title: Natural history of juvenile rheumatoid arthritis. A follow-up study of a case with special reference to clinical, electroencephalographic and neuropathological findings.
abstract: A detailed comparison between the clinical and EEG findings is made in a case of a boy with juvenile rheumatoid arthritis (JRA) who died at 15 years, 6.5 years after the beginning of the follow-up period. In the course of the disease, seven EEG recordings were made, showing a progressive diffuse slowing and disorganization with some improvement during short remissions. In relapses, diffuse slowing was associated with grave asymmetries in the EEG which, however, fluctuated and later disappeared without accompanying clinical or neuroradiological abnormalities. An abundancy of different residual findings, however, remained in the EEG after relapses. There were spike-and-wave paroxysms in every record except at the terminal stage. A stepwise slowing and disorganization was also seen in these paroxysms as background activity. The final cause of death was an intraventricular haemorrhage. No cerebral amyloidosis was found at autopsy. In conclusion, it is suggested that JRA is also a brain disease manifested as a cerebral vasculitis.
mesh_terms: D000293:Adolescent; D001171:Arthritis, Juvenile / Q000503:physiopathology; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D002543:Cerebral Hemorrhage / Q000473:pathology; D004569:Electroencephalography; D005500:Follow-Up Studies; D006801:Humans; D008297:Male