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Encephalocraniocutaneous Lipomatosis: A New Example of Ectomesodermal Dysgenesis

Encephalocraniocutaneous Lipomatosis: A New Example of Ectomesodermal Dysgenesis Abstract ACONGENITAL neurocutaneous disorder with the distinguishing histopathologic features of dysgenesis and neoplasia of the adipose tissue is presented in a 51/2-year-old epileptic, mentally retarded boy. To our knowledge, no such form of neurocutaneous lipomatosis has been reported. Thus the aim of this communication is threefold: (1) to give an account of the clinical and histopathologic characteristics of the disease; (2) to discuss it in relation to allied conditions; and (3) to entertain a possible pathogenetic mechanism. Report of a Case History.— A white boy, second in order of birth, was born on Aug 11, 1963. Hereditary and family history revealed no abnormalities. The mother's health during pregnancy was good, and the labor was uneventful. The birth weight was 2,779 gm (6 lb 2 ounces). He cried spontaneously. Abnormality of the right side of head, face, and right eye was noted at birth. Roentgenogram of the skull showed a slight References 1. Krabbe, K.H., and Bartels, E.D.: La lipomatose circonscrite multiple , Copenhagen: Einer Munksgaard, 1944. 2. Adair, F.E.; Pack, G.T.; and Farrior, J.H.: Lipomas , Amer J Cancer 16:1104-1120 ( (July) ) 1932. 3. Lyon, I.P.: Adiposis and Lipomatosis: Considered in Reference to Their Constitutional Relations and Symptomatology , Arch Intern Med 6:30-120 ( (July) ) 1910. 4. van Bogaert, L., and Borremans, P.: Sur une atrophie cérébelleuse corticale avec début de sclérose axiale et atteinte des noyaux gris centraux. Troubles mentaux. Lipomatose symétrique , J Neurol Psychiat 47:249-267 ( (May) ) 1947. 5. Herman, E.: Lipomatosis symetrica mit Druck-komplex in Bereiche der Cauda equina: Genesung nach subokzopitaler Lipoidolinjektion , Wien Med Wschr 88:1310-1312 ( (Dec) ) 1938. 6. Jansion, A., and Grandclaude, L.: Lipomes télangiectasiques multiples simulant une maladie de Recklinghausen , Bull Soc Franc Derm Syph 33:29-32, 1926. 7. Mollard and Chattot.: Lipomatose symétrique associée á un syndrome syringomyelique , Lyon Med 1:1049, 1908. 8. Krabbe, K.H.: Lipomatose Multiple en combinaison avec des symotomes névritiques , Acta Psychiat Neurol 14:165-178, 1939.Crossref 9. Brodie: Clinical Lectures on Surgery, Delivered at St. George's Hospital , Philadelphia: Lea and Blanchard, 201-202, 1846. 10. Madelung: Ueber den Fetthals (diffuses Lipom des Halses) , Arch Klin Chir 37:106-130, 1888. 11. Launois, and Bensaude: Bull et mém Soc med d hôp de Paris , series 3, 17:675, 1901. 12. Dold, H.: Arb path Anat Inst , Tübingen 6:563, 1908. 13. Inglis, K.: The So-Called Interscapular Gland and Tumors Arising Therein , J Anat 61:452-466 ( (July) ) 1927. 14. Brines, O.A., and Johnson, M.H.: Hibemoma, Special Fatty Tumor; Report of Case , Amer J Path 25:467-479 ( (May) ) 1949. 15. Cox, R.W.: "Hibernoma": Lipoma of Immature Adipose Tissue , J Path Bact 68:511-518 ( (Oct) ) 1954.Crossref 16. Dartigolles, U.: Lipomes diffus multiples chez une jeune fille , Bull et mem Soc med d hôp de Paris , series 3, 8:374, 1891. 17. Dieulafoy: Manuel de pathologie interne, Paris , ed 15, 1908, vol 4, p 747. 18. Dercum, and McCarthy: Autopsy in a Case of Adiposis Dolorosa , Amer J Med Sci 124:994-1006, 1902.Crossref 19. Baker, A.B., and Adams, J.M.: Lipomatosis of the Central Nervous System , Amer J Cancer 34:214-219 ( (Oct) ) 1938. 20. van Bogaert, L.: " Neuropathologie générale des phacomatoses et de quelques dystrophies neuro-cutanées moins bien connues, chez l'enfant ," in 18th Congr Assoc Pediatres Langue franc, Geneve 1961 , Basel, Switzerland: S. Karger, 1961, vol 2, pp 349-385. 21. Myle, G., and Lowenthal, A.: Lipomatose et lipolymphangiomatose faciale á localisation trigéminale, associées a des fragments de phacomatoses voisines , Acta Neurol Psychiat 51:473-486 ( (July) ) 1951. 22. Scherer, E.: Über die pialen Lipome des Gehirns, Beitrag eines Falles von ausgedehnter meningealer Lipomatose einer Grosshirnhemisphäre bei Mikrogyrie , Z Ges Neurol Psychiat 154:45-61, 1935.Crossref 23. Haberland, C., Perou, M: Bone Involvement in Sturge-Weber-Dimitri Syndrome: Combined Sturge-Weber-Dimitri and Klippel-Trénaunay-Weber Syndrome , Confin Neurol 28:413-422, 1966.Crossref 24. Alexander, G.L., and Norman, R.M.: The Sturge-Weber Syndrome , Bristol, England: John Wright & Sons Ltd., 1960. 25. Wohlwill, F.H., and Yakovlev, P.I.: Histopathology of Meningofacial Angiomatosis, Sturge-Weber Disease: Report of 4 Cases , J Neuropath Exp Neurol 16:341-364 ( (July) ) 1957.Crossref 26. Nellhaus, G.; Haberland, C.; and Hill, B.J.: Sturge-Weber Disease With Bilateral Intracranial Calcifications at Birth and Unusual Pathologic Findings , Acta Neurol Scand 43:314-347, 1967.Crossref 27. Yakovlev, P.I., and Wadsworth, R.: Schizencephalies: A Study of the Congenital Clefts in the Cerebral Mantle , J Neuropath Exp Neurol 5:169-206 ( (July) ) 1946.Crossref 28. Dekaban, A.: Large Defects in Cerebral Hemispheres Associated With Cortical Dysgenesis , J Neuropath Exp Neurol 26:512-530 ( (July) ) 1965.Crossref 29. Hallervorden, J.: Uber eine Kohlenoxydvergiftung im Fetalleben mit Entwicklungsstörung der Hirnrinde , Allg Z Psychiat 124:289, 1949. 30. Bartelmez, G.W., and Dekaban, A.S.: The Early Development of the Human Brain , Carnegie Institution of Washington, publ 621, Contribution to Embryology , No. 253, 37:15-32, 1962. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Encephalocraniocutaneous Lipomatosis: A New Example of Ectomesodermal Dysgenesis

