BSE - Symptoms of Creutzfeldt-Jakob disease

The pattern of symptoms of Creutzfeldt-Jakob disease (CJD) can vary depending on the type.

In sporadic CJD, the symptoms mainly affect the workings of the nervous system (neurological symptoms) and these symptoms rapidly worsen in the space of a few months.

In variant CJD, a person will usually first develop symptoms affecting their behaviour and emotions (psychological symptoms). These are then followed by additional neurological symptoms around four months later, which get worse over the following few months.

Familial CJD has the same sort of pattern as sporadic CJD, though it often takes longer for the symptoms to progress - usually around two years rather than a few months.

The pattern of iatrogenic CJD is unpredictable, as it depends on how a person becomes exposed to the infectious protein (prion) that causes CJD.

Read more about the types of Creutzfeldt-Jakob disease and causes of Creutzfeldt-Jakob disease.

Initial neurological symptoms

Initial neurological symptoms of sporadic CJD can include:

• difficulty walking, caused by problems of balance and co-ordination
• slurred speech
• numbness or pins and needles in different parts of the body
• dizziness
• vision problems such as double vision and hallucinations (seeing things that are not really there)

Initial psychological symptoms

Initial psychological symptoms of variant CJD can include:

• severe depression
• intense feelings of despair
• withdrawal from your family, friends and the world around you
• anxiety
• irritability
• difficulties sleeping (insomnia)

Advanced neurological symptoms

Advanced neurological symptoms of all forms of CJD can include:

• loss of physical co-ordination, which can affect a wide range of functions, such as walking, speaking and balance (ataxia)
• muscle twitches and spasms (myoclonus)
• loss of bladder control and bowel control
• blindness
• swallowing difficulties (dysphagia)
• loss of speech
• loss of voluntary movement (akinesia)

Advanced psychological symptoms

Advanced psychological symptoms of all forms of CJD include:

• loss of memory, which is often severe
• problems concentrating
• confusion
• feeling agitated
• aggressive behaviour
• loss of appetite, which can lead to weight loss
• paranoia, which is when you feel that people are secretly out to harm you 
• unusual and inappropriate emotional responses, such as laughing when you hear bad news or bursting into tears for no apparent reason

Final stages

As the disease progresses to its final stages, people with all forms of CJD will become totally bedridden. They often become totally unaware of their surroundings and require around-the-clock care. They also often lose the ability to speak and cannot communicate with their carers.

Death will then inevitably follow, usually in one of two ways:

• due to infection – such as the lung infection pneumonia
• due to respiratory failure – where the lungs stop working and a person is unable to breathe

Nothing can be done to prevent death in these circumstances.

Advancements in palliative care (the treatment of incurable conditions) mean than people with CJD often have a peaceful death.

Read more about treating Creutzfeldt-Jakob disease.