Creutzfeldt-Jakob disease Health Article

Symptoms

• Blurred vision (sometimes)
• Changes in gait (walking)
• Hallucinations
• Lack of coordination (for example, stumbling and falling)
• Muscle twitching
• Muscle stiffness
• Myoclonic jerks or seizures
• Nervous, jumpy feelings
• Personality changes
• Profound confusion, disorientation
• Rapidly developingdelirium or dementia
• Sleepiness
• Speech impairment

Additional symptoms that may be associated with this disease:

• Memory loss
• Anxiety, stress, and tension

Once symptoms appear, the disorder progresses rapidly and may be confused with other types of dementia -- like Alzheimer's disease. Both forms of CJD, however, are distinguished by extremely rapid progression from onset of symptoms to disability and death.

Signs and tests

A neurological and motor system examination shows muscle twitching and spasm. There is a strong startle response. Muscle tone may be increased, or there may be weakness and muscle wasting (loss of muscle tissue). There may be abnormal reflexes or an increase in the response of normal reflexes.

There is loss of coordination related to visual-spatial perception changes and changes in the cerebellum, the area of the brain that controls coordination (cerebellar ataxia). An eye examination shows areas of blindness that the person may not realize are present.

Tests used to diagnose this condition may include:

• EEG
• Spinal tap

Ultimately, the disease can only be confirmed by brain biopsy or autopsy.

Treatment

There is no known cure. Custodial care may be required early in the course of the disease. Medications may be needed to control aggressive behaviors. These include sedatives, antipsychotics, and others.

Provding a safe environment, controlling aggressive or agitated behavior, and meeting physiologic needs may require monitoring and assistance in the home or in an institutionalized setting. Family counseling may help in coping with the changes required for home care.

Visiting nurses or aides, volunteer services, homemakers, adult protective services, and other community resources may be helpful in caring for the person with CJD.

Behavior modification may be helpful, in some cases, for controlling unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (within the bounds of safety). Reality orientation, with repeated reinforcement of environmental and other cues, may help reduce disorientation.

Legal advice may be appropriate early in the course of the disorder to form advance directives, power of attorney, and other legal actions that may make it easier to make ethical decisions regarding the care of an individual with CJD.