The use of antioxidants in transmissible spongiform encephalopathies: a case report.
BACKGROUND
Transmissible spongiform encephalopathies (TSE), which include Creutzfeldt-Jakob disease and new variant Creutzfeldt-Jakob disease, are diseases characterized by progressive deterioration in the central nervous system with neuronal degeneration, vacuolatization of the neuropil, and gliosis. Little is known about the pathogenic mechanisms of infection, and controversy exits around the inciting infective agent. It has been shown that an important factor in pathogenesis is the immune system.


METHODS
The reported case points to beneficial effects when antioxidant therapies are used in transmissible spongiform encephalopathies. The case revealed an early reversal in cognitive decline and subsequent improvements in myoclonus, apnea and rigidity. Although death was the ultimate outcome, the patient succumbed to the illness over 22 months after the onset of symptoms when the early rapid decline predicted demise within a few months.


CONCLUSIONS
It is possible that strategies blocking the effect of proinflammatory cytokines and the resulting oxidative damage may stem the progressive damage to the neuropil that occurs in spongiform encephalopathies. Further investigation into the use of antioxidants and other types of agents quelling inflammation needs to be undertaken. If antioxidants could be combined with treatments for the inciting infective agent, a new direction could be taken in the outcome of transmissible spongiform encephalopathies including CJD and vCJD.
Drisko
Jeanne A
JA
Department of Obstetrics and Gynecology, School of Medicine, University of Kansas Medical Center, Kansas City 66160, USA. jdrisko@kumc.edu
D000368:Aged; D000975:Antioxidants / Q000008:administration & dosage / Q000627:therapeutic use*; D007562:Creutzfeldt-Jakob Syndrome / Q000188:drug therapy; D003704:Dementia / Q000209:etiology; D019587:Dietary Supplements; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D017096:Prion Diseases / Q000188:drug therapy* / Q000276:immunology; D017382:Reactive Oxygen Species
D000975:Antioxidants; D017382:Reactive Oxygen Species