Cortico-basal ganglionic degeneration a case report

Oliveira, J. Teotônio de; Cardoso, Francisco E. Cota

doi:10.1590/S0004-282X1992000200016

Abstracts

The case of a Brazilian patient with cortico-basal ganglionic degeneration (CBGD) is presented. Since three years ago, a 71-year old male displays asymmetric ideomotor apraxia, gait apraxia, cortical sensory impairment, myoclonus, limp dystonia and rigidity. His mental status is spared. There is neither consanguinity nor similar cases in his family. The differential diagnosis of CBGD is discussed. A brief review of the literature is made stressing the clinical and pathological features of CBGD. This disease is poorly known and probably underdiagnosed. Its diagnosis can be safely made based on clinical grounds.

cortico-basal ganglionar degeneration; clinicail diagnosis; management

É apresentado o caso de um paciente brasileiro com degeneração córtico-ganglio-nar basal (DCGB). Há três anos um homem de 71 anos de idade apresenta-se com apraxia ideomotora, apraxia de marcha, déficit sensitivo cortical, rigidez, mioclonias e distonia apendicular. Todos estes sinais são assimétricos. Seu estado mental está preservado. Não há consangüinidade ou casos semelhantes na família. O diagnóstico diferencial desta nova entidade é discutido. Também é feita breve revisão da literatura, dando ênfase aos aspectos clínicos e patológicos da DCGB. Esta doença é mal conhecida e, possivelmente, pouco diagnosticada. O diagnóstico pode ser feito, com segurança, baseado em dados clínicos.

degeneração cortiço ganglionar basal; diagnóstico clínico; conduta

Cortico-basal ganglionic degeneration a case report

Degeneração ganglionar córtico-basal: registro de caso

J. Teotônio de Oliveira; Francisco E. Cota Cardoso

Service of Neurology, Hospital das Clínicas, The Federal University of Minas Gerais (UFMG)

SUMMARY

The case of a Brazilian patient with cortico-basal ganglionic degeneration (CBGD) is presented. Since three years ago, a 71-year old male displays asymmetric ideomotor apraxia, gait apraxia, cortical sensory impairment, myoclonus, limp dystonia and rigidity. His mental status is spared. There is neither consanguinity nor similar cases in his family. The differential diagnosis of CBGD is discussed. A brief review of the literature is made stressing the clinical and pathological features of CBGD. This disease is poorly known and probably underdiagnosed. Its diagnosis can be safely made based on clinical grounds.

Key words: cortico-basal ganglionar degeneration, clinicail diagnosis, management.

RESUMO

É apresentado o caso de um paciente brasileiro com degeneração córtico-ganglio-nar basal (DCGB). Há três anos um homem de 71 anos de idade apresenta-se com apraxia ideomotora, apraxia de marcha, déficit sensitivo cortical, rigidez, mioclonias e distonia apendicular. Todos estes sinais são assimétricos. Seu estado mental está preservado. Não há consangüinidade ou casos semelhantes na família. O diagnóstico diferencial desta nova entidade é discutido. Também é feita breve revisão da literatura, dando ênfase aos aspectos clínicos e patológicos da DCGB. Esta doença é mal conhecida e, possivelmente, pouco diagnosticada. O diagnóstico pode ser feito, com segurança, baseado em dados clínicos.

Palavras-chave: degeneração cortiço ganglionar basal, diagnóstico clínico, conduta.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Dr. José Teotônio de Oliveira Hospital das Clínicas, UFMG - Serviço de Neurologia, 5º andar - Av. Alfredo Balena 110 . 30130 Belo Horizonte MG - Brasil.

1. Case Records of the Massachussets General Hospital. Case 38, 1985. N Engl J Med 1985, 313:739-748.
2. DeJong RN. The Neurologic Examination. Ed 4. Philadelphia: Harper & Row, 1987, p 73-77.
3. Folstein MF, Folstein SE, McHugh PR. Minimental state: a practical method for grading the cognition state for the clinician. J Psychiat Res 1975, 12:189-198.
4. Gibb WRG, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989, 112:1171-1192.
5. Golbe LI, Davis PH. Progressive supranuclear palsy: recent advances. In: Jankovic J, Tolosa E (eds): Parkinson's Disease and Movement Disorders. Baltimore: Urban & Schwarzenberg, 1988, p 121-130.
6. Greene PE, Fahn S, Lang AE, Watts RL, Eidelberg D, Powers JM. What is it? Case 1, 1990: Progressive unilateral rigidity, bradykinesia, tremulousness, and apraxia, leading to fixed postural deformity of the involved limb. Mov Disord 1990, 5:341-351.
7. Jagust WJ, Davies P, Tiller-Borcich JK, Reed BR. Focal Alzheimer's disease. Neurology 1990, 40:14-19.
8. Jankovic J, Fahn S. Dystonic syndromes. In: Jankovic J Tolosa E (eds.): Parkinson's Disease and Movement Disorders. Baltimore: Urban & Schwarzenberg, 1988, p 283-314.
9. Katzman R, Brown T, Fuld P, Peck A, Schechter R, Schimmel H. Validation of a short orientation-memory-concentration test of cognitive impairment. Am J Psychiat 1983, 140:734-739.
10. Lewis AJ, Gawel MJ. Diffuse Lewy body disease with dementia and oculomotor dysfunction. Mov Disord 1990, 5:143-147.
11 McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan EM. Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA work group under the auspices of Department of Health and Human Services Task Force on Alzheimer's Disease. Neurology 1984, 34:939-944.
12. Neary D. Non-Alzheimer's disease forms of cerebral atrophy. J Neurol Neurosurg Psychiat 1990, 13:929-1931.
13. Obeso JA, Artieda JA, Marsden CD. Different clinical presentations of myoclonus. In: Jankovic J, Tolosa E (eds): Parkinson's Disease and Movement Disorders. Baltimore: Urban & Schwarzenberg, 1988, p 263-274.
14. Prusdner SB. Prions and neurodegenerative diseases. N Engl J Med 1987, 317:1571-1581.
15. Quinn N. Multiple system atrophy: the nature of the beast. J Neurol Neurosurg Psychiat 1989 (Special Suppl):78-89.
16. Rebetz JJ, Kolodny EH, Richardson EP. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968, 18:20-33.
17. Riley DE, Lang AE. Corticobasal ganglionic degeneration: further observations in six additional cases. Neurology 1988, 38 (Suppl 1):360.
18. Riley DE, Lang AE, Lewis A, Reseh L, Ashby P, Horny Kiewicz D, Black S. Cortical-basal ganglionic degeneration. Neurology 1990, 40:1203-1212.
19. Watts RL, Mirra SS, Young RR, Burger PC, Villier JA, Heyman A. Cortico-basal ganglionic degeneration with neuronal achromasia: clinical-pathological study ot two cases. Neurology 1989, 39(Suppl 1):140.
20 Watts RL, Williams RS, Growdon JD, Young RR, Haley EO Jr, Beal MF. Cortico-basal ganglionic degeneration. Neurology 1985, 35(Suppl 1) :178.

