état Marbré

THE condition known as 6tat marbr6 was first described fully by C. Vogt 24 in 1911 under the name of status marmoratus and has since been the subject of considerable interest and study. Nevertheless no full description has as yet appeared in any English journal, and therefore a brief introductory summary of the main clinical and pathological features may not come amiss. On the clinical side, the condition usually manifests itself from earliest infancy, development is retarded and more or less severe mental defect is of constant appearance. The picture is one of double choreoathetosis, sometimes combined with epilepsy. Loss of emotional control is a prominent feature, and inability to use the muscles of expression and articulation sometimes occurs. In the majority of cases the condition remains stationary; in others severe generalized paralysis, often with torsion spasm and other contractures, eventually supervenes, and death occurs before or during early adult life. Pathologically the most striking feature is the peculiar marblelike appearance of certain areas of the striate body and to a lesser degree of other parts of the brain. The clinical picture, sometimes known as Vogt's disease, is considered by some to be sufficiently characteristic to be diagnosed during life, but, as we shall point out later, there are reasons for believing that the condition is not a clear-cut clinical or pathological entity. Although uncommon, the condition is probably not so rare as might at first appear. It is probable that the majority of cases have failed to come under the care of competent neurologists; some may have been attributed to meningitis or encephalitis lethargica, while others have almost certainly been overlooked in institutions or elsewhere amongst a mass of heterogeneous cerebral palsies of childhood.