A 60-year-old previously healthy man died with a progressive neurologic illness, characterized by deterioration of intellect and awareness, impaired ocular motility, and, terminally, widespread myoclonic jerks. The total course was about 11 weeks. The CSF showed a progressive increase in protein content (58 to 97 mg 100 ml), and the EEG showed diffuse slowing; laboratory investigations otherwise were unrevealing. Post-mortem examination showed extensive distinctive lesions in the thalamus and periaqueductal region bilaterally, accompanied by proliferative and inflammatory lesions in the walls of small blood vessels. These lesions were not those of subacute spongiform encephalopathy (Creutzfeldt-Jakob disease), and could not be classified with any of the known forms of human angiopathy. An infective process of an as yet unidentified type is a possibility.
ASJC Scopus subject areas
- Clinical Neurology