Treatment

There is currently no treatment that cures or slows progression in patients with prion diseases, but there are some treatments that may help to control some of the symptoms:

• Involuntary muscle contractions (myoclonus) can become bothersome and disabling, but usually improve with low doses of levetiracetam or clonazepam.
• Sleep problems are common. Difficulty getting restful sleep at night or movement and/or talking during sleep may improve with clonazepam. Some patients may have marked apnoea or stridor during sleep, which is an abnormal breathing sound caused by a blockage in the throat or larynx. Devices that facilitate nocturnal breathing may help.
• If hallucinations occur, the use of antipsychotics such as quetiapine should be considered.
• If a mood or anxiety disorder is present, patients may benefit from appropriate treatment with antidepressants. Those with fewer side effects are preferred.
• Swallowing problems are common in the course of the disease and require modifications in the texture of food, such as following a diet of blended solids or drinking gelified water.
• In the more advanced stages of the disease, additional patient care measures are required. You can find information about home care via this link. In advanced stages of the disease, patients often need palliative care at home or at specialist centres.
• Support for patients and families: the Foundation for Prion Diseases (Spain) and the CJD Foundation (USA) provide guidance and support for patients and families affected by prion diseases.