Granuloma eosinófilo da coluna cervical com manifestação neurológica, liquorica e radiológica atípica

Reed, Umbertina Conti; Bresolin, Antonio Umberto; Lefèvre, Antonio Branco

doi:10.1590/S0004-282X1975000200010

Resumos

É relatado um caso de granuloma eosinófilo da coluna cervical em menino branco de 10 anos de idade que apresentava dor cervical na altura de C5-C6, hemiparesia direita que evoluiu ulteriormente para tetraparesia, mista, de tipo motonêuronio inferior para membros superiores e de tipo motonêuronio superior para membros inferiores. O exame do líquido cefalorraquano (LCR) revelou alterações de tipo inflamatório. Radiografias da coluna cervical mostravam apenas aspecto irregular da apófise espinhosa de C6 com rarefação na base. Houve acentuada e rápida melhora do quadro neurológico e normalização do LCR na vigência de córticoterapia e, reagravamento da sintomatologia neurológica bem como das alterações no LCR após supressão da referida medicação. A biópsia do processo espinhoso da sexta vértebra cervical revelou tratar-se de granuloma eosinófilo. A evolução foi excelente após radioterapia local associada a corticosteróides. São discutidas as características do caso e daqueles referidos na literatura.

A case of eosinophilic granuloma of the cervical spine in a ten year-old white boy is reported. The patient complained of posterior cervical pain at the level of C5-C6 and right hemiparesis that progressed to mixed tetraparesis (lower motor neuron in upper limbs and upper motor neuron in lower limbs). Examination of the cerebrospinal fluid (CSF) disclosed an inflammatory reaction; radiologic studies of the cervical spine showed an irregular aspect with demineralization at the base of spinous process of C6. The patient was treatet intermitently with corticosteroids (prednisone); there was an improvement of neurologic symptoms and the examination of the CSF was normal within a few days, each time he took the drug. Neurologic symptons and abnormalities of the CSF recurred after discontinuation of corticotherapy. Examination of a fragment of the spinous process and neighboring tissues of C6, taken by biopsy, disclosed eosinophilic granuloma. Radiotherapy was then indicated, plus corticosteroids. There was remission of all neurologic symptoms and signs. The unusual features of the case are discussed in relation to others reported in the literature.

Granuloma eosinófilo da coluna cervical com manifestação neurológica, liquorica e radiológica atípica

Unusual neurological, cerebrospinal fluid and roentgenografic manifestations of eosinophilic granuloma of the cervical spine: a case report

Umbertina Conti Reed^I; Antonio Umberto Bresolin^I; Antonio Branco Lefèvre^II

^IDepartamento de Neuropsiquiatria da Faculdade de Medicina da Universidade de São Paulo: Médicos Residentes

^IIDepartamento de Neuropsiquiatria da Faculdade de Medicina da Universidade de São Paulo: Professor Adjunto

RESUMO

É relatado um caso de granuloma eosinófilo da coluna cervical em menino branco de 10 anos de idade que apresentava dor cervical na altura de C5-C6, hemiparesia direita que evoluiu ulteriormente para tetraparesia, mista, de tipo motonêuronio inferior para membros superiores e de tipo motonêuronio superior para membros inferiores. O exame do líquido cefalorraquano (LCR) revelou alterações de tipo inflamatório. Radiografias da coluna cervical mostravam apenas aspecto irregular da apófise espinhosa de C6 com rarefação na base. Houve acentuada e rápida melhora do quadro neurológico e normalização do LCR na vigência de córticoterapia e, reagravamento da sintomatologia neurológica bem como das alterações no LCR após supressão da referida medicação. A biópsia do processo espinhoso da sexta vértebra cervical revelou tratar-se de granuloma eosinófilo. A evolução foi excelente após radioterapia local associada a corticosteróides. São discutidas as características do caso e daqueles referidos na literatura.

