Familial oculoleptomeningeal amyloidosis.

Members of a German family living in Ohio (United States) have been found to have dominantly inherited amyloidosis causing dementia, seizures, strokes, coma and visual deterioration. Pathological examination of affected individuals has shown severe, diffuse amyloidosis of the leptomeninges and subarachnoid vessels associated with patchy fibrosis and obliteration of the subarachnoid space. Amyloid deposits were prominent on the ependymal surfaces and severe and diffuse neuronal loss and generalized subpial gliosis were found in the cerebrum and cerebellum. On occasion superficial brain infarcts were seen. Amyloid was also found in the vitreous, the retinal internal limiting membrane and the retinal vessels, particularly those in the nerve fibre layer. There was only minimal amyloid elsewhere.

Chronic hereditary nephritis. A clinicopathologic study of 23 new kindreds and review of the literature.

Thirty-three patients with chronic hereditary nephritis, obtained from 23 unrelated families, were evaluated with respect to clinicopathologic features. Renal tissue was examined by light microscopy in 25 cases, immunofluorescence in 19 cases, and electron microscopy in 16 cases. The light microscopic findings varied, and foam cells were present in only four cases. Immunofluorescence was negative in all but four cases, and in these the immunomicroscopic pattern was compatible with the findings of end stage glomeruli and hyaline arteriolar sclerosis. Although electron microscopy uniformly showed marked thinning or splitting of the glomerular basement membrane, parallel splitting of the glomerular basement membrane with interposition of electron dense granular particles was seen in only eight cases. Association of glomerular basement membrane splitting with granular particles was observed in four of six patients with IgA nephropathy, in two patients with benign familial hematuria, and in a normal kidney donor. Eleven patients, seven men and four women, had chronic renal failure requiring dialysis. Of five patients who received renal allografts, three are alive, with post-transplant survival ranging from 24 to 70 months. The other two died of septicemia.