Paralisia supranuclear progressiva (síndrome de steele-richardson-olszewski) apresentação de caso e revisão da literatura

Trevisol-Bittencourt, Paulo Cesar

doi:10.1590/S0004-282X1992000300018

Resumos

Paralisia supranuclear progressiva (PSP) foi inicialmente identificada como entidade distinta por Richardson, Steele e Olszewski há cerca de 25 anos. Observações clínicas subsequentes não apenas têm confirmado mas, também acrescentaram novas informações aos relatos pioneiros. PSP pode ser descrita como transtorno neurológico gradualmente incapacitante com rigidez extrapiramidal, bradieinesia, dificuldade na marcha com quedas frequentes, paralisia pseudo-bulbar, demência e oftalmoplegia supranuclear característica. Incide em pessoas na meia idade ou idosas, sem distinção racial ou sexual. PSP é importante causa de parkinsonismo e sua etiologia permanece obscura. Neste registro é apresentado o caso de um paciente de Santa Catarina, exibindo inequívoca evidência clínica de PSP. Este é o primeiro caso descrito neste Estado onde, baseando-se em recentes premissas epidemiológicas, poderemos supor a existência de pelo menos mais 50 pacientes com PSP. Em complementação, é apresentada revisão da literatura, com ênfase a aspectos clínicos e terapêuticos da PSP.

paralisia supranuclear progressiva; parkinsonismo; demência; epilepsia; flunarizina

Progressive supranuclear palsy (PSP) was first recognized as a distinct syndrome by Richardson, Steele and Olszewski roughly a quarter century ago. Subsequent clinical experience has corroborated and enlarged their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is a significant cause of parkinsonism and its etiology remains obscure. The case of a patient from Santa Catarina who presented definite clinical evidences of this syndrome is reported. This is the first description in this southern Brazilian State, where at least 50 more patients should exist, if we may extrapolate the prevalence rate of this condition in developed countries to this well developed area of Brazil. A review of the literature was undertaken with emphasis on recent clinical and therapeutic aspects of PSP.

progressive supranuclear palsy; parkinsonism; dementia; epilepsy; flunarizin

Paralisia supranuclear progressiva (síndrome de steele-richardson-olszewski) apresentação de caso e revisão da literatura

Progressive supranuclear palsy: a case report and review of literature

Paulo Cesar Trevisol-Bittencourt

Disciplina de Neurologia do Departamento de Clínica Médica e Grupo de Pesquisas em Engenharia Biomédica, Universidade Federal de Santa Catarina (UFSC)

RESUMO

Paralisia supranuclear progressiva (PSP) foi inicialmente identificada como entidade distinta por Richardson, Steele e Olszewski há cerca de 25 anos. Observações clínicas subsequentes não apenas têm confirmado mas, também acrescentaram novas informações aos relatos pioneiros. PSP pode ser descrita como transtorno neurológico gradualmente incapacitante com rigidez extrapiramidal, bradieinesia, dificuldade na marcha com quedas frequentes, paralisia pseudo-bulbar, demência e oftalmoplegia supranuclear característica. Incide em pessoas na meia idade ou idosas, sem distinção racial ou sexual. PSP é importante causa de parkinsonismo e sua etiologia permanece obscura. Neste registro é apresentado o caso de um paciente de Santa Catarina, exibindo inequívoca evidência clínica de PSP. Este é o primeiro caso descrito neste Estado onde, baseando-se em recentes premissas epidemiológicas, poderemos supor a existência de pelo menos mais 50 pacientes com PSP. Em complementação, é apresentada revisão da literatura, com ênfase a aspectos clínicos e terapêuticos da PSP.

Palavras-chave: paralisia supranuclear progressiva, parkinsonismo, demência, epilepsia, flunarizina.

SUMMARY

Progressive supranuclear palsy (PSP) was first recognized as a distinct syndrome by Richardson, Steele and Olszewski roughly a quarter century ago. Subsequent clinical experience has corroborated and enlarged their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is a significant cause of parkinsonism and its etiology remains obscure. The case of a patient from Santa Catarina who presented definite clinical evidences of this syndrome is reported. This is the first description in this southern Brazilian State, where at least 50 more patients should exist, if we may extrapolate the prevalence rate of this condition in developed countries to this well developed area of Brazil. A review of the literature was undertaken with emphasis on recent clinical and therapeutic aspects of PSP.

