Parkinsonismo precoce associado a lesões palidais de tipo "eye-of-the-tiger"

Barbosa, Egberto Reis; Bittar, Mirian Salvador; Bacheschi, Luiz Alberto; Comerlatti, Luiz Roberto; Scaff, Milberto

doi:10.1590/S0004-282X1995000200021

Resumos

Relata-se o caso de uma paciente com quadro parkisoniano instalado aos 26 anos de idade, 30 anos de evolução, boa resposta à levodopa e desenvolvimento precoce de discinesias induzidas por essa droga. Essas características associadas à ausência de outras manifestações neurológicas ao longo de todo o curso da moléstia sugeriam substrato anátomo-patológico superponível ao da forma clássica da doença de Parkinson. Entretanto, as imagens de ressonância magnética mostraram lesões palidais do tipo "eye-of-the-tiger", semelhantes às observadas na doença de Hallervorden-Spatz. O presente caso ilustra a heterogeneidade patológica dos casos de parkinsonismo de instalação precoce e a possibilidade do encontro desse padrão de lesões palidais em outras doenças degenerativas dos gânglios da base.

parkinsonismo precoce; doença de Hallervorden-Spatz; imagem de ressonância magnética

We report the case of a 56-years-old woman patient, born to unrelated parents, who since 26-years-old gradually developed bradykinesia, rigidity, tremor of both hands, and speech and gait difficulties. Her past history was unremarkable. There was no family history of neurologic disease. She was admitted to our Hospital at age 39 and at that time she presented a full parkinsonian syndrome. The following tests were normal or negative: routine blood studies, serum copper, ceruloplasmin and cerebrospinal fluid examination. There was not Kayser-Fleicher ring, and fundoscopic examination revealed no abnormalities. Levodopa was introduced and response was good for more than ten years, despite early-onset of dyskinesias (three months after the introduction of the drug). After 30 years under levodopa she still presents a moderate response but with severe fluctuations of the motor performance. Except for slowness of cognition she developed no other neuropsychological impairments, and a recent neurological examination disclosed no abnormalities besides a parkinsonian syndrome. One year ago, a magnetic resonance imaging (MRI) was perfomed and showed bilateral, symmetrical lesions with "eye-of-the-tiger" pattern. This case illustrates the pathological heterogenity of early-onset parkinsonism and suggests the possibility to find the typical MRI lesions seen in Hallervorden-Spatz disease in other degenerative affections involving globus pallidus.

early-onset parkinsonism; Hallervorden-Spatz disease; magnetic resonance imaging

Parkinsonismo precoce associado a lesões palidais de tipo "eye-of-the-tiger"

"Eye-of-the-tiger" sign at MRI in a patient with early-onset parkinsonism

Egberto Reis Barbosa^I; Mirian Salvador Bittar^II; Luiz Alberto Bacheschi^III; Luiz Roberto Comerlatti^I; Milberto Scaff^IV

^IMédico Assistente

^IIMédica Colaboradora

^IIIProfessor Adjunto

^IVProfessor Titular

RESUMO

Relata-se o caso de uma paciente com quadro parkisoniano instalado aos 26 anos de idade, 30 anos de evolução, boa resposta à levodopa e desenvolvimento precoce de discinesias induzidas por essa droga. Essas características associadas à ausência de outras manifestações neurológicas ao longo de todo o curso da moléstia sugeriam substrato anátomo-patológico superponível ao da forma clássica da doença de Parkinson. Entretanto, as imagens de ressonância magnética mostraram lesões palidais do tipo "eye-of-the-tiger", semelhantes às observadas na doença de Hallervorden-Spatz. O presente caso ilustra a heterogeneidade patológica dos casos de parkinsonismo de instalação precoce e a possibilidade do encontro desse padrão de lesões palidais em outras doenças degenerativas dos gânglios da base.

Palavras-chave:parkinsonismo precoce, doença de Hallervorden-Spatz, imagem de ressonância magnética.

