Some Problems of Pathogenesis in Schilder's Disease

Abstract

Since we do not yet know the real nature of Schilder's disease nor see a direct way of investigating it, research on this problem can usefully be carried out in two ways: (1) careful description of cases in all possible clinical and pathological detail, and (2) broad comparative studies. That collection of further case-material is still useful was shown, we hope, in the description of a family by one of us (A. M.) in collaboration with Tennent (24). In this family there was a pronounced tendency to gastro-intestinal troubles preceding the nervous symptoms, and the same has been reported by Globus and Strauss (15), Globus (14), and F. Bielschowsky (2). Too much importance must not be attached to such findings, as the onset of the disease just as often follows measles, influenza and other acute infectious conditions; and there is the striking familial taint of tuberculosis in the families described by Symonds (45) and van Bogaert and Scholz (47), and atrophy of the suprarenals was associated with the disease in the latest case reported by R. Pfister (29). The great variety of such preceding illnesses makes us rather chary of regarding them as of direct ætiological or pathogenic significance. It is, on the other hand, of the greatest importance to keep such occurrences carefully in mind, because we do not know yet what common pathogenic factor may lie hidden behind these various initial symptoms. As regards gastro-intestinal troubles, their significance in various avitaminous and other deficiency conditions is generally known, and they have been a very obvious initial symptom in the demyelinizing disease of monkeys, the histopathological kinship of which to Schilder's disease has already been shown in A. Meyer and Tennent's paper, and which will be discussed later in this contribution. This kinship appears to us to be closer than was recognized by Schob (35), Spielmeyer (42), Scherer (33), and Davison (9).