Weber's syndrome with recovery ct demonstration of an end-zone infarction in the territory of the mesencephalic artery

Oliveira-Souza, R.; Gusmão, D. L.

doi:10.1590/S0004-282X1991000100014

Abstracts

Weber's syndrome is one of the classically described brainstem syndromes. The mesencephalic artery and the syndromes resulting from occlusion of its branches have been attracting increasing interest in the past few years. We present here a case of Weber's syndrome emphasizing that (1) it is one of the major syndromes deriving from infarction in the territory of the mesencephalic artery; (2) that at least two clinical patterns of Weber's syndrome may be distinguished on the basis of the presence or lack of abnormal somnolence, mental confusion, and abulia; and (3) that each one of these patterns seems to be correlated with damage to distinct zones within the general territory of the mesencephalic artery.

A síndrome de Weber representa uma das síndromes clássicas do tronco cerebral. A artéria mesencefálica e as síndromes que resultam da oclusão dos seus ramos têm despertado interesse crescente nos últimos anos. Apresentamos um caso de síndrome de Weber, enfatizando que (1) é uma das principais síndromes decorrentes de infarto no território da artéria mesencefálica; (2) dois padrões clínicos, pelo menos, podem ser distinguidos da síndrome de Weber, com base na presença ou não de sonolência anormal, confusão mental e abulia; e (3) cada um desses padrões parece se correlacionar com lesão de zonas diferentes contidas no território geral da artéria mesencefálica.

CONTENTS CONTEÚDO

R. Oliveira-Souza; D. L. Gusmão

Neurology Service (Prof. H. Alvarenga), Gaffrée e Guinle Hospital, University of Rio de Janeiro (UNI-RIO).

SUMMARY

Weber's syndrome is one of the classically described brainstem syndromes. The mesencephalic artery and the syndromes resulting from occlusion of its branches have been attracting increasing interest in the past few years. We present here a case of Weber's syndrome emphasizing that (1) it is one of the major syndromes deriving from infarction in the territory of the mesencephalic artery; (2) that at least two clinical patterns of Weber's syndrome may be distinguished on the basis of the presence or lack of abnormal somnolence, mental confusion, and abulia; and (3) that each one of these patterns seems to be correlated with damage to distinct zones within the general territory of the mesencephalic artery.

RESUMO

A síndrome de Weber representa uma das síndromes clássicas do tronco cerebral. A artéria mesencefálica e as síndromes que resultam da oclusão dos seus ramos têm despertado interesse crescente nos últimos anos. Apresentamos um caso de síndrome de Weber, enfatizando que (1) é uma das principais síndromes decorrentes de infarto no território da artéria mesencefálica; (2) dois padrões clínicos, pelo menos, podem ser distinguidos da síndrome de Weber, com base na presença ou não de sonolência anormal, confusão mental e abulia; e (3) cada um desses padrões parece se correlacionar com lesão de zonas diferentes contidas no território geral da artéria mesencefálica.

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Acknowledgements - The authors are greatly indebted to Dr. P.W. Nathan (National Hospital for Nervous Diseases, Queen Square, London) whom so kindly reviewed, criticized and enriched the manuscripit. Dr. José Solon dde Mello performed and interpreted the EEGs (Hospital Dr. Philippe Pinel, Rio de Janeiro). We are also grateful to the whole staff of the Library of the Oswaldo Cruz Institute, Rio de Janeiro, for their invaluable help in the bibliographical searches.

REFERENCES

1. Adams RD, Victor M. Principles of Neurology. Ed 4. New York: McGraw-Hill, 1989, p 633.

2. Allen CMC. Predicting recovery after acute stroke. Br J Hosp Med 1984, 31:428-434.

3. Andrews BT, Chiles BW, Olsen WL, Pitts LH. The effect of intracerebral hematoma location on the risk of brain-stem compression and on clinical outcome. J Neurosurg 1988 69:518-522.

4. Astrup J, Siesjö BK, Simon L. Thresholds in cerebral ischemia: the ischemic penumbra. Stroke 1981, 12:723-724.

5. Bogousslavsky J, Regli F. Atteinte intra-axiale du nerf moteur oculaire commun dans les infarctus mésencéphaliques. Rev Neurol 1984, 140:263-270.

