Mielinolise pontina central e extra-pontinas: apresentação de um caso autopsiado e revisão da literatura

Pittella, José Eymard Homem; Gobbi, Helenice

doi:10.1590/S0004-282X1987000300011

Resumos

Relata-se um caso autopsiado de mielinólise pontina central e extra-pontina em paciente diabética de 16 anos, desidratada, que recebeu grande quantidade de solução salina isotônica intravenosa, diariamente, a partir do primeiro dia da internação hospitalar. Nos três primeiros dias houve aumento em mais de 30 mEq/1 do sódio plasmático em relação ao valor inicial. Após ligeira queda nos dias subsequentes, o sódio novamente aumentou e persistiu com níveis acima do normal por mais 12 dias. No sexto dia desta nova e persistente elevação do sódio surgiram manifestações neurológicas progressivas caracterizadas por mutismo, impossibilidade de movimentação da cabeça, tronco e membros, recusa de dieta oral e dificuldade de eliminar as secreções das vias aéreas respiratórias, que permaneceram até o óbito. O exame neuropatológico, além da mielinólise pontina central maciça, revelou lesões desmielinizantes focais múltiplas, com quadro histopatológico semelhante ao da ponte, na substância branca subcortical do lobo temporal esquerdo, trato óptico direito, cápsulas externa e extrema à direita, fascículo mamilar principal e substância branca subcortical do hemisfério cerebelar esquerdo. Faz-se revisão dos casos de mielinólise extra-pontina registrados na literatura, o presente caso representando a 30(9). Comenta-se o quadro clínico e a etiopatogênese da mielinólise pontina central e extra-pontina, destacando-se como um dos prováveis fatores causais, além da correção rápida e persistente do sódio plasmático, a oscilação dos níveis deste eletrólito em pacientes com hiponatremia e/ou desidratação.

An autopsied case of central pontine and extrapontine myelinolysis in a 16-year-old diabetic girl is described. Due to dehydration she was treated vigorously with daily intravenous isotonic saline, from the first day of hospitalization. In the first three days the serum sodium level increased by more than 30 mEq/1 when compared with the initial level. By the next days the serum sodium level, after a mild drop, rose again and was maintained above normal range for a further 12 days. On the sixth day of this new and sustained serum sodium increase, the patient presented progressive neurological manifestations that remained until her death, characterized by mutism, inability to eat, to move her head, trunk, and members and, in addition, retention of respiratory secretions. The neuropathological examination showed massive central pontine myelinolysis and similar myelinolytic lesions in the subcortical white matter of the temporal lobe, the right optic tract, the external and extreme capsules to the right, the main mammillary tract and the subcortical white matter of the left cerebellar hemisphere. The review of the literature on central pontine and extrapontine myelinolysis shows that the present case is the 30th of such condition. The clinical picture and the etiopathogenesis of central pontine and extrapontine myelinolysis are commented upon. It is suggested, as possible causative factors, the persistent and rapid correction of serum sodium concentration as well as its flutuation in patients with hyponatremia and/or dehydration.

Mielinolise pontina central e extra-pontinas: apresentação de um caso autopsiado e revisão da literatura

Central pontine and extrapontine myelinolysis: report of an autopsied case and review of the literature

José Eymard Homem Pittella; Helenice Gobbi

Laboratório de Neuropatologia, Departamento de Anatomia Patológica e Medicina Legal, Faculdade de Medicina da Universidade Federal de Minas Gerais, Belo Horizonte

