1980
Year 01
Prof. W. Bryan Mathews
Emeritus Professor of Neurology Oxford University, United Kingdom
THEME: SLOW VIRUS INFECTION OF THE NERVOUS SYSTEM
Two distinct groups causing serious degenerative diseases of the brain and
spinal cord are known, namely Kuru & Creutzfeldt-Jakob disease (CJD). It was
observed that along with scrapie in sheep, these diseases shared a common
pathology of spongiform degenerative disease of the brain and
encephalopathy. As the virus has not been detected, a possible alteration of
the transmissible agent by the host is suggested and slow virus disease is
placed under the category of “unconventional virus diseases”. The syndromic
presentation of CJD is cerebellar ataxia, aphasia, focal limb weakness, visual
hallucinations, myoclonus, visual failure due to cortical blindness and early
onset of dementia. Progressive spasticity, rigidity and vegetative state results
in a fatal outcome within months, with the EEG presentation being
generalised periodic spike complexes.
