Comprometimento de estruturas encefálicas não hipotalâmicas na moléstia de Hand-Schüller-Christian: relato de caso e revisão da literatura

Lima, José Geraldo Camargo; Silva, Ademir Baptista da

doi:10.1590/S0004-282X1975000200008

Resumos

É relatado um caso de moléstia de Hand-Schüller-Christian que, além de elementos da tríade clássica e retração concêntrica do campo visual, apresentava uma síndrome piramidal. A raridade do comprometimento de estruturas encefálicas não hipotalâmicas nessa moléstia é realçada. É enfatizada a ação benéfica da corticoterapia e radioterapia observada no caso.

A case of Hand-Schüller-Christian disease which presented a pyramidal syndrome besides elements of the classical triad and concentric retraction of the visual field is reported. The rare involvement of non-hypothalamic encephalic strutures in that disease is emphasized together with the favourable effect of corticotherapy and radiotherapy.

Comprometimento de estruturas encefálicas não hipotalâmicas na moléstia de Hand-Schüller-Christian. Relato de caso e revisão da literatura

Involvement of non-hypothalamic encephalic structures in Hand-Schüller-Christian disease. Report of a case and review of the literature

José Geraldo Camargo Lima^I; Ademir Baptista da Silva^II

^IDepartamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina: Professor Adjunto

^IIDepartamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina: Médico estagiário

RESUMO

É relatado um caso de moléstia de Hand-Schüller-Christian que, além de elementos da tríade clássica e retração concêntrica do campo visual, apresentava uma síndrome piramidal. A raridade do comprometimento de estruturas encefálicas não hipotalâmicas nessa moléstia é realçada. É enfatizada a ação benéfica da corticoterapia e radioterapia observada no caso.

SUMMARY

A case of Hand-Schüller-Christian disease which presented a pyramidal syndrome besides elements of the classical triad and concentric retraction of the visual field is reported. The rare involvement of non-hypothalamic encephalic strutures in that disease is emphasized together with the favourable effect of corticotherapy and radiotherapy.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Departamento de Neurologia e Neurocirurgia Escola Paulista de Medicina Caixa Postal 5496 01000 São Paulo, SP Brasil.

Trabalho do Departamento de Neurologia e Neurocirurgia da Escola Paulista de Medicina, apresentado no VI Congresso Brasileiro de Neurologia, (Rio de Janeiro-GB, 8-13 julho de 1974)

