Hostname: page-component-cd9895bd7-fscjk Total loading time: 0 Render date: 2025-01-03T12:31:50.523Z Has data issue: false hasContentIssue false
  • English
  • Français

A Critique of Current Views on Acrocephaly and Related Conditions

Published online by Cambridge University Press:  08 February 2018

L. Crome
L. Crome*
Affiliation:
Neuropathology Department The Fountain Hospital, London
Get access Rights & Permissions [Opens in a new window]

Extract

Certain conditions associated with mental deficiency derive their name from some characteristic skeletal deformity. One of these is abnormal highheadedness or acrocephaly, a term often used interchangeably with oxycephaly, tower skull or turricephaly. To be sure, certain criteria have been recommended by some workers for confining each of the above terms to different sub-varieties of highheadedness and many alternative names have also been proposed (Günther 1941), but none of these have taken root. A distinction is made by many writers between acrocephaly and hypsocephaly or ordinary highheadedness: it is stated that the essential feature of acrocephaly is not highheadedness in itself but exaggerated upward pointing or angling of the head at some distance along its superior curvature so that, in theory, an acrocephalic head need not necessarily be hypsocephalic. However, it is doubtful if this distinction can always be sustained since many otherwise quite normal individuals have abnormal angling of the skull, while some recorded cases of acrocephaly presented a smooth and rounded profile of their upper cranial curvature in photographs and lateral skull X-rays.