Archives of Neurology , Volume 22 (2) – Feb 1, 1970

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References (21)

Publisher
American Medical Association
Copyright
Copyright © 1970 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1970.00480200050005
Publisher site
See Article on Publisher Site

Abstract

Abstract ACONGENITAL neurocutaneous disorder with the distinguishing histopathologic features of dysgenesis and neoplasia of the adipose tissue is presented in a 51/2-year-old epileptic, mentally retarded boy. To our knowledge, no such form of neurocutaneous lipomatosis has been reported. Thus the aim of this communication is threefold: (1) to give an account of the clinical and histopathologic characteristics of the disease; (2) to discuss it in relation to allied conditions; and (3) to entertain a possible pathogenetic mechanism. Report of a Case History.— A white boy, second in order of birth, was born on Aug 11, 1963. Hereditary and family history revealed no abnormalities. The mother's health during pregnancy was good, and the labor was uneventful. The birth weight was 2,779 gm (6 lb 2 ounces). He cried spontaneously. Abnormality of the right side of head, face, and right eye was noted at birth. Roentgenogram of the skull showed a slight References 1. Krabbe, K.H., and Bartels, E.D.: La lipomatose circonscrite multiple , Copenhagen: Einer Munksgaard, 1944. 2. Adair, F.E.; Pack, G.T.; and Farrior, J.H.: Lipomas , Amer J Cancer 16:1104-1120 ( (July) ) 1932. 3. Lyon, I.P.: Adiposis and Lipomatosis: Considered in Reference to Their Constitutional Relations and Symptomatology , Arch Intern Med 6:30-120 ( (July) ) 1910. 4. van Bogaert, L., and Borremans, P.: Sur une atrophie cérébelleuse corticale avec début de sclérose axiale et atteinte des noyaux gris centraux. Troubles mentaux. Lipomatose symétrique , J Neurol Psychiat 47:249-267 ( (May) ) 1947. 5. Herman, E.: Lipomatosis symetrica mit Druck-komplex in Bereiche der Cauda equina: Genesung nach subokzopitaler Lipoidolinjektion , Wien Med Wschr 88:1310-1312 ( (Dec) ) 1938. 6. Jansion, A., and Grandclaude, L.: Lipomes télangiectasiques multiples simulant une maladie de Recklinghausen , Bull Soc Franc Derm Syph 33:29-32, 1926. 7. Mollard and Chattot.: Lipomatose symétrique associée á un syndrome syringomyelique , Lyon Med 1:1049, 1908. 8. Krabbe, K.H.: Lipomatose Multiple en combinaison avec des symotomes névritiques , Acta Psychiat Neurol 14:165-178, 1939.Crossref 9. Brodie: Clinical Lectures on Surgery, Delivered at St. George's Hospital , Philadelphia: Lea and Blanchard, 201-202, 1846. 10. Madelung: Ueber den Fetthals (diffuses Lipom des Halses) , Arch Klin Chir 37:106-130, 1888. 11. Launois, and Bensaude: Bull et mém Soc med d hôp de Paris , series 3, 17:675, 1901. 12. Dold, H.: Arb path Anat Inst , Tübingen 6:563, 1908. 