Abstract
Clinicopathologic documentation of the concurrence of a sudanophilic leukodystrophy indistinguishable from “Schilder disease” with adrenal insufficiency was first reported by Siemerling and Creutzfeldt (1923). A review by Hoefnagel et al. (1962) found only 4 other cases, but the number increased rapidly in the subsequent literature (See et al., 1971, references). The publication of a series of 9 cases by Powers and Schaumburg (1973) has focussed considerable interest on the disease, and it reopened the problem of its nosologic distinction from “Schilder disease”, as discussed at the end of this chapter. A recent review of the literature (Powell et al., 1975) accounts for about 50 cases.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Aguilar, M. J., O-Brien, J. S., Taber, P.: The syndrome of familial leukodystrophy, adrenal insufficiency and cutaneous melanosis. In: Inborn Disorders of Sphingolipid Metabolism. Proc. 3rd Int. Symposium Sphingolipidoses (Aronson, S. M., Volk, B. W., eds.), pp. 149–166. London: Pergamon Press 1967.
Burton, B. K., Nadler, H. L.: Schilder–s disease: Abnormal cholesterol retention and accumulation in cultivated fibroblasts. Pediat. Res. 8: 170–175, 1974.
Christensen, E., Melchior, J., Negri, S.: A comparative study of 16 cases of diffuse sclerosis with special reference to histopathological findings. Acta neurol. scand. 37: 163–208, 1961.
Collier, J., Greenfield, J. G.: The encephalitis periaxialis of Schilder: Clinical and pathological study, with an account of two cases, one of which was diagnosed during life. Brain 47: 489–519, 1924.
Crome, L., Zapella, M.: Schilder–s disease (sudanophilic leucodystrophy) in five male members of one family. J. Neurol. Neurosurg. Psychiat. 26: 431–438, 1963.
Farkas-Bargeton, E., Sarrut, S., Philippart, M., Launay, C.: Démyélinisation du système nerveux central assocée à une atrophie cortico-surrénale. Rev. Neurol. (Paris) 117: 627–641, 1967.
Haberfeld, W., Spieler, F.: Zur diffusen Hirn-Rückenmarkssklerose im Kindesalter. Dtsch. Z. Nervenheilk. 40: 436–463, 1910.
Hoefnagel, D., Van den Noort, S., Ingbar, S. H.: Diffuse cerebral sclerosis with endocrine abnormalities in young males. Brain 85: 553–568, 1962.
Marie, P., Foix, C.: Sclérose intra-cérébrale centrolobaire et symétrique. Rev. Neurol. (Paris) 27: 1–16, 1914.
Pilz, P.: Kombination von Morbus Addison und Morbus Schilder bei einer 43jährigen Frau. Zbl. ges. Neurol. Psychiat. 208: 132–132, 1973.
Poser, C. M., van Bogaert, L.: Natural history and evolution of the concept of Schilder–s diffuse sclerosis. Acta psychiat. neurol. scand. 31: 285–331, 1956.
Powell, H., Tindall, R., Schultz, P., et al.: Adrenoleukodystrophy: Electron microscopic findings. Arch. Neurol. 32: 250–260, 1915.
Powers, J. M., Schaumburg, H. H.: The adrenal cortex in adreno-leukodystrophy. Arch. Path. (Chic.) 96: 305–310, 1973.
Powers, J. M., Schaumburg, H. H., Adrenoleukodystrophy: Similar ultrastructural changes in adrenal cortical and Schwann cells. Arch. Neurol. (Chic.) 30: 406–408, 1974.
Schaumburg, H. H., Richardson, E. P., Johnson, P. C., et al.: Schilder–s disease. Sex-linked recessive transmission with specific adrenal changes. Arch. Neurol. (Chic.) 27: 457–460, 1972.
Schenk, V. W. D., Stam, F. C., Batenburg-Plenter, A. M.: A family with sudanophilic leucodystrophy. Acta neuropath. (Berl.) 9: 233–243, 1967.
Schilder, P.: Die Encephalitis periaxialis diffusa. Arch. Psychiat. 71: 327–356, 1924.
Schilder, P.: Zur Frage der Encephalitis periaxialis diffusa (sogenannte diffuse Sklerose). Z. Neurol. Psychiat. 15: 359–376, 1913.
Schilder, P.: Zur Kenntnis der sogenannten diffusen Sklerose (Ober Encephalitis periaxialis diffusa). Z. Neurol. Psychiat. 10: 1–60, 1912.
See, G., Dayras, J.-Cl., Czernichow, P., Guesry, P.: Addison–s disease and leukodystrophy. Clinical and anatomical study. Arch. franç. Pédiat. 28: 847–864, 1971.
Siemerling, E., Creutzfeldt, H. G.: Bronzekrankheit und sklerosierende Encephalomyelitis. Arch. Psychiat. 68: 217–244, 1923.
Strunk, P., Strunk, G.: Beitrag zur Klinik und Pathomorphologie orthochromatischer Leukodystrophien. Dtsch. Z. Nervenheilk. 186: 496–510, 1965.
van Bogaert, L., Edgar, G. H. F., Karcher, D.: Type orthochromatique (à substance soudanophile) diffus de la leucodystrophie familiale. Acta neuropath. (Berl.) 1: 289–307, 1961.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 1975 Springer-Verlag Wien
About this chapter
Cite this chapter
Friede, R.L. (1975). Adreno-Leukodystrophy and Its Relation to “Schilder Disease”. In: Developmental Neuropathology. Springer, Vienna. https://doi.org/10.1007/978-3-7091-3338-5_40
Download citation
DOI: https://doi.org/10.1007/978-3-7091-3338-5_40
Publisher Name: Springer, Vienna
Print ISBN: 978-3-7091-3340-8
Online ISBN: 978-3-7091-3338-5
eBook Packages: Springer Book Archive