Type
Original Articles
Information
Journal of Mental Science , Volume 107 , Issue 448 , May 1961 , pp. 459 - 474
Copyright
Copyright © Royal College of Psychiatrists, 1961 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Apert, M. E., “De l'acrocéphalosyndactylie”, Bull. Soc. mid. Hop., Paris, 1906, 23, 1310.Google Scholar
Idem and Regnault, F., “La dysostose acrocéphalique (à propos de l'étude du crâne d'un acrocéphalosyndactyle)”, Bull. Soc. méd. Hôp., Paris, 1929, 832.Google Scholar
Atkinson, F. R. B., “Hereditary craniofacial dysostosis, or Crouzon's disease”, Med. Press, 1937, 195, 118.Google Scholar
Benda, C. E., Developmental Disorders of Mentation and Cerebral Palsies, 1952, New York: Grune ' Stratton, p. 147.Google Scholar
Bennett, A. E., Keegan, J. J. and Hunt, H. B., “Oxycephalus: premature synostosis of the cranial sutures”, J. nerv. ment. Dis., 1936, 84, 274.CrossRefGoogle Scholar
Blank, C. E., The Genetics of Acrocephalosyndactyly, 1959, Thesis. London University.Google Scholar
Idem , “Apert's syndrome (a type of acrocephalosyndactyly)—observation on a British series of thirty-nine cases”, Ann. Hum. Genet., Lond., 1960, 24, 151.Google Scholar
Bolk, L., “On the premature obliteration of sutures in the human skull”, Amer. J. Anat., 1914–15, 17, 495.CrossRefGoogle Scholar
Böök, J. A., and Hesselvik, L., “Acrocephalosyndactyly”, Acta, paediat. (Uppsala), 1953, 42, 359.Google Scholar
Bronfenbrenner, A. N., “A case of skull deformity related to the so-called acrocephalosyndactylia”, Psychiat. Quart., 1932, 6, 612.Google Scholar
Buckley, R. W., and Yakovlev, P. I., “Dysostosis of skull, face and extremities (acrocephalosyndactyly)”, Amer. J. Dis. Child., 1948, 75, 688.Google Scholar
Bussola, E., “Acrocefalosindactilia e atrofia postneuritica dei nervi ottici”, Riv. oto-neurooftal, 1928, 5, 503.Google Scholar
Cohn, B. N. E., “True oxycephaly with syndactylism. Case report”, Amer. J. Surg., 1945, 68, 93.Google Scholar
Candreviotis, N., “Zur Entstehung des Lückenschädels”, Frankfurt. Z. Path., 1955, 66, 449.Google Scholar
Carpenter, G., “Two sisters showing malformation of the skull and other congenital abnormalities”, Reports of the Society for the Study of Diseases in Children, 1901, 1, 110 Google Scholar
Chotzen, F., “Eine eigenartige familiale Entwicklungsstörung (Akrocephalosyndaktylie, Dysostosis craniofacialis und Hypertelorismus)”, Mschr. Kinderheilk., 1932–33, 55, 97.Google Scholar
Clark, W. E. le G., The Tissues of the Body, 2nd ed., 1945, Oxford: Clarendon Press, p. 87.Google Scholar
Crome, L., “The brain and mental retardation”, Brit. méd. J., 1960, i, 897.Google Scholar
Crouzon, O., “Dysostose cranio-facial héréditaire”, Bull Soc. méd. Hôp., Paris, 1912, 33, 545.Google Scholar
Idem , Études sur les Maladies Familiales Nerveuses et Dystrophiques, 1929, Paris: Masson, p. 278.Google Scholar
Dandy, W. E., “An operation for scaphocephaly”, Arch. Surg. (Chicago), 1943, 47, 247.Google Scholar
Davis, B. F., “Acrocephalosyndac'ylism”, Amer. J. Dis. Child., 1915, 9, 446.Google Scholar
Davis, F. A., “Tower skull, oxycephalus”, Amer. J. Ophthal., 1925, 8, 513.CrossRefGoogle Scholar
Dorfmann, R., “Über Pathogenese und Therapie des Turmschädels”, v. Graefes Arch. Ophthal., 1908, 68, 412.CrossRefGoogle Scholar
Dussik, K. T., Zentralnervensystem und Sauerstoffmangelbelastung., 1949, Wilhelm Maudrich, Vienna.Google Scholar
Eshbaugh, D., “Relation of the changes in the brain to those in the skull of Crouzon's and similar diseases; with report of a case”, J. Neuropath., 1948, 7, 328.Google Scholar
Ferriman, D., Acrocephaly and Acrocephalosyndactyly., 1941, London: Oxford University Press.Google Scholar
Fletcher, H. M., “On oxycephaly”, Quart. J. Med., 1910–11, 4, 385.Google Scholar
Franceschetti, A., and Klein, D., “The mandibulo-facial dysostosis. A new hereditary syndrome”, Acta ophthal. (kbh.), 1949, 27, 141.Google Scholar
Grainger, R. B., O'Dell, B. L., and Hogan, A. G., “Congenital malformations as related to deficiencies of riboflavin and vitamin B12, source of protein, calcium to phosphorus ratio and skeletal phosphorus metabolism”, J. Nutr., 1954, 54, 33.CrossRefGoogle Scholar
Greig, D. M., “A hitherto undifferentiated congenital cranio-facial deformity”, Edinb. med. J., n.s., 1924, 31, 560.Google Scholar
Idem , “Oxycephaly”, ibid, n.s., 1926, 33, 189, 280 and 357.Google Scholar
Idem , “Acrodysplasia. Type: syndactylic oxycephaly”, ibid, n.s. 1935, 42, 537.Google Scholar
Grewel, F., “Untersuchung ueber Hypsicephalie, Turricephalie und praemature Synostose der Suturae coronariae”, Acta neerl. Morph., 1948–49, 6, 145.Google Scholar
Gross, H., “Zur Kenntnis der Beziehungen zwischen Gehirn und Schädelkapsel bei den Turricephalen, craniostenotischen Dysostosen”, Virchows Arch. path. Anat., 1957, 330, 365.Google Scholar
Günther, H., “Der Turmschädel als Konstitutionsanomalie und als klinisches Symptom”, Ergbn. inn. Med. Kinderheilk., 1931, 40, 40.Google Scholar
Idem , “Dysostosis craniofacialis hereditaria”, Endokrinologie, 1933–34, 13, 255.Google Scholar
Idem , “Hypertelorismus”, Ibid., 1933–34, 13, 61.Google Scholar
