behavior/neurological
• a severe cerebellar syndrome develops by 10 days of age
|
• locomotion is impeded anddoes not improve with age
|
head bobbing
(
J:24834
)
nervous system
• cerebellar Purkinje cells have hypotrophic and incompletely compartmentalized dendrites
• a result of late maturation of Purkinge cells and development of ectopic dendritic spines
|
• dendrites are hypotrophic and incompletely compartmentalized
• not innervated in 4 to 5 week old mice; spines are innervated in 6-14 month old mice
|
• Nissl staining shows mild granule cell degeneration
|
• normal foliation and lamination
• slightly smaller than normal
|
• abnormally large presynaptic elements
|
• spines of proximal dendritic branches are not innervated in 4 to 5 week old mice; but are in mice 6 to 14 months of age
|
muscle
• mice become spastic, especially in hind limbs
|