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Porencephaly, Hydranencephaly, Multilocular Cystic Encephalopathy

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Developmental Neuropathology

Abstract

Our understanding of the peculiarities of the reactions of immature nervous tissue to destructive lesions originates from the experimental investigations of Spatz (1921) who compared the histopathology of a double transection of the spinal cord in adult and newborn rabbits. The main conclusions of his 318 page report may be summarized as follows: 1. Liquefaction and dissolution of necrotic tissue develops in newborns with exceptional rapidity. Organization of the lesions is essentially completed after 8 days, and the last phagocytes have disappeared by the 12th day. There is little or no proliferation of “fixed” elements and no gliomesodermal scar develops, as organization is mainly carried out by macrophages which disappear rapidly from the lesions. The residual cavity forms a smooth-walled porus. Etiologically different lesions may produce similar residual pori. 2. The reactivity of glial tissue is very limited, in that there is no fibrillary gliosis and development of hypertrophic glial forms is rare. The changes in glial tissue may be interpreted, essentially, as an arrest in its development, particularly in regard to the abrogation of myelination gliosis. The walls of the porus are formed by a thin layer of glial tissue deficient in neurons, but there is no evidence of actual glial scarring.

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References

  • Aicardi, J., Goutières, F., de Verbois, A. H.: Multicystic encephalomalacia of infants and its relation to abnormal gestation and hydranencephaly. J. Neurol. Sci. 15: 357–373, 1972.

    Article  PubMed  CAS  Google Scholar 

  • Altshuler, G.: Toxoplasmosis as a cause of hydranencephaly. Amer. J. Dis. Child. 125: 251–252, 1973.

    PubMed  CAS  Google Scholar 

  • Bankl, H., Jellinger, K.: Zentralnervöse Schäden nach fetaler Kohlenoxydvergiftung. Beitr. path. Anat. 135: 350–376, 1967.

    CAS  Google Scholar 

  • Becker, H.: Über Hirngefäßausschaltung. II. Intracranielle Gefäßverschlüsse. Über experimentelle Hydranencephalie (Blasenhirn). Dtsch. Z. Nervenheilk. 161: 446–505, 1949.

    Google Scholar 

  • Binswanger, O.: Über eine Mißbildung des Gehirns. Virchow Arch. 87: 427–476, 1882.

    Article  Google Scholar 

  • Brocher, J. E. W.: Polyporencephalie. Z. ges. Neurol. Psychiat. 142: 107–119, 1932.

    Article  Google Scholar 

  • Cohen, M., Kristiansen, K.: Multiple encephalomalacia of infancy. Acta psychiat. neurol. scand. 29: 129–138, 1954.

    Article  CAS  Google Scholar 

  • Cohn, R., Neumann, M. A.: Porencephaly. J. Neuropath. exp. Neurol. 5: 257–270, 1946.

    Article  PubMed  CAS  Google Scholar 

  • Crome, L.: Multilocular cystic encephalopathy of infancy. J. Neurol. Neurosurg. Psychiat. 21: 146–152, 1958.

    Article  PubMed  CAS  Google Scholar 

  • Crome, L., Sylvester, P. E.: Hydranencephaly (hydrencephaly). Arch. Dis. Child. 33: 235–245, 1958.

    Article  PubMed  CAS  Google Scholar 

  • Crome, L., Williams, C.: The problem of familial multilocular encephalomalacia. Acta Paediat. 49: 175–184, 1960.

    Article  Google Scholar 

  • Cruveilhier, I.: Anatomic Pathologique du Corps Humain. Paris: Baillière 1829–1835.

    Google Scholar 

  • Cseh, I.: Multiple Cystenbildung im Gehirn. Frankfurt. Z. Path. 50: 334–546, 1937.

    Google Scholar 

  • Dahlmann, A.: Beitrag zur Kenntnis der symmetrischen Höhlen im Großhirn des Säuglings. Z. ges. Neurol. Psychiat. 3: 223–248, 1910.

    Article  Google Scholar 

  • Dekaban, A.: Large defects in cerebral hemispheres associated with cortical dysgenesis. J. Neuropath. exp. Neurol. 24: 512–530, 1965.

    Article  Google Scholar 

  • Elo, O., Otila, E.: Zum Entstehungsmechanismus der früherworbenen Hydrocephalie. Acta Paediat. 23: 503–547, 1939.

    Article  Google Scholar 

  • Farkas-Bargeton, E., Olsson, Y., Guth, L., Klatzo, I.: Glycogen reaction to cerebral stab wound during maturation of rat brain. Acta Neuropath. (Berl.) 22: 158–169, 1972.