13. Inglis, K.: The So-Called Interscapular Gland and Tumors Arising Therein , J Anat 61:452-466 ( (July) ) 1927. 14. Brines, O.A., and Johnson, M.H.: Hibemoma, Special Fatty Tumor; Report of Case , Amer J Path 25:467-479 ( (May) ) 1949. 15. Cox, R.W.: "Hibernoma": Lipoma of Immature Adipose Tissue , J Path Bact 68:511-518 ( (Oct) ) 1954.Crossref 16. Dartigolles, U.: Lipomes diffus multiples chez une jeune fille , Bull et mem Soc med d hôp de Paris , series 3, 8:374, 1891. 17. Dieulafoy: Manuel de pathologie interne, Paris , ed 15, 1908, vol 4, p 747. 18. Dercum, and McCarthy: Autopsy in a Case of Adiposis Dolorosa , Amer J Med Sci 124:994-1006, 1902.Crossref 19. Baker, A.B., and Adams, J.M.: Lipomatosis of the Central Nervous System , Amer J Cancer 34:214-219 ( (Oct) ) 1938. 20. van Bogaert, L.: " Neuropathologie générale des phacomatoses et de quelques dystrophies neuro-cutanées moins bien connues, chez l'enfant ," in 18th Congr Assoc Pediatres Langue franc, Geneve 1961 , Basel, Switzerland: S. Karger, 1961, vol 2, pp 349-385. 21. Myle, G., and Lowenthal, A.: Lipomatose et lipolymphangiomatose faciale á localisation trigéminale, associées a des fragments de phacomatoses voisines , Acta Neurol Psychiat 51:473-486 ( (July) ) 1951. 22. Scherer, E.: Über die pialen Lipome des Gehirns, Beitrag eines Falles von ausgedehnter meningealer Lipomatose einer Grosshirnhemisphäre bei Mikrogyrie , Z Ges Neurol Psychiat 154:45-61, 1935.Crossref 23. Haberland, C., Perou, M: Bone Involvement in Sturge-Weber-Dimitri Syndrome: Combined Sturge-Weber-Dimitri and Klippel-Trénaunay-Weber Syndrome , Confin Neurol 28:413-422, 1966.Crossref 24. Alexander, G.L., and Norman, R.M.: The Sturge-Weber Syndrome , Bristol, England: John Wright & Sons Ltd., 1960. 25. Wohlwill, F.H., and Yakovlev, P.I.: Histopathology of Meningofacial Angiomatosis, Sturge-Weber Disease: Report of 4 Cases , J Neuropath Exp Neurol 16:341-364 ( (July) ) 1957.Crossref 26. Nellhaus, G.; Haberland, C.; and Hill, B.J.: Sturge-Weber Disease With Bilateral Intracranial Calcifications at Birth and Unusual Pathologic Findings , Acta Neurol Scand 43:314-347, 1967.Crossref 27. Yakovlev, P.I., and Wadsworth, R.: Schizencephalies: A Study of the Congenital Clefts in the Cerebral Mantle , J Neuropath Exp Neurol 5:169-206 ( (July) ) 1946.Crossref 28. Dekaban, A.: Large Defects in Cerebral Hemispheres Associated With Cortical Dysgenesis , J Neuropath Exp Neurol 26:512-530 ( (July) ) 1965.Crossref 29. Hallervorden, J.: Uber eine Kohlenoxydvergiftung im Fetalleben mit Entwicklungsstörung der Hirnrinde , Allg Z Psychiat 124:289, 1949. 30. Bartelmez, G.W., and Dekaban, A.S.: The Early Development of the Human Brain , Carnegie Institution of Washington, publ 621, Contribution to Embryology , No. 253, 37:15-32, 1962.

Journal

Archives of NeurologyAmerican Medical Association

Published: Feb 1, 1970

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