6. Caplan DR. «Top of the basilar» syndrome. Neurology 1979, 30:72-79.

7. Castaigne P, Lhermitte F, Buge A, Escourolle R, Hauw JJ, Lyon-Caen O. Paramedian thalamic and midbrain infarcts: clinical and neuropathological study. Ann Neurol 1981, 10:127-148.

8. Cestan R, Gourgeois M. Syndrome de Weber avec autopsie. Rev Neurol 1900, 8:428-431.

9. Charcot JM. Sur un cas d'hystérie simulatrice du syndrome de Weber. In Clinique des Maladies du système Nerveux, T. 1. Paris: Veuve Babé et Cie, 1892, p 308-332.

10. Cushing H, Eisenhardt L. Meningiomas. Springfield: Charles C. Thomas, 1938, p 393.

11. Dehaene I, Dom R. A mesencephalic locked-in syndrome. J Neurol 1982, 227:255-259. 12l Dick JPR, Guiloff RJ. Stewart A, Blackstock J, Bielawska C, Paul EA, Marsden CD.

Mini-mental state examination in neurological patients. J Neurol Neurosurg Psychiat 1984, 47:496-499.

13. Dumont PAS, Laluci DH, Araújo MG. Thalamo-mesencephalic infarction after arterial catheterism (in Portuguese). Rev Méd IAMPSE 1987, 18:88-96.

14. Emura A, Takeuchi A, Hashimoto T, Inaba G, Akaoka I, Miyashita H. A case of Behcet's disease with Weber's syndrome. J Rheumatol 1986, 13:459-461.

15. Feeney DM, Baron JC. Diaschisis. Stroke 1986, 17:817-830.

16. Fisher CM. Abulia minor vs. (agitated behavior. Clin Neurosurg 1984, 31:9-31.

17. Fisher M, Smith TW, Jacobs R. Pure motor hemiplegia secondary to a saccular basilar artery aneurysm. Stroke 1982, 19:104-107.

18. Foix C, Hillemand F. Les artères de l'axe encéphalique jusq'au diencéphale inclusivement. Rev Neurol 1925, 6:705-739.

19. Friedman JH. Syndrome of diffuse encephalopathy due to nondominant thalamic infarction. Neurology 1985, 35:1524-1526.

20. Gillilan LA. The correlation of the blood supply to the human brain stem with clinical brain stem syndromes. J Neuropath Exp Neurol 1964, 23 :78-108.

21. Hinshaw DB, Thompson JR, Hasso AN, Casselman ES. Infarctions of the brainstem and cerebellum: a correlation of computed tomography and angiography. Radiology 1980, 137:105-112.

22. Ho KL. Pure motor hemiplegia due to infarction of the cerebral peduncle. Arch Neurol 1982, 39:524-526.

23. Jurgensen JC, Towfighi J, Brennan RW, Jeffreys WH. Symmetric brainstem necrosis in an adult following hypotension: an arterial end-zone infarct? Stroke 1983, 14:967-970.

24. Kaplan HA. Arteries of the brain : an anatomic study. Acta Radiol (Stock) 1956, 46: 364-370.

25. Kaplan HA. Collateral circulation of the brain. Neurology 1961, 11:9-15.

26. Karp JS, Hurtig HI. «Locked-in state» with bilateral midbrain infarcts. Arch Neurol 1974, 30:176-178.

27. Katz DI, Alexander MP, Mandell AM. Dementia following strokes in the mesencephalon and diencephalon. Arch Neurol 1987, 44 :1127-1133.

28. Koroshetz WJ, Ropper AJ. Artery-to-artery embolism causing stroke in the posterior circulation. Neurology 1987, 37 : 292-296.

29. Lassen NA, Olsen TS, Hojgaard K, Skriver E. Incomplete infarction: a CT-negative irreversible ischemic brain lesion. J Cer Blood Flow Metab 1983, 3(S1) :S602-603.

30. Lazorthes G, Poulhes, J, Bastide G, Roulleau J. Les territoires artériels du tronc cérébral: recherches anatomiques et syndromes vasculaires. Presse Méd 1958, 91 : 2048-2051.

31. Mehler MF. Reversible rostral basilar artery syndrome. Arch Intern Med 1988, 148: 166-169.

32. Mohr JP. Distal field infarction. Neurology 1969. 19:279.

33. Nathan PW, Smith MC. Long descending tracts in man: I. Review of present knowledge. Brain 1955, 78:248-303.

34. Oliveira-Souza R. Motor hemiplegia and the cerebral organization of movement in man: II. The myth of the human extrapyramidal system. Arq Neuro-Psiquiat (São Paulo) 1989, 47:16-27.

