Symptoms of prion disease

What happens at the start?

In sporadic CJD, the most common form, the first changes are usually in thinking and movement together. Memory lapses, confusion, low mood or a change in personality are common. So is difficulty walking, because balance and coordination are affected. Speech can become slurred. Some people feel dizzy, see double or have other trouble with vision. Some have numbness or pins and needles. Sudden jerky movements of an arm or leg, called myoclonus, often start early.

Variant CJD often begins differently. Psychological symptoms tend to come first, such as severe low mood, anxiety, irritability, trouble sleeping or pulling away from family and friends. Neurological symptoms usually follow around four months later. Painful or odd sensations in the limbs are a common feature of this form.

In the inherited forms the pattern resembles sporadic CJD, and the illness often moves more slowly, over about two years.

How do symptoms change over time?

Symptoms get worse over weeks and months. As the illness goes on, coordination is lost, muscles twitch and spasm, swallowing becomes harder, speech can be lost, and some people lose their sight. Memory loss, confusion, agitation, loss of appetite and unusual emotional responses are all part of the picture.

In the last stage a person is usually in bed, needs care around the clock and is largely unaware of their surroundings. Death often comes from an infection such as pneumonia. Good care at the end of life can make this peaceful, and the team looking after the person can arrange it.

Which symptoms can be treated?

Quite a lot of them. Jerky movements can be reduced with medicine, and specialists suggest starting with one called levetiracetam when the jerks disturb sleep, comfort or care. Low mood and anxiety can be treated. Sleep can often be improved without medicine first. Pain is not usually a big problem in sporadic CJD, though it is taken seriously when it is there, and paracetamol is the usual first step.

Distress and agitation are treated as a sign of something unmet, such as pain, fear, noise, a full bladder or an unfamiliar room. Specialists advise looking for that first and adjusting what is around the person, keeping medicines for severe distress. They also note that these symptoms can settle again quite quickly, so waiting and watching is often reasonable.

Help with eating and drinking, equipment at home and nursing support all add to a person’s comfort. The teams who do this work encourage families to ask for palliative care early, long before the last weeks.

Who should I ask about what I am seeing?

Nothing here can tell you what a particular symptom means. Only the doctor who is caring for the person can do that, and symptoms like these have many other causes. If you are worried about someone, their GP or neurologist is the place to start. In the United Kingdom, people with suspected prion disease are referred to the National Prion Clinic.

What to expect covers the practical side of each stage. A fuller clinical account is in symptoms: more detail, and the other conditions in this group are listed on our diseases page.