Creutzfeldt–Jakob Disease

Creutzfeldt–Jakob Disease

This disease has received intense scientific scrutiny. The pri-mary features of Creutzfeldt–Jakob disease are dementia, basal ganglia and cerebellar dysfunction, myoclonus, upper motor neuron lesions and rapid progression to stupor, coma, and death in a matter of months. The disease generally affects people 65 years of age or older, with a duration of 1 month to 6 years and an average life span after disease onset of 15 months (Karp, 1984). The clinical and pathological features of Creutzfeldt– Jakob have been produced experimentally by injecting animals with brain tissue from affected adults. It has unknowingly been transferred to humans by organ transplantation, cerebral elec-trodes and pituitary growth hormone. These incidents, although tragic, illustrated the infectious nature of this condition, and the agent of transmission is believed to be a prion-containing protein (not DNA or RNA). These prions have been detected in the cerebral cortex of autopsy specimens of both patients with Creutzfeldt–Jakob disease and victims of kuru, a fatal disease transmitted by cannibalism (Kaplan et al., 1994; Prusiner, 1987). Slow viruses have also been implicated as infectious agents in kuru. Creutzfeldt–Jakob has been accidentally transferred to humans by corneal and pituitary gland transplantation, electro-encephalogram electrodes, and ingesting meat infected with the disease (mad cow disease).

The memory loss in Creutzfeldt–Jakob disease involves all phases of memory, with recent (secondary) memory being the most impaired. Personality changes, immature behavior and paranoia are early signs, and virtually every aspect of brain func-tioning can be involved. Motor disorders including rigidity, inco-ordination, paresis and ataxia usually follow.

As with subacute sclerosing panencephalitis, the EEG in Creutzfeldt–Jakob disease shows periodic complexes and biopsy specimens that reveal a characteristic spongiform encephalopa-thy and occasional amyloid plaques.