SUMMARY

A case of eosinophilic granuloma of the cervical spine in a ten year-old white boy is reported. The patient complained of posterior cervical pain at the level of C5-C6 and right hemiparesis that progressed to mixed tetraparesis (lower motor neuron in upper limbs and upper motor neuron in lower limbs). Examination of the cerebrospinal fluid (CSF) disclosed an inflammatory reaction; radiologic studies of the cervical spine showed an irregular aspect with demineralization at the base of spinous process of C6. The patient was treatet intermitently with corticosteroids (prednisone); there was an improvement of neurologic symptoms and the examination of the CSF was normal within a few days, each time he took the drug. Neurologic symptons and abnormalities of the CSF recurred after discontinuation of corticotherapy. Examination of a fragment of the spinous process and neighboring tissues of C6, taken by biopsy, disclosed eosinophilic granuloma. Radiotherapy was then indicated, plus corticosteroids. There was remission of all neurologic symptoms and signs. The unusual features of the case are discussed in relation to others reported in the literature.

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1. ANSPACH, W. E. Xanthomatosis with involvement of a vertebral body. Amer. J. Dis. Child. 48:346, 1934.
2. AVERY, M. E.; MeAFEE, J. G. & GUILD, H. G. The course and prognosis of reticuloendotheliosis (eosinophilic granuloma, Schüller-Christian disease and Letterer-Siwe disease): a study of 40 cases. Amer. J. Med. 22:636, 1957.
3. VIOLI, L. V.; LASERSOHN, J. T. & LOPRESTI, J. T. Histiocytoais X (Schüller-Christian disease): a clinico-pathological survey, review of 10 patients and the results of prednisone therapy. Medicine (Baltimore). 42:119, 1963.
4. BAKER, A. B. & BAKER, L. H. Clinical Neurology. Harper & Row Publishers: Hangerstown, New York London, 1973, vol. 2, pag. 37-191.
5. BARNETT, A. B. & EINHORN, L. H. Pediatrics. Appleton-Century-Crofts, Educational Division Meredith Corporation, New York, 1972, vol. 5, pág. 1249.
6. BASS, M. H.; SAPIN, S. O. & HODES, H. L. Use of cortisone and corticotropin (ACTH) in treatment of reticuloendotheliosis in children. Amer. J. Dis. Child. 85:393, 1953.
7. BERAUD, C.; MANSUY, L. & PICARD, Y. D. Paraplègie par granulome éosinophile vertébral chez l'enfant. Ann. Chir. Infant. 7:147, 1966.
8. BLANKOFF, B. From eosinophilic granuloma to eosinophilic granuloma by the longest way around. Acta Orthop. Belg. 25:252, 1959.
9. COMPERE, E. L.; JOHNSON, W. E. & COVENTRY, M. B. Vertebra plana (Calve's disease) due to eosinophilic granuloma. J. Bone Joint Surg. 36A:969, 1954.
10. DAVIDSON, R. I. & SHILLITO, J. Eosinophilic granuloma of the cervical spine in children. Pediatrics 45:746, 1970.
11. DECOULX, P.; VANDENDORP, F.; SOULIER, A. E. & GODEFROY-VAUDEVILLE, L. Y. Granulome eosinophilique du rachis. J. Radiol. Electr. 35:90, 1954.
12. DUNDON, C. C.; WILLIAMS, H. A. & LAIPPLY, T. C. Eosinophilic granulome of bone. Radiology 47:433, 1946.
13. ELIAN, M.; BORNSTEIN, B.; MATZ, S.; ASKENASY, H. M. & SANDBANC, U. Neurological manifestation of general xanthomatosis: Hand-Schüller-Christian disease. Arch. Neurol. (Chicago) 21:115, 1969.
14. FAURÉ, C; MICHEL, J. & BUSSIERE, H. The Calve type of vertebra plana. Radiological and etiological study a propos of 19 cases. Ann. Radiol. 3:585, 1960.
15. FLOSI, A. Z.; OLIVEIRA, L. M.; INFANTE, O. & PEDALINI, L. Granuloma eosinófilo tratado pelo ACTH. Estudo clínico, histológico e radiodógico. Rev. Paul. Med. (São Paulo) 42:132, 1952.
16. FLOSI, A. Z.; ASSIS, L. M.; BLOISE, W.; COELHO NETTO, A. S. & ULHOA CINTRA, A. B. Treatment of eosinophilic granuloma by corticotropin: report of 4 cases with disappearance of bone lesions. J. Clin. Endocr. Metab. 17:994, 1957.
