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) |