A family is described in which 4 of 10 siblings developed a dementing illness that culminated in death within five to six years of onset. The pathological findings in 3 members were strikingly similar, and consisted of widespread nerve cell loss and astrocytosis within the cerebral cortex, status spongiosus within the outer cortical layers and, in 2, nerve cell loss and astrocytosis within the dorsomedial nucleus of the thalamus. It is concluded that the disorder described in this report does not conform precisely to any of the currently recognized categories of familial dementing disease.

A 63-year-old woman developed progressive rheumatoid disease complicated by a confusional state as well as persistent cerebrospinal fluid pleocytosis and hypoglycorrhachia. Neuropathological examination revealed extensive rheumatoid lesions in the cranial dura, falx, and choroid plexus. The choroid plexus has not been affected in any of the 16 previously reported cases of pathologically verified rheumatoid disease of the central nervous system. The findings in this patient support the observations of others regarding the role of the choroid plexus in development of CNS manifestations in systemic immune complex disease.

A 72-year-old hypertensive man had focal seizures and mild memory loss. At autopsy, there were many lacunar infarcts, particularly in the neostriatum. Rust-brown discoloration and spheroid formation within the globus pallidus and pars reticulata of the substantia nigra resembled Hallervorden-Spatz disease, in which more detailed analysis of the striatum may be worthwhile.