17. FLOSI, A. Z.; ASSIS, L. M.; COELHO NETTO, A. S.; BLOISE, W.; ULHOA CINTRA, A. B. & BARROS, R. P. Hormonal treatment of Hand-Schüller-Christian disease: report of a case with disappearance of the bone lesions. J. Clin. Endocr. Metab. 19:239, 1959.
18. GAVRAN, MC. H. H. & SPADY, H. A. Eosinophilic granuloma of bone. A study of 28 cases. J. Bone Jt. Surg. (Am. Edit.) 42 A: 979, 1960.
19. GIBSON, R. M. & EISEN, A. A. Eosinophilic granuloma of bone in two adjacent thoracic vertebrae: report of a case. J. Bone Joint Surg. (Br.) 45:566, 1963.
20. GLATT, M. A. Xanthoma or lipoid granuloma of temporal bone (Hand-Schüller-Christian syndrome) Arch. Otolaryng. 43:110, 1946.
21. GREEN, W. T. & FARBER, S. Eosinophilic or solitary granuloma of bone. J. Bone Jt. Surg. (Am. edit.) 24 A: 499, 1942.
22. HANSEN, P. B. The relationship of Hand-Schüller-Christian's disease, Letterer-Siwe's disease and eosinophilic granuloma of bone. Acta radiol. (Stockholm) 32:89, 1949.
23. HOWARD, R. C. Eosinophilic granuloma. J. Bone Joint Surg. 45 B: 624, 1963.
24. KARLEN, K. H. Case of the Hand-Schüller-Christian disease treated with cortisone. Acta Pediat. (Uppsala) 41:282, 1952.
25. KAYE, S. J. & FREIBERGER, R. H. Eosinophilic granuloma of the spine without vertebrae plana: a report of two unusual cases. Radiology 92:1188. 1969.
26. KIEFFER, S.; NESBIT, M. & D'ANGIO, G. Vertebrae plana due histiocytosis, X: serial studies. Acta Radiol. (Diagn.) 8:241, 1969.
27. LEVIN, H. Use of cortisone in the treatment of reticuloendotheliosis. J. Pediat. 46:531, 1955.
28. LIEBERMAN, P. H.; JONES, C. R.; DARGEON, H. W. K. & BEGG, C. F. A reapraisal of eosinophilic granuloma of bone, Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome. Medicine 48; 5:375, 1969.
29. LIEBESKIND, A.; JACOBSON, R.; ANDERSON, R. & SCHECHTER, M. M. Unusual neurological and roentgenografic manifestation of eosinophilic granuloma. Arch. Neurol. (Chicago) 28:131, 1973.
30. MACNAB, G. H. Discussion: eosinophilic granuloma, Letterer-Siwe disease, Hand-Schüller-Christian disease. Pediat. Soc. Med. (London) 48:718, 1955.
31. NYHOLM, K. K. Eosinophilic xantomat granulomatose og Letterer-Siwe's sygdom. Thesis. Kopenhagen, 1967.
32. NYHOLM, K. K. Eosinophilic xantomatous granulomatosis and Letterer-Siwe's disease. Acta Path. et Microbiol. Scand. section A, suplement 216, 1971.
33. OBERMAN, H. A. Idiopathic histiocytosis. A clinicopathologic study of 40 cases and review of the literature on eosinophilic granuloma of bone, HandSchüller-Christian disease and Letterer-Siwe disease. Pediatrics 28:307, 1967.
34. O'NEILL, J. F.; SKROMAK, S. J. & CASEY, P. R. Eosinophilic granuloma of ribs: review of literature and report of two cases with 4 and 6 1/2 years follow-up, respectively. J. Thorac. Surg. 29:528, 1955.
35. RITVO, M. Roentgen-dianosis of disease of the skull. An. Roentgenology 29: 183, 1949.
36. SBARBARO, S. J. & FRANCIS, K. C. Eosinophilic granuloma of bone. J. Amer. Med. Ass. 178:706, 1961.
37. SMITH, C. H. Hematologia Pediátrica. Salvat S. A. Editores, Barcelona, 1963, pag. 593.
38. SUNDELIUS, H. Zur Kenntnis der Lipoidosen speziell vom typus Schüller-Christian. Acta Med. Scand. 87:402, 1935-1936.
39. TAVERAS, J. M. & WOOD, E. H. Diagnostic Neuroradiology. The Willians & Wilkins, Baltimore, 1964, pág. 1143.
40. YABSLEY, R. H. & HARRIS, W. R. Solitary eosinophilic granuloma of a vertebral body causing paraplegia. J. Bone Joint Surg. 48 A: 1570, 1966.
41. Case records of the Massachstts General Hospital: weekly clinicopathological exercise, case 40342. New England J. Med. 251:354, 1954.