Heuyer, G., Lebovici, S., and Homualk, J., “Un cas de maladie de Crouzon avec surdité progressive. Encéphalographie gazeuse”, Arch, franç. Pediat., 1950, 7, 278.Google Scholar
Hövels, O., “Zur Systematik der Missbildungen des I. Visceralbogens unter besonderer Berücksichtigung der Dysostosis mandibulo-facialis”, Z. Kinderheilk., 1953, 73, 532 and 568.Google Scholar
Howkins, J., Jefferiss, D., and Handley, R. S., “True Oxycephaly”, Lancet, 1938, ii, 1226.Google Scholar
Ingraham, F. D., Alexander, E., and Matson, D. D., “Clinical studies in craniosynostosis”, Surgery, 1948, 24, 518.Google Scholar
Jacobsen, A. W., “Premature synostosis of cranial sutures. A report of five cases”, Arch. Pediat., 1930, 47, 556.Google Scholar
Jaensch, P. A., “Schädeldysostosen”, Z. ges. inn. Med., 1952, 7, 426.Google Scholar
Jensch, N., “Zur Genealogie und Klinik des Turmschädels”, Arch. Psychiat. Nervenkr., 1941–42, 114, 444.CrossRefGoogle Scholar
Kahn, A., and Fulmer, J., “Acrocephalosyndactylism”, New Engl. J. Med., 1955, 252, 379.Google Scholar
King, J. E. J., “Oxycephaly. A new operation and its results”, Arch. Neurol. Psychiat. (Chicago), 1938, 40, 1205.Google Scholar
Kissel, P., Dureux, J.-B., and Tridon, P., “Incidence des malformations du système nerveux au cours de malformations du squelette cranio-facial”, p. 159, 1959, In Malformations Congénitales du Cerveau, ed. by Heiyer, , Feld, and Gruner, . Paris: Masson.Google Scholar
Kreindler, A., and Schachter, M., “Sur une forme particulière de malformation craniofaciale (acrocéphalo-syndactylie, asymétrie faciale et ophtalmoptégie)”, Paris med., 1934, ii, 102.Google Scholar
Laitinen, L., Craniosynostosis. Premature Fusion of the Cranial Sutures, Helsinki, Ann. Paediat. Fenn., 1956, 2, suppt. 6.Google Scholar
Idem, and Sulamaa, M., “Craniosynostosis. Symptoms and results of treatment”, Ann. Paediat. Fenn., 1954–55, 1, 283.Google Scholar
McLaurin, R. L., and Matson, D. D., “Importance of Early Surgical Treatment of Craniosynostotis”, Pediatrics, 1952, 10, 637.Google Scholar
Martischnig, E., and Thalhammer, O., “Acrocephalosyndaktylie. (Erörterung der Genese vom Gesichtspunkt der Embryopathie)”, Helv. paediat. Acta, 1952, 7, 257.Google Scholar
Mount, L. A., “Premature closure of sutures of cranial vault—a plea for early recognition and early operation”, N. Y. St. J. Med., 1947, 47, 270.Google Scholar
Mullan, S., “Late moulding of scaphocephalic skull”, J. Dis. Child., 1960, 99, 55.Google Scholar
Park, E. A., and Powers, G. F., “Acrocephaly and scaphocephaly with symmetrically distributed malformations of the extremities”, Amer. J. Dis. Child., 1920, 20, 235.Google Scholar
Rather, L. J. (Trans.), Virchow, R. Disease, Life and Man., p. 13, 1958. Stanford: Stanford University Press.Google Scholar
Rieping, A., “Zur Pathogenese des Turmschädels”, Dtsch. Z. Chir., 1919, 148, 1.Google Scholar
Ruh, H. O., “Acrocephalosyndactylism—a teratological type”, Amer. J. Dis. Child., 1916, 11, 281.Google Scholar
Schönenberg, H., “Schädeldysostosen”, Ärztl. Wschr., 1958, 13, 909.Google Scholar
Sherne, J., “Dislocation of the eyeball as a complication of oxycephaly”, Brit. med. J., 1938, i, 565.Google Scholar
Sirkin, J., “Acrocephalosyndactylia: report of a case”, Amer. J. ment. Defic., 1943–44, 48, 335.Google Scholar
Stewart, R. M., “The nervous system in cleidocranial dysostosis: report of a case”, J. Neurol. Psychopath., 1928–29, 9, 217.Google Scholar
Swanzy, H. R., “On some of the congenital anomalies of the eye”, Trans, ophthal. Soc. U.K., 1898, 18, 1.Google Scholar
Thoma, R., “Über die prämaturen Synostosen der Schädelnähte und über das Wachstum, die Seneszenz und die Hyperostose des Schädels.”, Beitr. path. Anat., 1923–24, 72, 207.Google Scholar
Uhthoff, W., “Über hochgradigen Exophthalmus bei Schädeldeformität”, Klin. Mbl. Augenheilk., 1905, 43, 1.Google Scholar
Valentin, B., “Die Korrelation (Koppelung) von Missbildungen, erläutert am Beispiel der Akrocephalosyndaktylie”, Acta orthop. scand., 1938, 9, 235.Google Scholar
Virchow, R., “Ueber den Cretinismus, namentlich in Franken, und über pathologische Schädelformen”, Verh. phys.-med. Ges., Würzburg, 1852, 2, 230.Google Scholar
Weech, A. A., “Combined acrocephaly and syndactylism occurring in mother and daughter. A case report”, Bull. Johns Hopk. Hosp., 1927, 40, 73.Google Scholar
Weygandt, Dr., “Der Geisteszustand bei Turmschädel”, Dtsch. Z. Nervenheilk., 1921, 68 and 69, 495.Google Scholar
Weyers, H., “Zur Klinik und Pathologie der Dysostosis mandibulo-facialis”, Z. Kinderheilk., 1915, 69, 207.Google Scholar
Wigert, V., “Die Akrozephalosyndaktylie. Weiteres über die allgemeinen Skelettveränderungen”, Acta psychiat. (Kbh.), 1932, 7, 701.Google Scholar
Wheaton, S. W., “Two specimens of congenital cranial deformity in infants associated with fusion of the fingers and toes”, Trans, path. Soc. Lond., 1894, 45, 238.Google Scholar
Zellweger, H., and von Muralt, G., “Zur Pathologie des Septum pellucidum im Pneumoencephalogramm (PEG)”, Helv. paediat. Acta, 1952, 7, 229.Google Scholar
Zunin, C., “L'ipertelorismo di Greig e suoi rapporti con le sindromi di disostosi craniche e facciali”, Minerva pediat. (Torino), 1955, 7, 71.Google Scholar
Submit a response

eLetters

No eLetters have been published for this article.