RESUMO

Relata-se um caso autopsiado de mielinólise pontina central e extra-pontina em paciente diabética de 16 anos, desidratada, que recebeu grande quantidade de solução salina isotônica intravenosa, diariamente, a partir do primeiro dia da internação hospitalar. Nos três primeiros dias houve aumento em mais de 30 mEq/1 do sódio plasmático em relação ao valor inicial. Após ligeira queda nos dias subsequentes, o sódio novamente aumentou e persistiu com níveis acima do normal por mais 12 dias. No sexto dia desta nova e persistente elevação do sódio surgiram manifestações neurológicas progressivas caracterizadas por mutismo, impossibilidade de movimentação da cabeça, tronco e membros, recusa de dieta oral e dificuldade de eliminar as secreções das vias aéreas respiratórias, que permaneceram até o óbito. O exame neuropatológico, além da mielinólise pontina central maciça, revelou lesões desmielinizantes focais múltiplas, com quadro histopatológico semelhante ao da ponte, na substância branca subcortical do lobo temporal esquerdo, trato óptico direito, cápsulas externa e extrema à direita, fascículo mamilar principal e substância branca subcortical do hemisfério cerebelar esquerdo. Faz-se revisão dos casos de mielinólise extra-pontina registrados na literatura, o presente caso representando a 30⁹. Comenta-se o quadro clínico e a etiopatogênese da mielinólise pontina central e extra-pontina, destacando-se como um dos prováveis fatores causais, além da correção rápida e persistente do sódio plasmático, a oscilação dos níveis deste eletrólito em pacientes com hiponatremia e/ou desidratação.

SUMMARY

An autopsied case of central pontine and extrapontine myelinolysis in a 16-year-old diabetic girl is described. Due to dehydration she was treated vigorously with daily intravenous isotonic saline, from the first day of hospitalization. In the first three days the serum sodium level increased by more than 30 mEq/1 when compared with the initial level. By the next days the serum sodium level, after a mild drop, rose again and was maintained above normal range for a further 12 days. On the sixth day of this new and sustained serum sodium increase, the patient presented progressive neurological manifestations that remained until her death, characterized by mutism, inability to eat, to move her head, trunk, and members and, in addition, retention of respiratory secretions. The neuropathological examination showed massive central pontine myelinolysis and similar myelinolytic lesions in the subcortical white matter of the temporal lobe, the right optic tract, the external and extreme capsules to the right, the main mammillary tract and the subcortical white matter of the left cerebellar hemisphere. The review of the literature on central pontine and extrapontine myelinolysis shows that the present case is the 30th of such condition. The clinical picture and the etiopathogenesis of central pontine and extrapontine myelinolysis are commented upon. It is suggested, as possible causative factors, the persistent and rapid correction of serum sodium concentration as well as its flutuation in patients with hyponatremia and/or dehydration.

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Departamento de Anatomia Patológica e Medici/na Legal, Faculdade de Medicina da TJFMG - Av. Alfredo Balena, 190 - 30130 - Belo Horizonte, MG - Brasil.