Publication Dates

Publication in this collection
22 Feb 2011
Date of issue
June 1992

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

[1] 1. Case Records of the Massachussets General Hospital. Case 38, 1985. N Engl J Med 1985, 313:739-748.

[2] 2. DeJong RN. The Neurologic Examination. Ed 4. Philadelphia: Harper & Row, 1987, p 73-77.

[3] 3. Folstein MF, Folstein SE, McHugh PR. Minimental state: a practical method for grading the cognition state for the clinician. J Psychiat Res 1975, 12:189-198.

[4] 4. Gibb WRG, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989, 112:1171-1192.

[5] 5. Golbe LI, Davis PH. Progressive supranuclear palsy: recent advances. In: Jankovic J, Tolosa E (eds): Parkinson's Disease and Movement Disorders. Baltimore: Urban & Schwarzenberg, 1988, p 121-130.

[6] 6. Greene PE, Fahn S, Lang AE, Watts RL, Eidelberg D, Powers JM. What is it? Case 1, 1990: Progressive unilateral rigidity, bradykinesia, tremulousness, and apraxia, leading to fixed postural deformity of the involved limb. Mov Disord 1990, 5:341-351.

[7] 7. Jagust WJ, Davies P, Tiller-Borcich JK, Reed BR. Focal Alzheimer's disease. Neurology 1990, 40:14-19.

[8] 8. Jankovic J, Fahn S. Dystonic syndromes. In: Jankovic J Tolosa E (eds.): Parkinson's Disease and Movement Disorders. Baltimore: Urban & Schwarzenberg, 1988, p 283-314.

[9] 9. Katzman R, Brown T, Fuld P, Peck A, Schechter R, Schimmel H. Validation of a short orientation-memory-concentration test of cognitive impairment. Am J Psychiat 1983, 140:734-739.

[10] 10. Lewis AJ, Gawel MJ. Diffuse Lewy body disease with dementia and oculomotor dysfunction. Mov Disord 1990, 5:143-147.

[11] 11 McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan EM. Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA work group under the auspices of Department of Health and Human Services Task Force on Alzheimer's Disease. Neurology 1984, 34:939-944.

[12] 12. Neary D. Non-Alzheimer's disease forms of cerebral atrophy. J Neurol Neurosurg Psychiat 1990, 13:929-1931.

[13] 13. Obeso JA, Artieda JA, Marsden CD. Different clinical presentations of myoclonus. In: Jankovic J, Tolosa E (eds): Parkinson's Disease and Movement Disorders. Baltimore: Urban & Schwarzenberg, 1988, p 263-274.

[14] 14. Prusdner SB. Prions and neurodegenerative diseases. N Engl J Med 1987, 317:1571-1581.

[15] 15. Quinn N. Multiple system atrophy: the nature of the beast. J Neurol Neurosurg Psychiat 1989 (Special Suppl):78-89.

[16] 16. Rebetz JJ, Kolodny EH, Richardson EP. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968, 18:20-33.

[17] 17. Riley DE, Lang AE. Corticobasal ganglionic degeneration: further observations in six additional cases. Neurology 1988, 38 (Suppl 1):360.

[18] 18. Riley DE, Lang AE, Lewis A, Reseh L, Ashby P, Horny Kiewicz D, Black S. Cortical-basal ganglionic degeneration. Neurology 1990, 40:1203-1212.

[19] 19. Watts RL, Mirra SS, Young RR, Burger PC, Villier JA, Heyman A. Cortico-basal ganglionic degeneration with neuronal achromasia: clinical-pathological study ot two cases. Neurology 1989, 39(Suppl 1):140.

[20] 20 Watts RL, Williams RS, Growdon JD, Young RR, Haley EO Jr, Beal MF. Cortico-basal ganglionic degeneration. Neurology 1985, 35(Suppl 1) :178.