SUMMARY

We report the case of a 56-years-old woman patient, born to unrelated parents, who since 26-years-old gradually developed bradykinesia, rigidity, tremor of both hands, and speech and gait difficulties. Her past history was unremarkable. There was no family history of neurologic disease. She was admitted to our Hospital at age 39 and at that time she presented a full parkinsonian syndrome. The following tests were normal or negative: routine blood studies, serum copper, ceruloplasmin and cerebrospinal fluid examination. There was not Kayser-Fleicher ring, and fundoscopic examination revealed no abnormalities. Levodopa was introduced and response was good for more than ten years, despite early-onset of dyskinesias (three months after the introduction of the drug). After 30 years under levodopa she still presents a moderate response but with severe fluctuations of the motor performance. Except for slowness of cognition she developed no other neuropsychological impairments, and a recent neurological examination disclosed no abnormalities besides a parkinsonian syndrome. One year ago, a magnetic resonance imaging (MRI) was perfomed and showed bilateral, symmetrical lesions with "eye-of-the-tiger" pattern. This case illustrates the pathological heterogenity of early-onset parkinsonism and suggests the possibility to find the typical MRI lesions seen in Hallervorden-Spatz disease in other degenerative affections involving globus pallidus.

Key-words: early-onset parkinsonism, Hallervorden-Spatz disease, magnetic resonance imaging.

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Aceite: 7-outubro-1994.

Clínica Neurológica do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (FMUSP):

Dr Egberto Reis Barbosa - Divisão de Clínica Neurológica, Hospital das Clínicas, FMUSP - Caixa Postal 8091 - 01065-970 São Paulo SP - Brasil.

1. Adamo J Jr. Doença de Hallervorden-Spatz: descriçăo de um caso. Arq Neuropsiquiatr 1993, 51: 130-133.
2. Alberca R, Rafael E, Chincon I, Vadillo J, Navarro A. Late onset parkinsonian syndrome in Hallervorden-Spatz disease. J Neurol Neurosurg Psychiatry 1987, 50: 1665-1668.
3. Andrade LAF. Contribuiçăo ao estudo da doença de Parkinson de início precoce: análise clínica de 58 pacientes. Tese de Livre Docęncia, Escola Paulista de Medicina. Săo Paulo, 1994.
4. Angelini L, Nardocci N, Rumi V, Zorzi C, Strada L, Savoiardo M. Hallervorden-Spatz disease: clinical and MRI study of 11 cases diagnosed in life. J Neurol 1992, 239: 417-425.
5. Bury JS. Two cases of paralysis agitans in the same family in which improvement followed administration of hyoscine. Lancet 1902, 1: 1097.
6. Davison C. Pallido-pyramidal syndrome. J Neuropath Exp Neurol 1954, 13: 50.
7. Drayer BP. Magnetic resonance imaging and extrapyramidal movement disorders. Eur Neurol 1989, 29 (SuppI): 9-12.
8. Gershanik OS. Early-onset parkinsonism. In Jankovic J, Tolosa E (eds). Parkinson's disease and movement disorders. Baltimore: Williams and Wilkins, 1993, p 235-252.
9. Gibb WRB. Neuropathology in movement disorders. J Neurol Neurosurg Psychiatry 1989,52 (Suppl): 55-67.
10. Gibb WRB, Lees AJ. A comparison of clinical and pathological features of young- and old-onset Parkinson's disease. Neurology 1988, 38: 1402-1406.
11. Golbe LI. Young-onset Parkinson's disease: a clinical review. Neurology 1991,41: 168-173.
12. Hughes AJ, Daniel SE, Blankson S, Lees AJ. A clinicopathologic study of 100 cases of Parkinson's disease. Arch Neurol 1993, 50: 140-148.
13. Hunt JR. Progressive atrophy of the globus pallidus (primary atrophy of the pallidal system): a system disease of the paralysis type. Brain 1917, 40: 48-148.
14. Jankovic J, Kirkpatrick JB, Blomquist KA.Langlais PJ, Bird ED. Late - onset Hallervorden - Spatz disease presenting as familial parkinsonism. Neurology 1985, 35: 227-234.
15. Mutoh K, Okuno T, Ito M, Nakano S, Mikawa H, Fujisawa I, Asato R. MR imaging of a group I case of Hallervorden-Spatz disease. J Comp Assist Tomogr 1988,12: 851-853.
16. Rutledge JN, Hilal SK, Silver AJ, Defendini R, Fahn S. Study of movement disorders and brain iron by MR. AJNR 1987, 8: 397-411.
17. Scaff M, Barbosa ER, Assis JL, Canelas HM. Parkinsonismo juvenil: consideraçőes a respeito de 10 casos. Arq Neuropsiquiatr 1980, 38: 385-390.
18. Sethi KD, Adams RJ, Loring DW, El Gammal T. Hallervorden-Spatz syndrome: clinical and magnetic resonance imaging correlations. Ann Neurol 1988, 24: 692-694.
19. Shaffert DA, Johnsen SD, Johnson PC, Drayer BP. Magnetic resonance imaging in pathologically proven Hallervorden-Spatz disease. Neurology 1989, 39: 440-442.
20. Van Bogaert L. Contribution clinique et anatomique ŕ 1'étude de la paralysie agitante juvenile primitive. Rev Neurol (Paris) 1930, 2: 315.
21. Van der Knaap, Valk J, Nauta JJP. Pattern recogniton in magnetic resonance imaging of white matter disorders in children and young adults. Neuroradiology 1991, 33: 478-493.
22. Wang LN, Huang KW, Liu Ze Y. Sporadic late-onset Hallervorden-Spatz disease presenting as parkinsonism in a Chinese patient. Chin Med J 1990, 103: 686-688.
23. Willige H. Uber Paralysis agitans in jugendlichen. Alter Ztschr Gesamte Neurol Psychiatr 1911,4: 520-587.
24. Yokochi M, Narabayashi H, Iizuka R, Nagatsu T. Juvenile parkinsonism: some clinical, pharmacological, and neuropathological aspects. Adv Neurol 1984, 40: 407-413.