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. ANSPACH, W. E. Xanthomatosis with involvement of a vertebral body. Amer. J. Dis. Child. 48:346, 1934.

[2] 2. AVERY, M. E.; MeAFEE, J. G. & GUILD, H. G. The course and prognosis of reticuloendotheliosis (eosinophilic granuloma, Schüller-Christian disease and Letterer-Siwe disease): a study of 40 cases. Amer. J. Med. 22:636, 1957.

[3] 3. VIOLI, L. V.; LASERSOHN, J. T. & LOPRESTI, J. T. Histiocytoais X (Schüller-Christian disease): a clinico-pathological survey, review of 10 patients and the results of prednisone therapy. Medicine (Baltimore). 42:119, 1963.

[4] 4. BAKER, A. B. & BAKER, L. H. Clinical Neurology. Harper & Row Publishers: Hangerstown, New York London, 1973, vol. 2, pag. 37-191.

[5] 5. BARNETT, A. B. & EINHORN, L. H. Pediatrics. Appleton-Century-Crofts, Educational Division Meredith Corporation, New York, 1972, vol. 5, pág. 1249.

[6] 6. BASS, M. H.; SAPIN, S. O. & HODES, H. L. Use of cortisone and corticotropin (ACTH) in treatment of reticuloendotheliosis in children. Amer. J. Dis. Child. 85:393, 1953.

[7] 7. BERAUD, C.; MANSUY, L. & PICARD, Y. D. Paraplègie par granulome éosinophile vertébral chez l'enfant. Ann. Chir. Infant. 7:147, 1966.

[8] 8. BLANKOFF, B. From eosinophilic granuloma to eosinophilic granuloma by the longest way around. Acta Orthop. Belg. 25:252, 1959.

[9] 9. COMPERE, E. L.; JOHNSON, W. E. & COVENTRY, M. B. Vertebra plana (Calve's disease) due to eosinophilic granuloma. J. Bone Joint Surg. 36A:969, 1954.

[10] 10. DAVIDSON, R. I. & SHILLITO, J. Eosinophilic granuloma of the cervical spine in children. Pediatrics 45:746, 1970.

[11] 11. DECOULX, P.; VANDENDORP, F.; SOULIER, A. E. & GODEFROY-VAUDEVILLE, L. Y. Granulome eosinophilique du rachis. J. Radiol. Electr. 35:90, 1954.

[12] 12. DUNDON, C. C.; WILLIAMS, H. A. & LAIPPLY, T. C. Eosinophilic granulome of bone. Radiology 47:433, 1946.

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

[14] 14. FAURÉ, C; MICHEL, J. & BUSSIERE, H. The Calve type of vertebra plana. Radiological and etiological study a propos of 19 cases. Ann. Radiol. 3:585, 1960.

[15] 15. FLOSI, A. Z.; OLIVEIRA, L. M.; INFANTE, O. & PEDALINI, L. Granuloma eosinófilo tratado pelo ACTH. Estudo clínico, histológico e radiodógico. Rev. Paul. Med. (São Paulo) 42:132, 1952.

[16] 16. FLOSI, A. Z.; ASSIS, L. M.; BLOISE, W.; COELHO NETTO, A. S. & ULHOA CINTRA, A. B. Treatment of eosinophilic granuloma by corticotropin: report of 4 cases with disappearance of bone lesions. J. Clin. Endocr. Metab. 17:994, 1957.

[17] 17. FLOSI, A. Z.; ASSIS, L. M.; COELHO NETTO, A. S.; BLOISE, W.; ULHOA CINTRA, A. B. & BARROS, R. P. Hormonal treatment of Hand-Schüller-Christian disease: report of a case with disappearance of the bone lesions. J. Clin. Endocr. Metab. 19:239, 1959.

[18] 18. GAVRAN, MC. H. H. & SPADY, H. A. Eosinophilic granuloma of bone. A study of 28 cases. J. Bone Jt. Surg. (Am. Edit.) 42 A: 979, 1960.

[19] 19. GIBSON, R. M. & EISEN, A. A. Eosinophilic granuloma of bone in two adjacent thoracic vertebrae: report of a case. J. Bone Joint Surg. (Br.) 45:566, 1963.

