In 1920, two Austrian doctors called H.G.Creutzfeldt and A.Jakob first described a disease which in many respects is similar to Alzheimer's disease:

Looking at the victims brains under the microscope, they saw that many of the normal brain cells had died, causing the development of many tiny holes, too small to be seen by the naked eye, and a mesh of fine fibres. The appearance of the brain tissue resembled a microscopic sponge, and the expression "spongiform encephalopathy" was used to describe the disease. At first, suffers of Creutzfeldt-Jakob disease (CJD) are usually unaware that anything is wrong. Often there are subtle symptoms such as lapses of memory for daily events, and sometimes mood changes. Increasing apathy and loss of interest in normal daily and social activities is common. At this point, straightforward tasks that were previously simple become progressively more difficult for the sufferer. Early in the disease, the illness is frequently disregarded by friends, relatives and the sufferers themselves as the results of stress, being 'run down' or as mild depression.

Within weeks of the onset of the disease, other less easily discounted symptoms begin to appear. Neurological signs such as loss of balance, hesitancy in walking, deteriorating vision (sometimes including terrifying hallucinations), slurring and slowing of speech are common. After this, sufferers decline rapidly, becoming incontinent and experiencing involuntary, jerky movements of the limbs (known as myoclonus). Most patients slip into a comatose state without the ability to speak or move. Fortunately, the very apathy and loss of mental functions which CJD causes seems to protect victims from awareness of their fate. People affected by CJD usually die within 6 months of the onset of the disease. Only in 10% of cases does the disease run a more prolonged course of, for example, 2-5 years.

There is no known cure for CJD nor is there any treatment which can halt the progress of the disease.


© AJC 1997.