1. APPALANARASAYYA, K. & DEVI, O. B. Hand-Schüller-Christian disease. Report of a case and review of the literature. International Surgery 54:353, 1970.
2. ARAGONÉS, J. M.; OLIVERAS, C.; OBACH, J.; NEVY Jr. A.; BANDRINAS, F. & JALAS, R. Aspects neurologiques de l'histiocytose X. Rev. Neurol. (Paris) 124:473, 1971.
3. AVERY, M. E.; McAFFEE, J. G. GUILDE, H. G. The course and prognosis of reticulo-endotheliosis. Amer. J. Med. 22:636, 1957.
4. BEARD, W.; FOSTER, D. B.; KEPES, J. J. & GUILLAN, R. A. Xanthomatosis of central nervous system. Neurology (Minneapolis) 20:305, 1970.
5. BENDER, J. & HOLZMAN, I. N. Histiocytosis X. Granulomatous reticuloendotheliosis whose evolution may have some bearing on the classification. Arch. Dermatol. 78:692, 1958.
6. BOSSA, G. Sul morbo di Schüller-Christian. Riforma Medica 54:315, 1938.
7. CHESTER, W. & KUGEL, V. H. Lipoid granulomatosis (type Hand-Schüller-Christian): report of a case. Arch. Path. 14.595, 1932.
8. CHIARI, H. Die generalisierte Xanthomatose vom Typus Schüller-Christian. Engerbn. d. allg. Path. U. path. Anat. 24:396, 1931.
9. CHIARI, H. Ueber veraenderungen im Zentralnervensystem bei generalisierter Xanthomatose vom Typus Schüller-Christian. Virchows Arch. f. path. Anat. 288-527, 1933.
10. DAVISON, C. Xanthomatosis and central nervous system (Schüller-Christian syndrome). Arch. Neurol. Psychiat. (Chicago) 30:75, 1933.
11. DONINI, G. & FERRARI, G. Su di un caso di sindrome di Hand-Schüler-Christian con reperto meningeo ecezionale e con lesione granulomatosa cerebrale. Giornale Psychiat. e Neuropatol. 87:51, 1959.
12. DUVAL, A. R.; LAPRESLE, J.; FARDEAU, M. & AMSTUTZ, h. Les determinations nerveuses de la maladie de Hand-Schüller-Christian: Étude d'une observation anatomo-clinique. Sem. Hop. Paris 14:1425, 1966.
13. ELIAN, M.; BORNSTEIN, B.; MATZ, S.; ASKENASY, H. M. & SANDBANK, V. Neurological manifestations of general xanthomatosis; Hand-Schüller-Christian disease. Arch. Neurol. (Chicago) 21:115, 1969.
14. FARRERAS VALENTE, P. Medicina Interna, Ed. Marin S.A., 7Ş Ed., Barcelona, 1967.
15. GOLDNER, M. G. & VOLK, B. W. Fulminant normocholesterolemic xanthomatosis (histiocytosis X). Arch. Inter. Med. 95:689, 1955.
16. HEINE, J. Beitrag zur Schüller-Christian'schen krankheit. Beitrage zur Pathologischen anatomie und zur allgeimeinen Pathologic 94:412, 1934.
17. HENSCHEN, F. Ueber Christian's Syndrome und dessen beziehnungen zur allgemeinen Xanthomatose, Acta. Paediat. (Uppsala) 12:1, 1931.
18. HERZENBERG, H. Die Skelettform der Niemann-Pickschen krakheit. Virchows Arch. f. path. Anat. 269-614, 1928.
19. IMAMURA, M. & MUROYA, K. Lymph node ultrastructure in Hand-Schüller-Christian. disease. Cancer 27:956, 1971.
20. MARINESCO, G.; DRAGANESCO, S.; STROESCO, G. & PALADE, G. Examen anatomoclinique d'un cas atipique de la maladie de Schüller-Christian. Ann. d'Anat. path. 14:673, 1937.
21. MASSOFF, W. Das Gehirn bei der Lipoidgranulomatose. Beitrag zur pathologischen Anatomie und zur allgeimeinen Pathol. 110:544, 1949.
22. MEYER, E. Hand-Schüller-Christian disease or eosinophilic xantomatous granuloma, Amer. J. Med. 15:130, 1953.
23. MILLER, A. A. & RAMSDEN, F. Neural and visceral xanthomatosis in adults. J. Clin. Path. 18:622, 1965.
24. MULLER, D. & ORTHNER, R. Intrazerebrale Lipoidgranulomatose. Beirieht uber zwei Falle. Dtsch. Ztschr. f. Nervenh. 187:608, 1965.
25. NEZELOF, C; BASSET, F. & ROUSSEAU, M. F. Histiocytosis X: histogenetic arguments for a Langerhans cell origin. Biomedicine 18:365, 1973.
26. TEILUM, G. Zerebrale und viscerale Xanthomathose mit Diabetis insipidus. Beitrage zur pathologischen Anatomie und zur allgeimeinen Pathol. 106:460, 1941.
27. WATJEN, J. Beitrag zur kenntnis des morbus Schüller-Christian. Beitrage zur pathologischen Anatomie und zur allgeimeinen Pathol. 96:443, 1936.

Datas de Publicação

Publicação nesta coleção
09 Abr 2013
Data do Fascículo
Jun 1975

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. APPALANARASAYYA, K. & DEVI, O. B. Hand-Schüller-Christian disease. Report of a case and review of the literature. International Surgery 54:353, 1970.

[2] 2. ARAGONÉS, J. M.; OLIVERAS, C.; OBACH, J.; NEVY Jr. A.; BANDRINAS, F. & JALAS, R. Aspects neurologiques de l'histiocytose X. Rev. Neurol. (Paris) 124:473, 1971.

[3] 3. AVERY, M. E.; McAFFEE, J. G. GUILDE, H. G. The course and prognosis of reticulo-endotheliosis. Amer. J. Med. 22:636, 1957.

[4] 4. BEARD, W.; FOSTER, D. B.; KEPES, J. J. & GUILLAN, R. A. Xanthomatosis of central nervous system. Neurology (Minneapolis) 20:305, 1970.

[5] 5. BENDER, J. & HOLZMAN, I. N. Histiocytosis X. Granulomatous reticuloendotheliosis whose evolution may have some bearing on the classification. Arch. Dermatol. 78:692, 1958.

[6] 6. BOSSA, G. Sul morbo di Schüller-Christian. Riforma Medica 54:315, 1938.

[7] 7. CHESTER, W. & KUGEL, V. H. Lipoid granulomatosis (type Hand-Schüller-Christian): report of a case. Arch. Path. 14.595, 1932.