1. ADAMS, J.H. - Central pontine myelinolysis. In H. Jacob (ed.): Proceedings of the 4th International Congress of Neuropathology. Thieme, Stuttgart, vol. 3:303, 1962.
2. ADAMS, R.D.; VICTOR, M. & MANCALL, E.L. - Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. Arch. Neurol. Psychiat. 81:154, 1959.
3. ALLSOP, J. & TURNER, B. - Cerebellar degeneration associated with chronic alcoholism. J. neurol. Sci. 3:238, 1966.
4. BERRY, K. & OLSZEWSKI, J. - Central pontine myelinolysis: a case report. Neurology 13:531, 1963.
5. BURCAR, P.J.; NORENBERG, M.D. & YARNELL, P.R. - Hyponatremia and central pontine myelinolysis. Neurology 27:223, 1977.
6. CAMBIER, J.; MASSON, M.; DAIROU, R.; GRAY, F.; HENIN, D. & LAURENT, D. - Myélinolyse centro-pontine associée à des lésions plus diffuses de la substance blanche, troubles hydroélectrolytiques en relation avec un traitement diurétique. Rev. neurol. (Paris) 133:89, 1977.
7. CHASON, J.L.; LANDERS, J.W. & GONZALEZ, J.E. - Central pontine myelinolysis. J. Neurol. Neurosurg. Psychiat. 27:317, 1964.
8. CHIANG, T.Y.; ORDINARIO, A.T. & BARRON, K.D. - Central pontine myelinolysis. Proceedings of the Weekly Seminar in Neurology, Hines V.A. Hospital, suppl. 1:36, 1967.
9. CONGER, J.D.; McINTYRE, J.A. & JACOBY, W.J., Jr. - Central pontine myelinolysis associated with inappropriate antidiuretic hormone secretion. Amer. J. Med. 47:813, 1969.
10. ENDO, Y.; ODA, M. & HARA, M. - Central pontine myelinolysis: a study of 37 cases in 1000 consecutive autopsies. Acta neuropath. 53:145, 1981.
11. FINLAYSON, M.H.; SNIDER, S.; OLIVA, L.A. & GAULT, M.H. - Cerebral and pontine myelinolysis: two cases with fluid and electrolyte imbalance and hypotension. J. neurol. Sci. 18:399, 1973.
12. FORNO, L.S. & RIVERA, L. - Central pontine myelinolysis. J. Neuropath, exp. Neurol. 34:77, 1975.
13. GOEBEL, H.H. & HERMAN-BEN ZUR, P. - Central pontine myelinolysis. In P.J. Vinken & G.W. Bruyn (eds.): Handbook of Clinical Neurology. North Holland, Amsterdam, vol. 28, pâg. 285, 1976.
14. GOLDMAN, J.E. & HOROUPIAN, D.S. - Demyelination of the lateral geniculate nucleus in central pontine myelinolysis. Ann. Neurol. 9:185, 1981.
15. GOULON, M.; BABINET, P.; RAPHAËL, J.C.; GROSBUIS & GAJDOS, Ph. - Les manifestations neurologiques des hyponatrémies. Rev. neurol. (Paris) 125:219, 1971.
16. HO, K-C; HODACH, R.; VARMA, R. & THORSTEISON, V. - Kernicterus and central pontine myelonolysis in a 14-year-old boy with fulminating viral hepatitis. Ann. Neurol. 8:633, 1980.
17. IBAYASHI, S.; OGATA, J.; KATSUHIKO, A.; SADOSHIMA, S.; UEDA, K.; FUJISHIMA, M.; OMAE, T.; SAWA, H. & TATEISHI, J. - Central pontine and extrapontine myelinolysis following rapid correction of hyponatremia: report of an autopsy case. No To Shinkei 36:575, 1984.
18. KALNINS, R.M.; BERKOVIC, S.F. & BLADIN, P.F. - Central pontine myelinolysis with widespread extrapontine lesions: a report of two cases. Clin. exp. Neurol. 20:189, 1984.
19. KEPES, J.J.; REECE, C.A. & OXLEY, D..K. - Central pontine myelinolysis in a 7-year_old boy. J. Neurol. Neurosurg. Psychiat. 28:39, 1965.
20. KLAV1NS, J.V. - Central pontine myelinolysis. J. Neuropath, exp. Neurol. 22:302, 1963.
21. KLEINSCHMIDT-DeMASTERS, B.K. & NORENBERG, M.D. - Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis. Science 211:1068, 1981.
22. LANDERS, J.W. ; CHASON, J.L. & SAMUEL, V.N. - Central pontine myelinolysis: a pathogenetic hypothesis. Neurology 15:968, 1965.
23. LAURENO, R. - Central pontine myelinolysis following rapid correction of hyponatremia. Ann. Neurol. 13:232, 1983.
24. MATHEWS, T. & MOOSSY, J. - Central pontine myelinolysis: lesion evolution and pathogenesis. J. Neuropath, exp. Neurol. 34:77, 1975.
25. MATHIESON, G. & OLSZEWSKI, J. - Central pontine myelinolysis with other cerebral changes: a report of two cases. Neurology 10:345, 1960.
26. MATSUOKA, T.; MIYOSHI, K. ; SAKA, K.; HAYASHI, S. & KAGEYAMA, N. Central pontine myelinolysis: a report of three cases. Acta neuropath. 5:117, 1965.
27. McCORMICK, W.F. & DANNEEL, CF. - Central pontine myelinolysis. Arch. int. Med. 119:444, 1967.
28. MONSEU, G. & FLAMENT-DURAND, J. - Pathogenesis of central pontine myelinolysis: a clinical and pathological description of three cases. Path. Europ. 6:75, 1971.
29. MONTEIRO, L. - La myélinolyse du centre du pont dans le cadre d'un nouveau syndrome histopathologique de topographie systématisée: à propos d'un cas anatomo-clinique. J. neurol. Sci. 13:293, 1971.
30. NARINS, R.G. - Therapy of hyponatremia: does haste make waste? N. Engl. J. Med. 314:1573, 1986.
31. NORENBERG, M.D.; LESLIE, K.O. & ROBERTSON, A.S. - Association between rise in serum sodium and central pontine myelinolysis. Ann. Neurol. 11:128, 1982.
32. NORENBERG, M.D. & PAPENDICK, R.E. - Chronicity of hyponatremia as a factor in experimental myelinolysis. Ann. Neurol. 15:544, 1984.
33. OKEDA, R. - Centrale pontine Myelinolyse: pathogenetische Aspekte aufgrund morphometrischer Untersuchungen des Briickenfusses. Acta neuropath. 27:233, 1974.
34. POWERS, J.M. & McKEEVER, P.E. - Central pontine myelinolysis: an ultrastructural and elemental study. J. neurol. Sci. 29:65, 1976.
35. ROSMAN, N.P.; KAKULAS, B.A. & RICHARDSON, E.P., Jr. - Central pontine myelinolysis in a child with leukemia. Arch. Neurol. 14:273, 1966.
36. SCHNECK, S.A. - Neuropathological features of human organ transplantation: II. Central pontine myelinolysis and neuroaxonal dystrophy. J. Neuropath, exp. Neurol. 25:18, 1966.
37. SHERINS, R.J. & VERITY, M.A. - Central pontine myelinolysis associated with acute haemorrhagic pancreatitis. J. Neurol. Neurosurg. Psychiat. 31:583, 1968.
38. STERNS, R.H.; RIGGS, J.E. & SCHCHET, S.S., Jr. - Osmotic demyelination syndrome following correction of hyponatremia. N. Engl. J. Med. 314:1535, 1986.
39. TOMLINSON, B.E.; PIERIDES, A.M. & BRADLEY, W.G. - Central pontine myelinolysis; two cases with associated electrolyte disturbance. Quart. J. Med. 45:373, 1976.
40. TYLER, H.R. - Neurological complications of dialysis, transplantation, and other forms of treatment in chronic uremia. Neurology 15:1081, 1965.
41. WAXMAN, S.G. - Membranes, myelin, and the pathophysiology of multiple sclerosis. N. Engl. J. Med. 306:1529, 1982.
42. WILD, E. - Contribution a l'anatomo-clinique de la my61inolyse du pont. Arch, suiss. Neurol. Neurochir. Psychiat. 112:271, 1973.
43. WRIGHT, D.G.; LAURENO, R. & VICTOR, M. - Pontine and extrapontine myelinolysis. Brain 102:361, 1979.
44. WRIGHT, J.R., Jr.; YATES, A. J.; SHARMA, H.M. & THIBERT, P. - Central pontine myelinolysis following saline treatment of a diabetic rat for dehydration. J. comp. Path. 93:509, 1983.
45. ZWICK, D.; FRIEDE, R.L. & ROESSMAN, U. - Central spinal myelinolysis. Neurology 35:891, 1985.