    Article  CAS  Google Scholar 

  • Ferguson, W. F.: Perinatal mortality in multiple gestations. Obstet. Gynec. 23: 861–870, 1964.

    PubMed  CAS  Google Scholar 

  • Ford, F. R.: Disseminated encephalomalacia with cavity formation. In: Diseases of the Nervous System in Infancy, Childhood and Adolescence, Third Edition, pp. 363–366. Springfield, Ill.: C. C Thomas 1952.

    Google Scholar 

  • Fowler, M., Brown, C., Cabrera, K. F.: Hydrencephaly in a baby after an aircraft accident to the mother: Case report and autopsy. Pathology 3: 21–30, 1971.

    Article  PubMed  CAS  Google Scholar 

  • Fowler, M., Dow, R., White, T. A., Greer, C. H.: Congenital hydrocephalus-hydrencephaly in five siblings, with autopsy studies: a new disease. Develop. Med. Child. Neurol. 14: 173–188, 1972.

    Article  PubMed  CAS  Google Scholar 

  • Friede, R. Enzyme histochemistry of neuroglia. Progr. Brain Res. 35–47, 1965.

    Google Scholar 

  • Guttmacher, A. F., Kohl, S. G.: The fetus in multiple gestations. Obstet. Gynec. 12: 528–541, 1958.

    PubMed  CAS  Google Scholar 

  • Halsey, J. H., Jr., Allen, N., Chamberlin, H. R.: Chronic decerebrate state of infancy. Arch. Neurol. (Chic.) 19: 339–346, 1968.

    Article  Google Scholar 

  • Halsey, J. H., Jr., Allen, N., Chamberlin, H.R. : The morphogenesis of hydranencephaly. J. Neurol. Sci. 12: 187–218, 1971.

    Article  PubMed  Google Scholar 

  • Hartley, W. J., Alexander, G., Edwards, M. J.: Brain cavitation and micrencephaly in lambs exposed to prenatal hyperthermia. Teratology 9: 299–304, 1974.

    Article  PubMed  CAS  Google Scholar 

  • Heschl, R.: Gehirndefect und Hydrocephalus. Vjschr. f. d. prakt. Heilkunde 61: 59–74, 1859.

    Google Scholar 

  • Heschl, R.: Ein neuer Fall von Porencephalie. Vjschr. f. d. prakt. Heilkunde 72: 102–104, 1861.

    Google Scholar 

  • Ilberg, G.: Beschreibung des Centralnervensystems eines 6tägigen, syphilitischen Kindes mit unentwickeltem Großhirn. Arch. Psychiat. 34: 140–170, 1901.

    Article  Google Scholar 

  • Jakob, A.: Über ein dreieinhalb Monate altes Kind mit totaler Erweichung beider Großhirnhemisphären („Kind ohne Großhirn”). Dtsch. Z. Nervenheilk. 117–119; 240–265, 1931.

    Google Scholar 

  • Johnson, E. E., Warner, M., Simonds, J. P.: Total absence of the cerebral hemispheres. J. Pediat. 38: 69–79, 1951.

    Article  PubMed  CAS  Google Scholar 

  • Kluge, H.: Untersuchungen über Hydranencephalie (Gruveilhier). Z. Heilk. 23: 208–255, 1902.

    Google Scholar 

  • Konigsmark, B. W., Sidman, R. L.: Origin of brain macrophages in the mouse. J. Neuropath. exp. Neurol. 22: 643–676, 1963.

    Article  PubMed  CAS  Google Scholar 

  • Környey, S.: Beiträge zur Entwicklungsmechanik und Pathologie des fötalen Zentralnervensystems. Arch. Psychiat. 72: 755–787, 1925.

    Article  Google Scholar 

  • Kramer, W.: Multilocular encephalomalacia. J. Neurol. Neurosurg. Psychiat. 19: 209–216, 1956.

    Article  PubMed  CAS  Google Scholar 

  • Lange-Cosack, H.: Die Hydranencephalie (Blasenhirn) als Sonderform der Großhirnlosigkeit. Arch. Psychiat. Nervenkr. 117: 1–51; 595–640, 1944.

    Article  Google Scholar 

  • Lindenberg, R., Swanson, P. D.: “Infantile hydranencephaly”—A report of five cases of infarction of both cerebral hemispheres in infancy. Brain 90: 839–850, 1967.

    Article  PubMed  CAS  Google Scholar 

  • Lumsden, E. E.: Multiple cystic softening of the brain in the newborn. J. Neuropath. exp. Neurol. 9: 119–138, 1950.

