucleus in Huntington's disease (HD). A. Sagittal section through the right cerebellum of a representative 82‐year‐old male control individual depicting the roof ridge‐like fastigial nucleus (FN). B. Complete neuronal loss in the FN of a 91‐year‐old male HD patient (CAG‐repeats in the mutated HD allele, 42; age at HD onset, 55 years; duration of HD, 36 years; Vonsattel grade of striatal atrophy, 2; patient 8; Tables 1 and 5). Arrows point to remaining FN nerve cells and inset shows severely shrunken FN nerve cell (arrow). C. Severe neuronal loss in the FN of a 70‐year‐old female HD patient (CAG‐repeats in the mutated HD allele, 44; age at HD onset, 50 years; duration of HD, 20 years; Vonsattel grade of striatal atrophy, 2; patient 7; Tables 1 and 5). Arrows mark remaining FN nerve cells and inset shows ballooned FN nerve cell (arrow): enlarged cytoplasm with basophilic center, peripheral displaced nucleus, Nissl substance and lipofuscin granules. D. The FN of a 65‐year‐old male HD patient with a severe loss of nerve cells (CAG‐repeats in the mutated HD allele, 45; age at HD onset, 53 years; duration of HD, 12 years; Vonsattel grade of striatal atrophy, 4; patient 4; Tables 1 and 5). Arrows indicate surviving FN nerve cells and inset shows ballooned surviving FN nerve cells (arrow). E. Surviving FN nerve cell of a 64‐year‐old male HD patient (CAG‐repeats in the mutated HD allele, 41; age at HD onset, 55 years; duration of HD, 9 years; Vonsattel grade of striatal atrophy, 2; patient 3; Table 1) showing a large compact cytoplasmic protein aggregate (arrow). F. Remaining FN nerve cell of a 68‐year‐old female HD patient (CAG‐repeats in the mutated HD allele, 46; age at HD onset, 40 years; duration of HD, 28 years; Vonsattel grade of striatal atrophy, 3; patient 5; Table 1) depicting granular cytoplasmic protein aggregates. (A–D) Aldehyde‐fuchsin Darrow red staining; (E,F) 1C2 immunostaining; 100 μm polyethylene glycol sections.