Datas de Publicação

Publicação nesta coleção
22 Jun 2011
Data do Fascículo
Set 1987

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

[1] 1. ADAMS, J.H. - Central pontine myelinolysis. In H. Jacob (ed.): Proceedings of the 4th International Congress of Neuropathology. Thieme, Stuttgart, vol. 3:303, 1962.

[2] 2. ADAMS, R.D.; VICTOR, M. & MANCALL, E.L. - Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. Arch. Neurol. Psychiat. 81:154, 1959.

[3] 3. ALLSOP, J. & TURNER, B. - Cerebellar degeneration associated with chronic alcoholism. J. neurol. Sci. 3:238, 1966.

[4] 4. BERRY, K. & OLSZEWSKI, J. - Central pontine myelinolysis: a case report. Neurology 13:531, 1963.

[5] 5. BURCAR, P.J.; NORENBERG, M.D. & YARNELL, P.R. - Hyponatremia and central pontine myelinolysis. Neurology 27:223, 1977.

[6] 6. CAMBIER, J.; MASSON, M.; DAIROU, R.; GRAY, F.; HENIN, D. & LAURENT, D. - Myélinolyse centro-pontine associée à des lésions plus diffuses de la substance blanche, troubles hydroélectrolytiques en relation avec un traitement diurétique. Rev. neurol. (Paris) 133:89, 1977.