[8] 8. CHIARI, H. Die generalisierte Xanthomatose vom Typus Schüller-Christian. Engerbn. d. allg. Path. U. path. Anat. 24:396, 1931.

[9] 9. CHIARI, H. Ueber veraenderungen im Zentralnervensystem bei generalisierter Xanthomatose vom Typus Schüller-Christian. Virchows Arch. f. path. Anat. 288-527, 1933.

[10] 10. DAVISON, C. Xanthomatosis and central nervous system (Schüller-Christian syndrome). Arch. Neurol. Psychiat. (Chicago) 30:75, 1933.

[11] 11. DONINI, G. & FERRARI, G. Su di un caso di sindrome di Hand-Schüler-Christian con reperto meningeo ecezionale e con lesione granulomatosa cerebrale. Giornale Psychiat. e Neuropatol. 87:51, 1959.

[12] 12. DUVAL, A. R.; LAPRESLE, J.; FARDEAU, M. & AMSTUTZ, h. Les determinations nerveuses de la maladie de Hand-Schüller-Christian: Étude d'une observation anatomo-clinique. Sem. Hop. Paris 14:1425, 1966.

[13] 13. ELIAN, M.; BORNSTEIN, B.; MATZ, S.; ASKENASY, H. M. & SANDBANK, V. Neurological manifestations of general xanthomatosis; Hand-Schüller-Christian disease. Arch. Neurol. (Chicago) 21:115, 1969.

[14] 14. FARRERAS VALENTE, P. Medicina Interna, Ed. Marin S.A., 7Ş Ed., Barcelona, 1967.

[15] 15. GOLDNER, M. G. & VOLK, B. W. Fulminant normocholesterolemic xanthomatosis (histiocytosis X). Arch. Inter. Med. 95:689, 1955.

[16] 16. HEINE, J. Beitrag zur Schüller-Christian'schen krankheit. Beitrage zur Pathologischen anatomie und zur allgeimeinen Pathologic 94:412, 1934.

[17] 17. HENSCHEN, F. Ueber Christian's Syndrome und dessen beziehnungen zur allgemeinen Xanthomatose, Acta. Paediat. (Uppsala) 12:1, 1931.

[18] 18. HERZENBERG, H. Die Skelettform der Niemann-Pickschen krakheit. Virchows Arch. f. path. Anat. 269-614, 1928.

[19] 19. IMAMURA, M. & MUROYA, K. Lymph node ultrastructure in Hand-Schüller-Christian. disease. Cancer 27:956, 1971.

[20] 20. MARINESCO, G.; DRAGANESCO, S.; STROESCO, G. & PALADE, G. Examen anatomoclinique d'un cas atipique de la maladie de Schüller-Christian. Ann. d'Anat. path. 14:673, 1937.

[21] 21. MASSOFF, W. Das Gehirn bei der Lipoidgranulomatose. Beitrag zur pathologischen Anatomie und zur allgeimeinen Pathol. 110:544, 1949.

[22] 22. MEYER, E. Hand-Schüller-Christian disease or eosinophilic xantomatous granuloma, Amer. J. Med. 15:130, 1953.

[23] 23. MILLER, A. A. & RAMSDEN, F. Neural and visceral xanthomatosis in adults. J. Clin. Path. 18:622, 1965.

[24] 24. MULLER, D. & ORTHNER, R. Intrazerebrale Lipoidgranulomatose. Beirieht uber zwei Falle. Dtsch. Ztschr. f. Nervenh. 187:608, 1965.

[25] 25. NEZELOF, C; BASSET, F. & ROUSSEAU, M. F. Histiocytosis X: histogenetic arguments for a Langerhans cell origin. Biomedicine 18:365, 1973.

[26] 26. TEILUM, G. Zerebrale und viscerale Xanthomathose mit Diabetis insipidus. Beitrage zur pathologischen Anatomie und zur allgeimeinen Pathol. 106:460, 1941.

[27] 27. WATJEN, J. Beitrag zur kenntnis des morbus Schüller-Christian. Beitrage zur pathologischen Anatomie und zur allgeimeinen Pathol. 96:443, 1936.

Brasil

Brasil

Comprometimento de estruturas encefálicas não hipotalâmicas na moléstia de Hand-Schüller-Christian: relato de caso e revisão da literatura

Involvement of non-hypothalamic encephalic structures in Hand-Schüller-Christian disease: report of a case and review of the literature

Resumos

Datas de Publicação