Key words: progressive supranuclear palsy, parkinsonism, dementia, epilepsy, flunarizin.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Agradecimentos  O autor expressa sua gratidão ao apoio material da National Society for Epilepsy do Reino Unido; ao inestimável estímulo do amigo Ley Sander; ao Dr. Li Shih Man, por ter referido o paciente; aos Drs. Renato Garcia Ojeda e Monique von Hertwig, pela valiosa ajuda na redação deste artigo.

Dr. Paulo Cesar Trevisol Bittencourt Disciplina de Neurologia, Departamento de Clínica Médica - Hospital Universitário, UFSC - 88049 Florianópolis SC - Brasil.

1. Bernick C. Methysergide-induced akathisia. Clin Neuropharmacol 1988, 11 :87-89.
2. Brusa A. Degenerescence plurisystematisée du neuraxe de caractère sporadique, à debut tardif et évolution subaiguë. Rev Neurol 1961, 104 : 412-429.
3. Bruns R. Difficulties in long term management of Parkinson's disease. Aust Fam Psysician 1984, 13:14-16.
4. Cambier J, Masson M, Viader F. Limodin J, Strube A. Le syndrome frontal de la paralysie supranucléaire progressive. Rev Neurol 1985, 141 : 528-536.
5. Chavany JA, van Bogaert L, Godlewski S. Sur un syndrome de rigidité à predominance axiale avec perturbation des automatismes oculo-palpebraux d'origine encephalitique. Presse Med 1957, 59:958-962.
6. Daroff RB. Progressive supranuclear palsy: a brief personalized history. Yale J Biol Med 1987, 60:119-122.
7. Dubinsky RM, Jankovic J. Progressive supranuclear palsy and a multi-infarct state. Neurology 1987, 37:570-576.
8. Duncombe AS, Lees AJ. Methysergide in progressive supranuclear palsy. Neurology 1985, 35:936-937.
9. Duvoisin RC, Golbe LI, Lepore FE. Progressive supranuclear palsy. Can J Neurol Sci 1987, 14:547-554.
10. Goffinet AM, De Voider AG, Gillain C, Rectem D, Bol A, Michel C, Cogneau M, Labar D, Laterre C. Positron tomography demonstrates frontal lobe hypometabolism in progressive supranuclear palsy. Ann Neurol 1989, 25:131-139.
11. Gomori AJ, Sima AAF. An atypical case of progressive supranuclear palsy. Can J Neurol Sci 1984, ll : 48-52.
12. Guiloff RJ, George RJ, Marsden CD. Reversible supranuclear ophthalmoplegia associated with parkinsonism. J Neurol Neurosurg Psychiatry 1980, 43 :552-554.
13. Imai H, Narabayashi H, Sakata E. «Pure akinesia» and the later added supranuclear ophthalmoplegia. Adv Neurol 1987, 45 :207-212.
14. Izzo KL, Dilorenzo P, Roth A. Rehabilitation in progressive supranuclear palsy: case report. Arch Phys Med Rehab 1986, 67:473-476.
15. Jankovic J. Progressive supranuclear palsy: paraneoplastic effect of bronchial carcinoma. Neurology 1985, 35 :446-447.
16. Kato T, Hirano A, Weinberg MN, Jacobs AK. Spinal cord lesions in progressive supranuclear palsy: some new observations. Acta Neuropathol 1986, 17 :11-14.
17. Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome. Neurology 1983, 13:273-278.
18. Neumann MA. Heterogenous system degeneration with particular involvement of reticular substance: correlation with neurologic disorders and the concept of Creutzfeld-Jacob disease. J Neuropath Exp Neurol 1961, 20:316.
19. Newman GC. Treatment of progressive supranuclear palsy with tricyclic antidepressants. Neurology 1985, 35:1189-1193.
20. Nygaard TG, Duvoisin RC, Manocha M, Chokroverty S. Seizures in progressive supranuclear palsy. Neurology 1989, 39:138-140.
21. Page NGR, Lean JS, Sanders MIX Vertical supranuclear gaze palsy with secondary syphilis. J Neurol Neurosurg Psychiatry 1982, 45 : 86-88.
22. Rafal RD, Grimm RJ. Progressive supranuclear palsy: functional analysis of the response to methysergide and antiparkinsonian agents. Neurology 1981, 31:1507-1518.
23. Ramachandran T, Kim RC, Culebras A. Progressive supranuclear palsy and falcotentorial meningioma. Arch Neurol 1982, 39:68.
24. Richardson JC, Steele J, Olszweski J. Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia: a clinical report on eight cases of heterogenous system degenerations Trans Am Neurol Assoc 1963, 88 :25-29.
25. Saitoh H, Yoshii F, Shinohara Y. Computed tomographic findings in progressive supranuclear palsy. Neuroradiology 1987, 29:168-171.
26. Schneider LS, Chui HC, Progressive supranuclear palsy manifesting with depressive features. J Am Geriatr Soc 1986, 34 :663-665.
27. Sonsalla PK, Golbe LI. Deprenyl as prophylaxis against Parkinson's disease. Clin Neu-ropharmacol 1988, 11:387-400.
28. Steele J, Richardson JC, Olszweski J. Progressive supranuclear palsy. Arch Neurol 1964, 10:333-359.
29. Steele J. Progressive supranuclear palsy. Brain 1972, 95 : 693-704.
30. Strub RL. Frontal lobe syndrome in a patient with bilateral globus pallidus lesions. Arch Neurol 1989, 46:1024-1027.
31. Trevisol-Bittencourt PC. Flunarizina induzindo> parkinsonismo. Arq Cat Med 1990, 19:81-84.
32. Trzepacz PT, Murcko AC, Gillespie MP. Progressive supranuclear palsy misdiagnosed as schizophrenia. J Nerv Ment Pis 1985, 173:377-378.
33. Verhaart WJC. Degeneration of the brain stem and the cerebellum: an example of heterogenous systemic degeneration of the central nervous system. J Neuropath Exp Neurol 1958, 17:382-391.
34. Watts RL, Krieg MM, Freeman A, Mandir A, Iuvone PM. Progressive supranuclear palsy : clinical, physiological, radiographic and biochemical assessment. Abstracts of XIV World Congress of Neurology. October 22-27, New Delhi, 1989.