[7] 7. CHASON, J.L.; LANDERS, J.W. & GONZALEZ, J.E. - Central pontine myelinolysis. J. Neurol. Neurosurg. Psychiat. 27:317, 1964.

[8] 8. CHIANG, T.Y.; ORDINARIO, A.T. & BARRON, K.D. - Central pontine myelinolysis. Proceedings of the Weekly Seminar in Neurology, Hines V.A. Hospital, suppl. 1:36, 1967.

[9] 9. CONGER, J.D.; McINTYRE, J.A. & JACOBY, W.J., Jr. - Central pontine myelinolysis associated with inappropriate antidiuretic hormone secretion. Amer. J. Med. 47:813, 1969.

[10] 10. ENDO, Y.; ODA, M. & HARA, M. - Central pontine myelinolysis: a study of 37 cases in 1000 consecutive autopsies. Acta neuropath. 53:145, 1981.

[11] 11. FINLAYSON, M.H.; SNIDER, S.; OLIVA, L.A. & GAULT, M.H. - Cerebral and pontine myelinolysis: two cases with fluid and electrolyte imbalance and hypotension. J. neurol. Sci. 18:399, 1973.

[12] 12. FORNO, L.S. & RIVERA, L. - Central pontine myelinolysis. J. Neuropath, exp. Neurol. 34:77, 1975.

[13] 13. GOEBEL, H.H. & HERMAN-BEN ZUR, P. - Central pontine myelinolysis. In P.J. Vinken & G.W. Bruyn (eds.): Handbook of Clinical Neurology. North Holland, Amsterdam, vol. 28, pâg. 285, 1976.

[14] 14. GOLDMAN, J.E. & HOROUPIAN, D.S. - Demyelination of the lateral geniculate nucleus in central pontine myelinolysis. Ann. Neurol. 9:185, 1981.

[15] 15. GOULON, M.; BABINET, P.; RAPHAËL, J.C.; GROSBUIS & GAJDOS, Ph. - Les manifestations neurologiques des hyponatrémies. Rev. neurol. (Paris) 125:219, 1971.

[16] 16. HO, K-C; HODACH, R.; VARMA, R. & THORSTEISON, V. - Kernicterus and central pontine myelonolysis in a 14-year-old boy with fulminating viral hepatitis. Ann. Neurol. 8:633, 1980.

[17] 17. IBAYASHI, S.; OGATA, J.; KATSUHIKO, A.; SADOSHIMA, S.; UEDA, K.; FUJISHIMA, M.; OMAE, T.; SAWA, H. & TATEISHI, J. - Central pontine and extrapontine myelinolysis following rapid correction of hyponatremia: report of an autopsy case. No To Shinkei 36:575, 1984.

[18] 18. KALNINS, R.M.; BERKOVIC, S.F. & BLADIN, P.F. - Central pontine myelinolysis with widespread extrapontine lesions: a report of two cases. Clin. exp. Neurol. 20:189, 1984.

[19] 19. KEPES, J.J.; REECE, C.A. & OXLEY, D..K. - Central pontine myelinolysis in a 7-year_old boy. J. Neurol. Neurosurg. Psychiat. 28:39, 1965.

[20] 20. KLAV1NS, J.V. - Central pontine myelinolysis. J. Neuropath, exp. Neurol. 22:302, 1963.

[21] 21. KLEINSCHMIDT-DeMASTERS, B.K. & NORENBERG, M.D. - Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis. Science 211:1068, 1981.

[22] 22. LANDERS, J.W. ; CHASON, J.L. & SAMUEL, V.N. - Central pontine myelinolysis: a pathogenetic hypothesis. Neurology 15:968, 1965.

[23] 23. LAURENO, R. - Central pontine myelinolysis following rapid correction of hyponatremia. Ann. Neurol. 13:232, 1983.

[24] 24. MATHEWS, T. & MOOSSY, J. - Central pontine myelinolysis: lesion evolution and pathogenesis. J. Neuropath, exp. Neurol. 34:77, 1975.

