| United Kingdom | 180,000+ cases (1986-2020) |
| Ireland | 1,650+ cases |
| France | 910+ cases |
| Germany | 400+ cases |
| Spain | 150+ cases |
| Portugal | 100+ cases |
| Japan | 36 cases |
| United States | 4 cases (2003, 2005, 2006) |
| Canada | 20+ cases |
| Brainstem | The dorsal motor nucleus of the vagus nerve is one of the earliest and most affected regions |
| Cerebellum | Responsible for the progressive ataxia characteristic of BSE |
| Thalamus | Involved in sensory processing |
| Databases | OMIMOrphanetClinicalTrialsPubMed |