35. Olsen TS, Bruhn P, öberg RGE. Cortical hypoperfusion as a possible cause of «subcortical aphasia». Brain 1986, 109:393-410.

36. Percheron G. Les artères du thalamus humain: II. Artères et territoires thalamiques paramédians de l'artère basilaire communicante. Rev Neurol 1976, 132:309-324.

37. Ross ED. Localization of the pyramidal tract in the internal capsule by whole brain dissection. Neurology 1980, 30:59-64.

38. Rowley HA. Lowenstein DH, Rowbotham MC, Simon RP. Thalamomesencephalic strokes after cocaine abuse. Neurology 1989, 39:428-430.

39. Segarra JM. Cerebral vascular disease and behavior: I. The syndrome of the mesencephalic artery (basilar artery bifurcation). Arch Neurol 1970, 22:408-418.

40. Sieben G, de Reuck J, Vander Eeken H. Thrombosis of the mesencephalic artery. Acta Neurol Belg 1977, 77:151-162.

41. Souques A. Double syndrome de Weber. Rev Neurol 1900, 8:148-149.

42. Stiller J, Shanzer S, Yang W. Brainstem lesion with pure motor hemiparesis: computed tomographic demonstration. Arch Neurol 1982, 39 :660-661.

43. Vander Eecken H, Adams RD. The anatomy and functional significance of the meningeal arterial anastomoses of the human brain. J Neurophath Exp Neurol 1953, 12:132-157.

44. Verhaart WJC. The pes pedunculi and pyramid. J Comp Neurol 1948, 88:139-155.

45. Weber H. A contribution to the pathology of the crura cerebri. Med Chir Trans 1863, 28:121-189.

Dr. Ricardo de Oliveira-Souza - Rua General Belford 226 - 20961 - Rio de Janeiro RJ - Brazil.