[25] 25. MATHIESON, G. & OLSZEWSKI, J. - Central pontine myelinolysis with other cerebral changes: a report of two cases. Neurology 10:345, 1960.

[26] 26. MATSUOKA, T.; MIYOSHI, K. ; SAKA, K.; HAYASHI, S. & KAGEYAMA, N. Central pontine myelinolysis: a report of three cases. Acta neuropath. 5:117, 1965.

[27] 27. McCORMICK, W.F. & DANNEEL, CF. - Central pontine myelinolysis. Arch. int. Med. 119:444, 1967.

[28] 28. MONSEU, G. & FLAMENT-DURAND, J. - Pathogenesis of central pontine myelinolysis: a clinical and pathological description of three cases. Path. Europ. 6:75, 1971.

[29] 29. MONTEIRO, L. - La myélinolyse du centre du pont dans le cadre d'un nouveau syndrome histopathologique de topographie systématisée: à propos d'un cas anatomo-clinique. J. neurol. Sci. 13:293, 1971.

[30] 30. NARINS, R.G. - Therapy of hyponatremia: does haste make waste? N. Engl. J. Med. 314:1573, 1986.

[31] 31. NORENBERG, M.D.; LESLIE, K.O. & ROBERTSON, A.S. - Association between rise in serum sodium and central pontine myelinolysis. Ann. Neurol. 11:128, 1982.

[32] 32. NORENBERG, M.D. & PAPENDICK, R.E. - Chronicity of hyponatremia as a factor in experimental myelinolysis. Ann. Neurol. 15:544, 1984.

[33] 33. OKEDA, R. - Centrale pontine Myelinolyse: pathogenetische Aspekte aufgrund morphometrischer Untersuchungen des Briickenfusses. Acta neuropath. 27:233, 1974.

[34] 34. POWERS, J.M. & McKEEVER, P.E. - Central pontine myelinolysis: an ultrastructural and elemental study. J. neurol. Sci. 29:65, 1976.

[35] 35. ROSMAN, N.P.; KAKULAS, B.A. & RICHARDSON, E.P., Jr. - Central pontine myelinolysis in a child with leukemia. Arch. Neurol. 14:273, 1966.

[36] 36. SCHNECK, S.A. - Neuropathological features of human organ transplantation: II. Central pontine myelinolysis and neuroaxonal dystrophy. J. Neuropath, exp. Neurol. 25:18, 1966.

[37] 37. SHERINS, R.J. & VERITY, M.A. - Central pontine myelinolysis associated with acute haemorrhagic pancreatitis. J. Neurol. Neurosurg. Psychiat. 31:583, 1968.

[38] 38. STERNS, R.H.; RIGGS, J.E. & SCHCHET, S.S., Jr. - Osmotic demyelination syndrome following correction of hyponatremia. N. Engl. J. Med. 314:1535, 1986.

[39] 39. TOMLINSON, B.E.; PIERIDES, A.M. & BRADLEY, W.G. - Central pontine myelinolysis; two cases with associated electrolyte disturbance. Quart. J. Med. 45:373, 1976.

[40] 40. TYLER, H.R. - Neurological complications of dialysis, transplantation, and other forms of treatment in chronic uremia. Neurology 15:1081, 1965.

[41] 41. WAXMAN, S.G. - Membranes, myelin, and the pathophysiology of multiple sclerosis. N. Engl. J. Med. 306:1529, 1982.

[42] 42. WILD, E. - Contribution a l'anatomo-clinique de la my61inolyse du pont. Arch, suiss. Neurol. Neurochir. Psychiat. 112:271, 1973.

[43] 43. WRIGHT, D.G.; LAURENO, R. & VICTOR, M. - Pontine and extrapontine myelinolysis. Brain 102:361, 1979.

[44] 44. WRIGHT, J.R., Jr.; YATES, A. J.; SHARMA, H.M. & THIBERT, P. - Central pontine myelinolysis following saline treatment of a diabetic rat for dehydration. J. comp. Path. 93:509, 1983.

[45] 45. ZWICK, D.; FRIEDE, R.L. & ROESSMAN, U. - Central spinal myelinolysis. Neurology 35:891, 1985.