behavior/neurological
• mice exhibit gradually diminishing dystonia beginning at 10-13 weeks of age, leaving only a very mild ataxic gait
• postures of the head/neck and trunk abate first (with about 70% reduction in Purkinje cell density), with abnormal limb movements often persisting (with about 85% loss of Purkinje cells) and abating last (with about 90% loss of Purkinje cells)
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nervous system
• Purkinje cell linear density decreases with age; Purkinje cells decrease rapidly in some mice while in others, loss is slower
• Purkinje cell loss appears to be random in cerebellar regions and does not correlate with affected body parts
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