    Article  PubMed  CAS  Google Scholar 

  • Lykke, A.: A case of congenital toxoplasmosis. Clin. Reports of the Adelaide Children Hospital 3: 62–66, 1956/57.

    Google Scholar 

  • Lyon, G., Robain, O.: Etude comparative des encéphalopathies circulatoires prénatales et para-natales (hydranencéphalies, porencéphalies et encéphalomalacies dystiques de la substance blanche). Acta Neuropath. (Berl.) 9: 79–98, 1967.

    Article  CAS  Google Scholar 

  • McElfresh, A. E., Arey, J. B.: Generalized cytomegalic inclusion disease. J. Pediat. 51: 146–156, 1957.

    Article  PubMed  CAS  Google Scholar 

  • Manterola, A., Towbin, A., Yakovlev, P. I.: Cerebral infarction in the human fetus near term. J. Neuropath. exp. Neurol. 25: 479–488, 1966.

    Article  PubMed  CAS  Google Scholar 

  • Martin, J.-J., Guazzi, G. C., de Barsy, T.: Sur l’indidence et la signification de certaines glioses fibrillaires du tronc cérébral chez les très jeunes enfants. Z. Hirnforsch. 11: 7–12, 1969.

    CAS  Google Scholar 

  • Meier, E.: Ober einen Fall von totaler Erweichung beider Grahirnhemisphären bei einem 5 Monate alten Kinde. Tb. Kinderheilk. 76: 552–567, 1912.

    Google Scholar 

  • Meyer, J. E.: Zur Ätiologie und Pathogenese des fetalen und frühkindlichen Cerebralschadens. Anatomisch-statistische Untersuchungen. Z. Kinderheilk. 67: 123–136, 1949.

    Article  Google Scholar 

  • Muir, C. S.: Hydranencephaly and allied disorders: a study of cerebral defects in Chinese children. Arch. Dis. Child. 34: 231–246, 1959.

    Article  PubMed  CAS  Google Scholar 

  • Myers, R. E.: Brain pathology following fetal vascular occlusion: An experimental study. Invest. Ophthal. 8: 41–50, 1969.

    PubMed  CAS  Google Scholar 

  • Myers, R. E.: Cystic brain alterations after incomplete placental abruption in monkey. Arch. Neurol. (Chic.) 21: 133–141, 1969.

    Article  PubMed  CAS  Google Scholar 

  • Navin, J. J., Angevine, J. M.: Congenital cytomegalic inclusion disease with porencephaly. Neurology (Minneap.) 18: 470–472, 1968.

    Article  CAS  Google Scholar 

  • Negrin, J., Lepow, H., Miller, B.: Multilocular encephalomalacia. J. Neuropath. exp. Neurol. 11: 62–68, 1952.

    Article  PubMed  Google Scholar 

  • Neuburger, F.: Fall einer intrauterinen Hirnschädigung nach Leuchtgasvergiftung der Mutter. Ber. gerichtl. Med. 13: 85–95, 1935.

    Google Scholar 

  • Norman, R. M., Urich, H., Woods, G. E.: The relationship between prenatal porencephaly and the encephalomalacias of early life. T. ment. Sci. 104: 758–771, 1958.

    CAS  Google Scholar 

  • Obersteiner, H.: Ein porencephalisches Gehirn. Arb. Neurol. Inst. Wien. Univ. 8: 1–66, 1902.

    Google Scholar 

  • Osburn, B. I., Johnson, R. T., Silverstein, A. M., Predergast, R. A., Jochim, M. M., Levy, A. B.: Experimental viral-induced congenital encephalopathies. II. The pathogenesis of bluetongue vaccine virus infection in fetal lambs. Lab. Invest. 25: 206–210, 1971.

    PubMed  CAS  Google Scholar 

  • Osburn, B. I., Johnson, R. T., Silverstein, A. M., Predergast, R. A., Johnson, R. T., Parshall, C. J., Jr.: Experimental viral-induced congenital encephalopathies. I. Pathology of hydranencephaly and porencephaly caused by bluetongue vaccine virus. Lab. Invest. 25: 197–205, 1971.

    PubMed  CAS  Google Scholar 

  • Osterberg, K. A., Wattenberg, L. W.: The age dependency of enzymes in reactive glia. Proc. Soc. exp. Biol. Med. 113: 145–148, 1963.

    PubMed  CAS  Google Scholar 

  • Otto, R.: Ein Fall von Porencephalie mit Idiotie und angeborener spastischer Gliederstarre. Arch. Psychiat. 16: 215–239, 1885.

    Article  Google Scholar 

  • Playfair, J. H. L., Wolfendale, M. R., Kay, H. E. M.: The leucocytes of peripheral blood in the human fetus. Brit. J. Haemat. 9: 336–344, 1963.

