Table 1 Comparison of neurological signs, pathological and biochemical features of the BSE/vCJD vs. myelopathic syndrome in primates
BSE/vCJD | Myelopathy | |
|---|---|---|
Neurological signs | ||
Behaviour | Aggressiveness | No obvious modification |
Tremors | Important permanent, increased during movement | Inconstant, very subtle |
Ataxia | Cerebellar ataxia | Ataxia of limbs |
Loss of equilibrium | ||
Sensory symptoms | Hyperreactivity (jump without habituation following visual or auditory stimulus) | No evidence for hyperreactivity |
Impaired precise vision | ||
Sensitive symptoms | Apparent exacerbated hyperaesthesia of limbs | Apparent exacerbated hyperaesthesia of limbs |
Motricity | Uncoordinated locomotion | Dysmetria/fine motor impairment of upper limbs (animals failed to catch tiny dry grapes but not balls, then systematically caught food with their mouths), followed by progressive paresis (vacuum cleaner feeding) |
Lesions | ||
Hemispheres | Spongiform change, gliosis, neuronal lesions | No obvious lesion |
Wallerian degeneration of optic tracts | ||
Cerebellum | Granule cells moderately rarefied | |
Medulla oblongata | Bilateral necrotic lesions of spinal nuclei of trigeminal nerve | |
Spinal cord | Bilateral necrotic lesions of anterior horns (lower cervical cord) | |
Wallerian degeneration of gracilis funiculi | ||
PrP | ||
Brain | PK-resistant PrPd detectable with all the techniques | No detectable PrPd |
Spinal cord | PK-resistant PrPd detectable with all the techniques (grey matter) | No detectable PrPd (except R5 primate) |
