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STURGE-WEBER-DIMITRI SYNDROME: Cephalic Form of Neurocutaneous Hemangiomatosis

STURGE-WEBER-DIMITRI SYNDROME: Cephalic Form of Neurocutaneous Hemangiomatosis Abstract THE ANATOMICAL basis for Sturge-Weber-Dimitri syndrome is a hemangiomatous dysplasia in the cephalic region. Since port-wine nevi are frequently found also on the trunk and extremities, the syndrome may be considered as a partial or regional manifestation of generalized neurocutaneous hemangiomatosis. As such, it may be classified along with von Recklinghausen's disease, tuberous sclerosis, and neurocutaneous melanomatosis, for in all of these disorders the skin manifestations indicate the possible presence of changes in the nervous system, as well as in other systems. The clinical features of the syndrome are a vascular nevus of the face, associated with signs of cerebral dysfunction, such as epilepsy, mental deficiency, and hemiplegia, and roentgenographic evidence of sinuous shadows within the cranial cavity. The skin lesion is generally a nevus flammeus of the face or scalp, which often exhibits striking evidence of being confined to the areas of distribution of branches of the trigeminal nerve. References 1. Sturge, W. Allen: A Case of Partial Epilepsy, Apparently Due to a Lesion of One of the Vaso-Motor Centres of the Brain , Tr. Clin. Soc. London 12:162, 1879. 2. Kalischer, S.: Demonstration des Gehirns eines Kindes mit Teleangiectasie der linksseitigen Gesichts-Kopfhaut und Hirnoberflache , Berl. klin. Wchnschr. 34:1059, 1897. 3. Volland: Über zwei Fälle von zerebralem Angiom nebst Bemerkungen über Hirnangiome , Ztschr. Erforsch. u. Behandl. jugendl. Schwachsinns 6:130, 1913. 4. Weber, F. P.: Right-Sided Hemi-Hypotrophy Resulting from Right-Sided Congenital Spastic Hemiplegia, with a Morbid Condition of the Left Side of the Brain, Revealed by Radiograms , J. Neurol. & Psychopath. 3:134, 1922-1923. 5. Dimitri, V.: Tumor cerebral congenito (angioma cavernosum) , Rev. Asoc. méd. argent. 36: (Sect. Soc. de neurol. y psiquiat.):63, 1923. 6. Krabbe, K. H.: Facial and Meningeal Angiomatosis Associated with Calcifications of the Brain Cortex , Arch. Neurol. & Psychiat. 32:737, 1934. 7. Harvey, S. C., and Burr, H. S.: Development of the Meninges , Arch. Neurol. & Psychiat. 15:545, 1926. 8. DuShane, G. P.: Embryology of Vertebrate Pigment Cells: II. Birds , Quart. Rev. Biol. 19:98, 1944. 9. Lichtenstein, B. W., and Rosenberg, C.: Sturge-Weber-Dimitri's Disease: Report of an Abortive Case with Observations on the Form, Chemical Nature, and Pathogenesis of the Cerebral Cortical Concretions , J. Neuropath. & Exper. Neurol. 6:369, 1947. 10. Wachsmuth, N., and Löwenthal, A.: Détermination chimique d'éléments minéraux dans les calcifications intracérébrales de la maladie de Sturge-Weber , Acta neurol. et psychiat. belg. 50:305, 1950. 11. Bergstrand, H.; Olivecrona, H., and Tönnis, W.: Sturge-Webers Krankheit , Leipzig, Georg Thieme, 1936, pp. 17-35, 73-87. 12. Alexander, L., and Woodhall, B.: Calcified Epileptogenic Lesions as Caused by Incomplete Interference with the Blood Supply of the Diseased Areas , J. Neuropath. & Exper. Neurol. 2:1, 1943. 13. Lichtenstein, B. W., and Lev, M.: Calcification of the Cerebral Cortex Associated with a Meningotheliomatous Meningioma: Pathologic Study with Comment on Incomplete Types of the Neurocutaneous Syndrome , Arch. Neurol. & Psychiat. 49:507, 1943. 14. Scherer, E.: Über die pialen Lipome des Gehirns , Ztschr. ges. Neurol. u. Psychiat. 154:45, 1935. 15. Sugar, O.: Central Neurological Complications of Hypoparathyroidism , A. M. A. Arch. Neurol. & Psychiat. 70:86, 1953. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Neurology & Psychiatry American Medical Association

STURGE-WEBER-DIMITRI SYNDROME: Cephalic Form of Neurocutaneous Hemangiomatosis

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References (20)

