Transmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt disease
Definition of Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease of mental function and movement.
Causes, incidence, and risk factors
CJD is believed to result from a protein called a prion. A prion causes normal proteins to fold abnormally. This affects the other proteins’ ability to function.
Signs and tests
Early in the disease, a neurological and mental status examination will show memory problems and changes in other mental functions. Later in the course of the disease, a motor system examination will show muscle twitching and spasm, a strong startle response, and an increase in muscle tone. There may also be weakness and loss of mucle tissue () and abnormal reflexes or increased normal reflex responses.
There is no known cure. Interleukins and other medications may be use in an attempt to slow the progression of the disease. Custodial care may be required early in the course of the disease. Medications may be needed to control aggressive behaviors.
The outcome is usually very poor. Within 6 months or less of the onset of symptoms, the person will become incapable of self-care.
Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 8/29/2009