Table 1.

Quantitation of neuronal intranuclear inclusions the size of the nucleolus or larger in brain and spinal cord of Q71-B homozygotes

Brain region (midline sections) or spinal cord (few sections) Percentage of neurons with intranuclear inclusions the size of the nucleolus or larger (±SD)
Q71-B hom (3-4 months of age) Q71-B hom (8 months of age)
Olfactory bulb 6 ± 4 23 ± 10.5
Frontal cortex 0 0
Hippocampus 0 0
Thalamus 0 1.5 ± 2.5
Cerebellum cortex 0 1 ± 2.5
Deep cerebellar nuclei 18 ± 6.5 41 ± 11
Pontine nuclei 15 ± 11 41 ± 6.5
Spinal cord 16.5 ± 7.5 21.5 ± 12.5
  • Paraffin-embedded spinal cord and midsagittal brain sections of 3- to 4-month-old and 8-month-old Q71-B homozygotes (hom) were stained with ataxin-3 antibody 146, counterstained with hematoxylin, and analyzed by light microscopy. We determined the percentage of neurons with intranuclear inclusions the size of the nucleolus or larger in each microscopic field (20 neurons per field). The results are the average of the values obtained in three to six fields (60-120 neurons per brain region or spinal cord) with the corresponding SD.