• Information
• Publications
  • Brain and Spine Booklets
  • Forthcoming titles
  • Get involved publications
  • Case studies
• Glossary
• Neurological centres
• Headstrong
• Information Access toolkit

|

There are broad similarities between all forms of CJD, involving neurological and psychiatric problems that get progressively worse. However, the precise symptoms and course do differ between types of the disease, and also between different people. No two affected people are the same.

The following section describes the early signs and symptoms of the four types of CJD. Most of those affected experience dementia (an irreversible decline of mental faculties) and physical problems such as unsteadiness or jerky movements.

For those who wish to have the information, the sections describing the signs and symptoms of sporadic and variant CJD are followed by details of how these conditions progress.

Sporadic CJD - signs and symptoms
This is often reported as having "come out of the blue." Early symptoms may be like those of depression, but progress very rapidly (within a few weeks) to the confusion and memory problems commonly seen in dementia. Neurological difficulties, such as loss of co-ordination and balance (called progressive cerebellar ataxia) together with progressive blindness usually follow, but are occasionally the first signs of CJD.

Sporadic CJD - progression
As the disease progresses, people with sporadic CJD become increasingly confused, unable to walk, to communicate, to be aware of their surroundings and to care for themselves. Brief jerking movements of the muscles (called myoclonus) occur in most cases. People usually become unable to speak, immobile and commonly die from pneumonia, to which their immobility leaves them especially susceptible.The illness is usually distressingly rapid.Three-quarters of people with sporadic CJD die within six months of diagnosis, while others die within just a few weeks. Rarely, the illness may last for a year or more.

Genetic /familial CJD
The symptoms of genetic/familial CJD vary between different people, depending partly on the type of gene mutation responsible. In some cases the illness is very like that of sporadic CJD in type, duration and progression, while in others it is a more slowly developing dementia which progresses over a few years.

Iatrogenic CJD
The symptoms of iatrogenic CJD vary according to the cause.The signs and symptoms following human dura mater grafts are generally similar to those of sporadic CJD. Human growth hormone cases follow a rather different course, with a progressive cerebellar ataxia (see above for Sporadic CJD – signs and symptoms) and dementia tending to develop later.

Variant CJD - signs and symptoms
Typically, this begins with a range of mainly emotional or behavioural disturbances, such as social withdrawal, depression, anxiety or agitation. Sometimes, delusions (abnormal beliefs) and hallucinations (abnormal experiences, including seeing or hearing things which aren’t there) may occur. In addition, patients may have unpleasant sensations, pain or numbness in the limbs or other parts of the body.

These initial features may be difficult to distinguish from primarily psychiatric or psychological problems. However, specifically neurological symptoms then develop, although often only after a few months, and usually include poor balance and clumsiness. Abnormal movements such as the restless or fidgety movements of chorea or the odd positions and posture of dystonia may be apparent. Typically, jerky muscle movements (myoclonus) appear in the later stages, and forgetfulness and confusion develop and lead into dementia.

Variant CJD - progression
People with vCJD gradually become unable to do things for themselves, and their increasing mental impairment leads to dependence on carers for ordinary daily activities.They may develop eating and swallowing difficulties and require artificial feeding.The average life expectancy following diagnosis is just over a year, and the longest recorded duration a little more than three years.