1. Adams RD, Victor M. Principles of Neurology. Ed 4. New York: McGraw-Hill, 1989, p 633.
2. Allen CMC. Predicting recovery after acute stroke. Br J Hosp Med 1984, 31:428-434.
3. Andrews BT, Chiles BW, Olsen WL, Pitts LH. The effect of intracerebral hematoma location on the risk of brain-stem compression and on clinical outcome. J Neurosurg 1988 69:518-522.
4. Astrup J, Siesjö BK, Simon L. Thresholds in cerebral ischemia: the ischemic penumbra. Stroke 1981, 12:723-724.
5. Bogousslavsky J, Regli F. Atteinte intra-axiale du nerf moteur oculaire commun dans les infarctus mésencéphaliques. Rev Neurol 1984, 140:263-270.
6. Caplan DR. «Top of the basilar» syndrome. Neurology 1979, 30:72-79.
7. Castaigne P, Lhermitte F, Buge A, Escourolle R, Hauw JJ, Lyon-Caen O. Paramedian thalamic and midbrain infarcts: clinical and neuropathological study. Ann Neurol 1981, 10:127-148.
8. Cestan R, Gourgeois M. Syndrome de Weber avec autopsie. Rev Neurol 1900, 8:428-431.
9. Charcot JM. Sur un cas d'hystérie simulatrice du syndrome de Weber. In Clinique des Maladies du système Nerveux, T. 1. Paris: Veuve Babé et Cie, 1892, p 308-332.
10. Cushing H, Eisenhardt L. Meningiomas. Springfield: Charles C. Thomas, 1938, p 393.
11. Dehaene I, Dom R. A mesencephalic locked-in syndrome. J Neurol 1982, 227:255-259.
13. Dumont PAS, Laluci DH, Araújo MG. Thalamo-mesencephalic infarction after arterial catheterism (in Portuguese). Rev Méd IAMPSE 1987, 18:88-96.
14. Emura A, Takeuchi A, Hashimoto T, Inaba G, Akaoka I, Miyashita H. A case of Behcet's disease with Weber's syndrome. J Rheumatol 1986, 13:459-461.
15. Feeney DM, Baron JC. Diaschisis. Stroke 1986, 17:817-830.
16. Fisher CM. Abulia minor vs. (agitated behavior. Clin Neurosurg 1984, 31:9-31.
17. Fisher M, Smith TW, Jacobs R. Pure motor hemiplegia secondary to a saccular basilar artery aneurysm. Stroke 1982, 19:104-107.
18. Foix C, Hillemand F. Les artères de l'axe encéphalique jusq'au diencéphale inclusivement. Rev Neurol 1925, 6:705-739.
19. Friedman JH. Syndrome of diffuse encephalopathy due to nondominant thalamic infarction. Neurology 1985, 35:1524-1526.
20. Gillilan LA. The correlation of the blood supply to the human brain stem with clinical brain stem syndromes. J Neuropath Exp Neurol 1964, 23 :78-108.
21. Hinshaw DB, Thompson JR, Hasso AN, Casselman ES. Infarctions of the brainstem and cerebellum: a correlation of computed tomography and angiography. Radiology 1980, 137:105-112.
22. Ho KL. Pure motor hemiplegia due to infarction of the cerebral peduncle. Arch Neurol 1982, 39:524-526.
23. Jurgensen JC, Towfighi J, Brennan RW, Jeffreys WH. Symmetric brainstem necrosis in an adult following hypotension: an arterial end-zone infarct? Stroke 1983, 14:967-970.
24. Kaplan HA. Arteries of the brain : an anatomic study. Acta Radiol (Stock) 1956, 46: 364-370.
25. Kaplan HA. Collateral circulation of the brain. Neurology 1961, 11:9-15.
26. Karp JS, Hurtig HI. «Locked-in state» with bilateral midbrain infarcts. Arch Neurol 1974, 30:176-178.
27. Katz DI, Alexander MP, Mandell AM. Dementia following strokes in the mesencephalon and diencephalon. Arch Neurol 1987, 44 :1127-1133.
28. Koroshetz WJ, Ropper AJ. Artery-to-artery embolism causing stroke in the posterior circulation. Neurology 1987, 37 : 292-296.
29. Lassen NA, Olsen TS, Hojgaard K, Skriver E. Incomplete infarction: a CT-negative irreversible ischemic brain lesion. J Cer Blood Flow Metab 1983, 3(S1) :S602-603.
30. Lazorthes G, Poulhes, J, Bastide G, Roulleau J. Les territoires artériels du tronc cérébral: recherches anatomiques et syndromes vasculaires. Presse Méd 1958, 91 : 2048-2051.
31. Mehler MF. Reversible rostral basilar artery syndrome. Arch Intern Med 1988, 148: 166-169.
32. Mohr JP. Distal field infarction. Neurology 1969. 19:279.
33. Nathan PW, Smith MC. Long descending tracts in man: I. Review of present knowledge. Brain 1955, 78:248-303.
34. Oliveira-Souza R. Motor hemiplegia and the cerebral organization of movement in man: II. The myth of the human extrapyramidal system. Arq Neuro-Psiquiat (São Paulo) 1989, 47:16-27.
35. Olsen TS, Bruhn P, öberg RGE. Cortical hypoperfusion as a possible cause of «subcortical aphasia». Brain 1986, 109:393-410.
36. Percheron G. Les artères du thalamus humain: II. Artères et territoires thalamiques paramédians de l'artère basilaire communicante. Rev Neurol 1976, 132:309-324.
37. Ross ED. Localization of the pyramidal tract in the internal capsule by whole brain dissection. Neurology 1980, 30:59-64.
38. Rowley HA. Lowenstein DH, Rowbotham MC, Simon RP. Thalamomesencephalic strokes after cocaine abuse. Neurology 1989, 39:428-430.
39. Segarra JM. Cerebral vascular disease and behavior: I. The syndrome of the mesencephalic artery (basilar artery bifurcation). Arch Neurol 1970, 22:408-418.
40. Sieben G, de Reuck J, Vander Eeken H. Thrombosis of the mesencephalic artery. Acta Neurol Belg 1977, 77:151-162.
41. Souques A. Double syndrome de Weber. Rev Neurol 1900, 8:148-149.
42. Stiller J, Shanzer S, Yang W. Brainstem lesion with pure motor hemiparesis: computed tomographic demonstration. Arch Neurol 1982, 39 :660-661.
43. Vander Eecken H, Adams RD. The anatomy and functional significance of the meningeal arterial anastomoses of the human brain. J Neurophath Exp Neurol 1953, 12:132-157.
44. Verhaart WJC. The pes pedunculi and pyramid. J Comp Neurol 1948, 88:139-155.
45. Weber H. A contribution to the pathology of the crura cerebri. Med Chir Trans 1863, 28:121-189.