Datas de Publicação

Publicação nesta coleção
22 Fev 2011
Data do Fascículo
Set 1992

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

[1] 1. Bernick C. Methysergide-induced akathisia. Clin Neuropharmacol 1988, 11 :87-89.

[2] 2. Brusa A. Degenerescence plurisystematisée du neuraxe de caractère sporadique, à debut tardif et évolution subaiguë. Rev Neurol 1961, 104 : 412-429.

[3] 3. Bruns R. Difficulties in long term management of Parkinson's disease. Aust Fam Psysician 1984, 13:14-16.

[4] 4. Cambier J, Masson M, Viader F. Limodin J, Strube A. Le syndrome frontal de la paralysie supranucléaire progressive. Rev Neurol 1985, 141 : 528-536.

[5] 5. Chavany JA, van Bogaert L, Godlewski S. Sur un syndrome de rigidité à predominance axiale avec perturbation des automatismes oculo-palpebraux d'origine encephalitique. Presse Med 1957, 59:958-962.

[6] 6. Daroff RB. Progressive supranuclear palsy: a brief personalized history. Yale J Biol Med 1987, 60:119-122.

[7] 7. Dubinsky RM, Jankovic J. Progressive supranuclear palsy and a multi-infarct state. Neurology 1987, 37:570-576.

[8] 8. Duncombe AS, Lees AJ. Methysergide in progressive supranuclear palsy. Neurology 1985, 35:936-937.

[9] 9. Duvoisin RC, Golbe LI, Lepore FE. Progressive supranuclear palsy. Can J Neurol Sci 1987, 14:547-554.

[10] 10. Goffinet AM, De Voider AG, Gillain C, Rectem D, Bol A, Michel C, Cogneau M, Labar D, Laterre C. Positron tomography demonstrates frontal lobe hypometabolism in progressive supranuclear palsy. Ann Neurol 1989, 25:131-139.

