Pharmacological treatment with polyanions or amphotericin B in hamsters with experimental scrapie reveals that it is possible to delay the appearance of the disease only when the drug is given before the invasion of the agent into the clinical target areas of the brain. We suggest such early treatment may be possible for individuals at high risk of acquiring the disease, such as healthy mutation-positive relatives of patients with familial Creutzfeldt-Jakob disease or Gerstmann-Str~iussler syndrome, or recipients of potentially contaminated pituitary-extracted human growth hormone.
Therapy
of transmissible spongiform encephalopathies (TSE) of man, such as Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler syndrome (GSS), has been so far unsuccessful (2). This failure has been attributed to the late administration of medical treatment during the course of the disease (2). We suggest that any possible beneficial effect of anti-TSE drugs is possible only before the invasion of the infectious agent in the CNS, an event that might occur several years before the appearance of clinical signs of the disease. This assumption derives from several studies on the pathogenesis of experimental scrapie in rodents (32), which are excellent animal models of human spongiform encephalopathies.