[20] 20. GLATT, M. A. Xanthoma or lipoid granuloma of temporal bone (Hand-Schüller-Christian syndrome) Arch. Otolaryng. 43:110, 1946.

[21] 21. GREEN, W. T. & FARBER, S. Eosinophilic or solitary granuloma of bone. J. Bone Jt. Surg. (Am. edit.) 24 A: 499, 1942.

[22] 22. HANSEN, P. B. The relationship of Hand-Schüller-Christian's disease, Letterer-Siwe's disease and eosinophilic granuloma of bone. Acta radiol. (Stockholm) 32:89, 1949.

[23] 23. HOWARD, R. C. Eosinophilic granuloma. J. Bone Joint Surg. 45 B: 624, 1963.

[24] 24. KARLEN, K. H. Case of the Hand-Schüller-Christian disease treated with cortisone. Acta Pediat. (Uppsala) 41:282, 1952.

[25] 25. KAYE, S. J. & FREIBERGER, R. H. Eosinophilic granuloma of the spine without vertebrae plana: a report of two unusual cases. Radiology 92:1188. 1969.

[26] 26. KIEFFER, S.; NESBIT, M. & D'ANGIO, G. Vertebrae plana due histiocytosis, X: serial studies. Acta Radiol. (Diagn.) 8:241, 1969.

[27] 27. LEVIN, H. Use of cortisone in the treatment of reticuloendotheliosis. J. Pediat. 46:531, 1955.

[28] 28. LIEBERMAN, P. H.; JONES, C. R.; DARGEON, H. W. K. & BEGG, C. F. A reapraisal of eosinophilic granuloma of bone, Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome. Medicine 48; 5:375, 1969.

[29] 29. LIEBESKIND, A.; JACOBSON, R.; ANDERSON, R. & SCHECHTER, M. M. Unusual neurological and roentgenografic manifestation of eosinophilic granuloma. Arch. Neurol. (Chicago) 28:131, 1973.

[30] 30. MACNAB, G. H. Discussion: eosinophilic granuloma, Letterer-Siwe disease, Hand-Schüller-Christian disease. Pediat. Soc. Med. (London) 48:718, 1955.

[31] 31. NYHOLM, K. K. Eosinophilic xantomat granulomatose og Letterer-Siwe's sygdom. Thesis. Kopenhagen, 1967.

[32] 32. NYHOLM, K. K. Eosinophilic xantomatous granulomatosis and Letterer-Siwe's disease. Acta Path. et Microbiol. Scand. section A, suplement 216, 1971.

[33] 33. OBERMAN, H. A. Idiopathic histiocytosis. A clinicopathologic study of 40 cases and review of the literature on eosinophilic granuloma of bone, HandSchüller-Christian disease and Letterer-Siwe disease. Pediatrics 28:307, 1967.

[34] 34. O'NEILL, J. F.; SKROMAK, S. J. & CASEY, P. R. Eosinophilic granuloma of ribs: review of literature and report of two cases with 4 and 6 1/2 years follow-up, respectively. J. Thorac. Surg. 29:528, 1955.

[35] 35. RITVO, M. Roentgen-dianosis of disease of the skull. An. Roentgenology 29: 183, 1949.

[36] 36. SBARBARO, S. J. & FRANCIS, K. C. Eosinophilic granuloma of bone. J. Amer. Med. Ass. 178:706, 1961.

[37] 37. SMITH, C. H. Hematologia Pediátrica. Salvat S. A. Editores, Barcelona, 1963, pag. 593.

[38] 38. SUNDELIUS, H. Zur Kenntnis der Lipoidosen speziell vom typus Schüller-Christian. Acta Med. Scand. 87:402, 1935-1936.

[39] 39. TAVERAS, J. M. & WOOD, E. H. Diagnostic Neuroradiology. The Willians & Wilkins, Baltimore, 1964, pág. 1143.

[40] 40. YABSLEY, R. H. & HARRIS, W. R. Solitary eosinophilic granuloma of a vertebral body causing paraplegia. J. Bone Joint Surg. 48 A: 1570, 1966.

[41] 41. Case records of the Massachstts General Hospital: weekly clinicopathological exercise, case 40342. New England J. Med. 251:354, 1954.