    Article  PubMed  CAS  Google Scholar 

  • Rivera, A., Jr., Brann, A. W., Jr., Myers, R. E.: Brain glycogen of the recovering asphyxiated monkey newborn. Exp. Neurol. 309–315, 1970.

    Google Scholar 

  • Rubinstein, L. J., Klatzo, I., Miguel, J.: Histochemical observations on oxidative enzyme activity of glial cells in a local brain injury. J. Neuropath. exp. Neurol. 21: 116–136, 1962.

    Article  PubMed  CAS  Google Scholar 

  • Schob, F.: Totale Erweichung beider Großhirnhemisphären bei einem zwei Monate alten Säugling. J. Psychol. Neurol. 40: 365–381, 1930.

    Google Scholar 

  • Sdiütte, E.: Die pathologische Anatomie der Porencephalie; zusammenfassendes Referat. Centralbl. Allg. Path. 13: 633–662, 1902.

    Google Scholar 

  • Seitz, L.: Über die durch intrauterine Gehirnhemorrhagien entstandenen Gehirndefecte und die Encephalitis congenita. Arch. Gynaek. 83: 701–720, 1907.

    Article  Google Scholar 

  • Solcher, H.: Zur Neuroanatomie und Neuropathologie der Frühfetalzeit. Monogr. Neurol. Psychiat. (Berl.) 127: 1–78, 1968.

    CAS  Google Scholar 

  • Spatz, H.: Über die Vorgänge nach experimenteller Rückenmarksdurchtrennung mit besonderer Berücksichtigung der Unterschiede der Reaktionsweise des reifen und des unreifen Gewebes nebst Beziehungen zur menschlichen Pathologie. (Porencephalie und Syringomyelie.) Nissl-Alzheimer Histolog. Histopathol. Arb.. EB: 49–367, 1921.

    Google Scholar 

  • Spielrneyer, W.: Ein hydranencephales Zwillingspaar. Arch. Psychiat. 39: 807–819, 1905.

    Article  Google Scholar 

  • Stevenson, L. D., McGowan, L. E.: Encephalomalacia with cavity formation in infants. Arch. Path. 34: 286–330, 1942.

    Google Scholar 

  • Stovring, J.: Kinking of the supraclinoid segment of the internal carotid artery over neighboring anatomic structures in acute and subacute intracranial mass lesions. Amer. J. Roentgenol. 104: 75–82, 1968.

    PubMed  CAS  Google Scholar 

  • Sumi, S. M., Hager, H.: Electron microscopic study of the reaction of the newborn rat brain to injury. Acta Neuropath. (Berl.) 10: 324–335, 1968.

    Article  CAS  Google Scholar 

  • Timmons, J. D., de Alvarez, R. R.: Monoamniotic twin pregnancies. Amer. J. Obstet. Gynec. 86: 875–881, 1963.

    PubMed  CAS  Google Scholar 

  • von Kahlden, L. C.: Über Porencephalie. Behr. Path. Anat. 18: 231–404, 1895.

    Google Scholar 

  • Watson, E. H.: Hydranencephaly. Amer. J. Dis. Child. 67: 282–287, 1944.

    Google Scholar 

  • Weiss, M. H., Young, H. F., McFarland, D. E.: Hydranencephaly of postnatal origin. T. Neurosura. 32: 715–720, 1970.

    Article  CAS  Google Scholar 

  • Winkelman, N. W., Moore, M. T.: Progressive degenerative encephalopathy. Occurrence in infancy, with antenatal onset simulating “swayback” of lambs; report of a case. Arch. Neurol. Psychiat. 48: 54–71, 1942.

    Google Scholar 

  • Yakovlev, P. I., Wadsworth, R. D.: Schizencephalies. A study of the congenital clefts in the cerebral mantle. I. Clefts with fused lips. J. Neuropath. exp. Neurol. 5: 116–130, 1946.

    Article  PubMed  CAS  Google Scholar 

  • Yakovlev, P. I., Wadsworth, R.D. : Schizencephalics. A study of the congenital clefts in the cerebral mantle. II. Clefts with hydrocephalus and lips separated. J. Neuropath. exp. Neurol. 5: 169–206, 1946.

    Article  PubMed  CAS  Google Scholar 

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Friede, R.L. (1975). Porencephaly, Hydranencephaly, Multilocular Cystic Encephalopathy. In: Developmental Neuropathology. Springer, Vienna. https://doi.org/10.1007/978-3-7091-3338-5_11

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  • DOI: https://doi.org/10.1007/978-3-7091-3338-5_11

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