[11] 11. Gomori AJ, Sima AAF. An atypical case of progressive supranuclear palsy. Can J Neurol Sci 1984, ll : 48-52.

[12] 12. Guiloff RJ, George RJ, Marsden CD. Reversible supranuclear ophthalmoplegia associated with parkinsonism. J Neurol Neurosurg Psychiatry 1980, 43 :552-554.

[13] 13. Imai H, Narabayashi H, Sakata E. «Pure akinesia» and the later added supranuclear ophthalmoplegia. Adv Neurol 1987, 45 :207-212.

[14] 14. Izzo KL, Dilorenzo P, Roth A. Rehabilitation in progressive supranuclear palsy: case report. Arch Phys Med Rehab 1986, 67:473-476.

[15] 15. Jankovic J. Progressive supranuclear palsy: paraneoplastic effect of bronchial carcinoma. Neurology 1985, 35 :446-447.

[16] 16. Kato T, Hirano A, Weinberg MN, Jacobs AK. Spinal cord lesions in progressive supranuclear palsy: some new observations. Acta Neuropathol 1986, 17 :11-14.

[17] 17. Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome. Neurology 1983, 13:273-278.

[18] 18. Neumann MA. Heterogenous system degeneration with particular involvement of reticular substance: correlation with neurologic disorders and the concept of Creutzfeld-Jacob disease. J Neuropath Exp Neurol 1961, 20:316.

[19] 19. Newman GC. Treatment of progressive supranuclear palsy with tricyclic antidepressants. Neurology 1985, 35:1189-1193.

[20] 20. Nygaard TG, Duvoisin RC, Manocha M, Chokroverty S. Seizures in progressive supranuclear palsy. Neurology 1989, 39:138-140.

[21] 21. Page NGR, Lean JS, Sanders MIX Vertical supranuclear gaze palsy with secondary syphilis. J Neurol Neurosurg Psychiatry 1982, 45 : 86-88.

[22] 22. Rafal RD, Grimm RJ. Progressive supranuclear palsy: functional analysis of the response to methysergide and antiparkinsonian agents. Neurology 1981, 31:1507-1518.

[23] 23. Ramachandran T, Kim RC, Culebras A. Progressive supranuclear palsy and falcotentorial meningioma. Arch Neurol 1982, 39:68.

[24] 24. Richardson JC, Steele J, Olszweski J. Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia: a clinical report on eight cases of heterogenous system degenerations Trans Am Neurol Assoc 1963, 88 :25-29.

[25] 25. Saitoh H, Yoshii F, Shinohara Y. Computed tomographic findings in progressive supranuclear palsy. Neuroradiology 1987, 29:168-171.

[26] 26. Schneider LS, Chui HC, Progressive supranuclear palsy manifesting with depressive features. J Am Geriatr Soc 1986, 34 :663-665.

[27] 27. Sonsalla PK, Golbe LI. Deprenyl as prophylaxis against Parkinson's disease. Clin Neu-ropharmacol 1988, 11:387-400.

[28] 28. Steele J, Richardson JC, Olszweski J. Progressive supranuclear palsy. Arch Neurol 1964, 10:333-359.

[29] 29. Steele J. Progressive supranuclear palsy. Brain 1972, 95 : 693-704.

[30] 30. Strub RL. Frontal lobe syndrome in a patient with bilateral globus pallidus lesions. Arch Neurol 1989, 46:1024-1027.

[31] 31. Trevisol-Bittencourt PC. Flunarizina induzindo> parkinsonismo. Arq Cat Med 1990, 19:81-84.

[32] 32. Trzepacz PT, Murcko AC, Gillespie MP. Progressive supranuclear palsy misdiagnosed as schizophrenia. J Nerv Ment Pis 1985, 173:377-378.

[33] 33. Verhaart WJC. Degeneration of the brain stem and the cerebellum: an example of heterogenous systemic degeneration of the central nervous system. J Neuropath Exp Neurol 1958, 17:382-391.

[34] 34. Watts RL, Krieg MM, Freeman A, Mandir A, Iuvone PM. Progressive supranuclear palsy : clinical, physiological, radiographic and biochemical assessment. Abstracts of XIV World Congress of Neurology. October 22-27, New Delhi, 1989.