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. Author manuscript; available in PMC: 2020 Jun 10.
Published in final edited form as: Acta Neuropathol. 2019 Jul 17;138(5):859–876. doi: 10.1007/s00401-019-02043-7

Table 3.

Metrics in which a spectrum of pathological changes was observed

Category of pathological change Stain Metric Spectrum of change observed
PC cell loss LH&E PC body linear density (cells/mm)a
LH&E PC nucleolus linear density (cells/mm)
CB PC body linear density (cells/mm)
CB-GAD Percentage of empty basketsa
Heterotopic PCs LH&E Heterotopic PC linear density (cells/mm)
LH&E Heterotopic PCs per PCa
CB Heterotopic PC linear density (cells/mm)
CB Heterotopic PCs per PC
PC dendritic changes LH&E PC dendritic swelling linear density (swellings/mm)
LH&E PC dendritic swellings per PC
Bielschowsky PC dendritic swelling linear density (swellings/mm)
Bielschowsky PC dendritic swellings per PCa
CB PC dendritic swelling linear density (swellings/mm)
CB PC dendritic swellings per PC
PC axonal changes (torpedoes) LH&E Torpedo linear density (torpedoes/mm)
LH&E Torpedoes per PCa
Bielschowsky Torpedo linear density (torpedoes/mm)
Bielschowsky Torpedoes per PC
CB Torpedo linear density (torpedoes/mm)
CB Torpedoes per PCa
CB Multiple torpedo density (multiple torpedoes/mm)
PC axonal changes (other than torpedoes) CB PC axonal recurrent collaterals density (recurrent collaterals per mm)
CB PC thickened axonal profiles density (thickened axonal profiles per mm)a
CB PC axonal branching density (branches per mm)
CB PC terminal sprout rating
CB PC plexus percentage
CB PC puncta density (PC with puncta per mm)
CB PC puncta per PC
PC axonal changes (torpedo-related) CB Percentage of torpedoes with axonal recurrent collaterals
CB Torpedoes with axonal recurrent collaterals (per mm)a
CB Percentage of torpedoes with thickened axons
CB Torpedoes with thickened axons (per mm)
CB Percentage of torpedoes with branching axons
CB Torpedoes with branching axons (per mm)
Basket cell axonal hypertrophy Bielschowsky Basket cell ratinga
Climbing fiber-PC synaptic changes VGlut2 Climbing fiber synaptic density in molecular layer
VGlut2 Number of climbing fibers in the outer 20% of the molecular layera

√ = a spectrum of change was observed, with ET generally at the low end and SCA1/2/6 or MSA at the high end of severity

CB calbindin, GAD glutamic acid decarboxylase, LH&E Luxol fast blue/hematoxylin and eosin, PC Purkinje cell, VGLut2 vesicular glutamate transporter type 2

a

10 metrics in the ET neuropathological score