Summary
The various morphological manifestations of intracranial vascular anomalies include the rare diffuse meningocerebral angiomatosis, sometimes termed “angiodysgenetic necrotising encephalopathy” on account of the severe associated hypoxic damage to the cerebral cortex and white matter. It may occur in any age group and displays both sporadic and familial forms. A further case is reported which is apparently unique in presenting as an intrauterine death with extensive subarachnoid haemorrhage. Associated pathological abnormalities of the adrenal glands indicate that cerebral damage may have commenced from as early as the 20th week of pregnancy. Possible relationships of this entity to other central nervous system vascular malformations are discussed.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Alexander GL, Norman RM (1960) The Sturge-Weber syndrome. Wright, Bristol
Arseni C, Nereantiu F, Nicolescu P (1973) The infantile form of diffuse sclerosis with meningeal angiomatosis. Neurology 23:1297–1301
Baro F (1964) Angiomatose méningée non calcificante granulaire de l'écorce, sclérose diffuse axiale et cutis marmorata congenita. Nouvelle observation clinique sporadique du syndrome décrit par Divry et van Bogaert, 1946. Acta Neurol Belg 64:1042–1063
Bruens JH, Guazzi GG, Marin JJ (1968) Infantile form of meningeal angiomatosis with sudanophilic leucodystrophy with complex abiotrophies. Study of a second family. J Neurol Sci 7:417–425
Byrnes AL, Boellaard JW, (1958) Renal agenesis and meningocerebral angiomatosis. Arch Pathol, 66:23–31
Divry P, van Bogaert L (1946) Une maladie familiale charactérisé par une angiomatose diffuse cortico-méningée non calcificante et une démyélinisation progressive de la substance blanche. J Neurol Neurosurg Psychiatry 9:41–45
Foix C, Alajouanine T (1926) La myélite nécrotique subaigue, Myélite centrale angio-hypertrophique à évolution progressive. Paraplégie amyotrophique lentement ascendante, d'abord spasmodique, puis flasque, s'accompagnant de dissociation albumino-cytologique. Rev Neurol (Paris) 33:1–42
Friede RL (1989)Developmental neuropathology. Springer-Verlag, Berlin Heidelberg New York Tokyo pp 395–404
Guazzi GG, Martin JJ (1967) La Iorme infantile de la leucodystrophie sudanophile avec angiomatose méningée non calcificante. Acta Neurol Belg 67:463–474
Hooft C, Delore G, Bogaert L van, Guazzi GG (1965) Sudanophilic leukodystrophy with meningeal angiomatosis in two brothers: infantile form of diffuse sclerosis with meningeal angiomatosis. J Neurol Sci 2:30–51
Hori A, Jacob H (1974) Diffuse Memigoleukoangiomatosen mit sekundärer Pseudoverkalkung der Axone im tiefen Mark bei makrocephalem, chondrodystrophischem Neugeborenen. Acta Neuropathol (Berl) 28:273–277
Jellinger K (1986) Vascular malformations of the central nervous system: a morphological overview. Neurosurg Rev 9:117–216
Jellinger K, Kucsko L, Seitelberger F (1966) Diffuse meningocerebrale Angiodysplasie mit hypoplasiogener Isthumusstenose bei einem Neugeborenen. Beitr Pathol Anat Allg Pathol 33:41–72
Louis-Bar D (1950) Les rapportes entre les angiomatoses du type Sturge-Weber et les autres dysplasies (Forme de passage). Acta neurol Belg 50:680–688
Maekawa K, Suga Y, Shirai S, Maki Y, Hori A (1979) A case of tuberous sclerosis with calcification of leukoangiomatosi. Brain Dev 1:127–132
Martin JJ, Navarro C, Roussel JM, Michielsen P (1973) Familial capillaro-venous leptomeningeal angiomatosis. Eur Neurol 9:202–215
Nellhaus G, Haberland C, Hill BJ (1967) Sturge-Weber disease with bilateral intracranial calcifications at birth and unusual pathologic findings. Acta Neurol Scand 43:314–347
Ogasawara S, Nittame G (1976) A case of tuberous sclerosis with capillary angiomatosis. Adv Neurol Sci 20:557–562
Potter EL (1948) Diffuse angiectasis of the cerebral meninges in the newborn infant. Arch Pathol 46:87–96
Rothemund E, Dietl H, Anzil AP, Mehraein P (1981) Gibt es eine angiodysgenetische nekrotisierende Encephalopathie? Jahrestagung der Deutschen Gesellschat für Neuropathologie und Neuroanatomie e.V., Freiburg i. Br., October 7–10, 1981.
Schmitt HP, Bersch W, Carls C (1988) “Angiodysgenetic necrotising encephalopathy” and its different manifestations. Acta neuropathol (Berl) 75:621–626
Stehbens WE, Sahgal KK, Nelson L, Shaher RM (1973) Aneurysm of vein of Galen and diffuse meningeal angiectasia. Arch Pathol 95:333–335
Urich H (1976) Encephalofacial angiomatosis (Sturge-Weber disease). In: Blackwood W, Corsellis JAN (eds) Greenfield's neuropathology, 3rd edn. Arnold, London, pp 417–421
van Bogaert L, Marin JJ (1971) Analyse critique de la pathologie de l'angiomatose cerebromeningce diffuse non calcificante et de l'encephalopathie de Binswanger. J Neurol Sci 14:301–314
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Bloxham, C.A. Angiodysgenetic necrotising encephalopathy: presentation as an intrauterine death. Acta Neuropathol 84, 221–224 (1992). https://doi.org/10.1007/BF00311400
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00311400