Abstracts
Journal: Annals of the Rheumatic Diseases (1950)

Several authors have described a proliferative endarteritis with occlusion of small cerebral vessels, areas of cortical devastation, meningeal fibrosis, and the formation of nodules composed of mesodermal and glial elements, in acute rheumatic fever.Four cases are presented here because the neuropatho- logical appearances considered to be typical of chronic rheumatic encephalitis were associated with unusual neurological manifestations.The first patient, delivered by instruments of a syphilitic mother.At 3 years she had an acute attack of " brain fever" with convulsions.She became " difficult ", had an I.Q. of 43 and an enlarged heart with mitral regur- gitation.She died at 35 years of pulmonary tuberculosis.Necropsy showed occlusion of small arteries and capil- laries with endothelial and adventitial proliferation; lemon-shaped and butterfly-shaped areas of the cortex were devoid of nerve cells, and small nodules were scattered along the meninges.There was rheumatic endocarditis and mitral stenosis.The second patient did not talk until the age of 3 years.At 10 she had chorea, became " difficult" with a low I.Q.She died at 21 of tuberculous pneumonia.Necropsy revealed old rheumatic endocarditis and endarteritis of the leptomeningeal vessels, cortical areas devoid of nerve cells, and demyelination and occlusion of vessels in the basal ganglia.The third patient was normal until an attack of " brain fever" at 19 months, after which she was unable to walk or talk.Torsion and spasm of the muscles of the trunk and neck developed, with choreoathetotic movements of the limbs.A diagnosis of torsion dystonia was made.She died at the age of 19 years of bronchopneumonia.Necropsy revealed rheumatic endocarditis with mitral stenosis.The brain showed acellular cortical areas, endarteritis of meningeal vessels, and nodular thickenings of the arachnoid.The basal ganglia and cerebellum were most affected, and there were heavy deposits of iron in the globus pallidus.The fourth patient was backward from birth and spoke and walked with difficulty.At 9 there was a right-sided pyramidal defect, and later a left-sided one also.She died at 11; at necropsy large deposits of iron were found in the globus pallidus.There was much glial proliferation, especidlly in the periva°scular areas, around which there were necrosis and demyelination.The lepto- meninges were thickened and there were granulomatous nodules along the walls of the lateral ventricles.The histological findings in these four cases are those of a chronic productive encephalitis.The vascular changes in the brain, associated with similar alterations in vessels elsewhere, especially in the heart, are considered sufficiently specific to establish the meningo-encephalitis.The deposits of iron in the fourth case were far in excess of the amount expected in encephalitis and suggested a diagnosis of Hallervorden-Spatz disease, a familial condition in which extrapyramidal hyperkinesis appears at about 10, with death in the thirties.A large amount of iron pigment in the globus pallidus and substantia nigra is characteristic.The clinical signs also include pyramidal defect, gliosis and thickening of the leptomeninges.It is suggested that Hallervorden-Spatz disease may not be a degenerative morbid entity.Torsion dystonia is a clinical concept and Hallervorden-Spatz disease primarily neuropathological.The fourth case suggests that torsion dystonia and Hallervorden- Spatz disease are manifestations of rheumatic meningoencephalitis.John Marshall.