Publisher
American Medical Association
Copyright
Copyright © 1954 American Medical Association. All Rights Reserved.
ISSN
0096-6886
DOI
10.1001/archneurpsyc.1954.02320390021002
Publisher site
See Article on Publisher Site

Abstract

Abstract THE ANATOMICAL basis for Sturge-Weber-Dimitri syndrome is a hemangiomatous dysplasia in the cephalic region. Since port-wine nevi are frequently found also on the trunk and extremities, the syndrome may be considered as a partial or regional manifestation of generalized neurocutaneous hemangiomatosis. As such, it may be classified along with von Recklinghausen's disease, tuberous sclerosis, and neurocutaneous melanomatosis, for in all of these disorders the skin manifestations indicate the possible presence of changes in the nervous system, as well as in other systems. The clinical features of the syndrome are a vascular nevus of the face, associated with signs of cerebral dysfunction, such as epilepsy, mental deficiency, and hemiplegia, and roentgenographic evidence of sinuous shadows within the cranial cavity. The skin lesion is generally a nevus flammeus of the face or scalp, which often exhibits striking evidence of being confined to the areas of distribution of branches of the trigeminal nerve. References 1. Sturge, W. Allen: A Case of Partial Epilepsy, Apparently Due to a Lesion of One of the Vaso-Motor Centres of the Brain , Tr. Clin. Soc. London 12:162, 1879. 2. Kalischer, S.: Demonstration des Gehirns eines Kindes mit Teleangiectasie der linksseitigen Gesichts-Kopfhaut und Hirnoberflache , Berl. klin. Wchnschr. 34:1059, 1897. 3. Volland: Über zwei Fälle von zerebralem Angiom nebst Bemerkungen über Hirnangiome , Ztschr. Erforsch. u. Behandl. jugendl. Schwachsinns 6:130, 1913. 4. Weber, F. P.: Right-Sided Hemi-Hypotrophy Resulting from Right-Sided Congenital Spastic Hemiplegia, with a Morbid Condition of the Left Side of the Brain, Revealed by Radiograms , J. Neurol. & Psychopath. 3:134, 1922-1923. 5. Dimitri, V.: Tumor cerebral congenito (angioma cavernosum) , Rev. Asoc. méd. argent. 36: (Sect. Soc. de neurol. y psiquiat.):63, 1923. 6. Krabbe, K. H.: Facial and Meningeal Angiomatosis Associated with Calcifications of the Brain Cortex , Arch. Neurol. & Psychiat. 32:737, 1934. 7. Harvey, S. C., and Burr, H. S.: Development of the Meninges , Arch. Neurol. & Psychiat. 15:545, 1926. 8. DuShane, G. P.: Embryology of Vertebrate Pigment Cells: II. Birds , Quart. Rev. Biol. 19:98, 1944. 9. Lichtenstein, B. W., and Rosenberg, C.: Sturge-Weber-Dimitri's Disease: Report of an Abortive Case with Observations on the Form, Chemical Nature, and Pathogenesis of the Cerebral Cortical Concretions , J. Neuropath. & Exper. Neurol. 6:369, 1947. 10. Wachsmuth, N., and Löwenthal, A.: Détermination chimique d'éléments minéraux dans les calcifications intracérébrales de la maladie de Sturge-Weber , Acta neurol. et psychiat. belg. 50:305, 1950. 11. Bergstrand, H.; Olivecrona, H., and Tönnis, W.: Sturge-Webers Krankheit , Leipzig, Georg Thieme, 1936, pp. 17-35, 73-87. 12. Alexander, L., and Woodhall, B.: Calcified Epileptogenic Lesions as Caused by Incomplete Interference with the Blood Supply of the Diseased Areas , J. Neuropath. & Exper. Neurol. 2:1, 1943. 13. Lichtenstein, B. W., and Lev, M.: Calcification of the Cerebral Cortex Associated with a Meningotheliomatous Meningioma: Pathologic Study with Comment on Incomplete Types of the Neurocutaneous Syndrome , Arch. Neurol. & Psychiat. 49:507, 1943. 14. Scherer, E.: Über die pialen Lipome des Gehirns , Ztschr. ges. Neurol. u. Psychiat. 154:45, 1935. 15. Sugar, O.: Central Neurological Complications of Hypoparathyroidism , A. M. A. Arch. Neurol. & Psychiat. 70:86, 1953.

Journal

A.M.A. Archives of Neurology & PsychiatryAmerican Medical Association

Published: Mar 1, 1954

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