Datas de Publicação

Publicação nesta coleção
20 Jan 2011
Data do Fascículo
Jun 1995

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

[1] 1. Adamo J Jr. Doença de Hallervorden-Spatz: descriçăo de um caso. Arq Neuropsiquiatr 1993, 51: 130-133.

[2] 2. Alberca R, Rafael E, Chincon I, Vadillo J, Navarro A. Late onset parkinsonian syndrome in Hallervorden-Spatz disease. J Neurol Neurosurg Psychiatry 1987, 50: 1665-1668.

[3] 3. Andrade LAF. Contribuiçăo ao estudo da doença de Parkinson de início precoce: análise clínica de 58 pacientes. Tese de Livre Docęncia, Escola Paulista de Medicina. Săo Paulo, 1994.

[4] 4. Angelini L, Nardocci N, Rumi V, Zorzi C, Strada L, Savoiardo M. Hallervorden-Spatz disease: clinical and MRI study of 11 cases diagnosed in life. J Neurol 1992, 239: 417-425.

[5] 5. Bury JS. Two cases of paralysis agitans in the same family in which improvement followed administration of hyoscine. Lancet 1902, 1: 1097.

[6] 6. Davison C. Pallido-pyramidal syndrome. J Neuropath Exp Neurol 1954, 13: 50.

[7] 7. Drayer BP. Magnetic resonance imaging and extrapyramidal movement disorders. Eur Neurol 1989, 29 (SuppI): 9-12.

[8] 8. Gershanik OS. Early-onset parkinsonism. In Jankovic J, Tolosa E (eds). Parkinson's disease and movement disorders. Baltimore: Williams and Wilkins, 1993, p 235-252.

[9] 9. Gibb WRB. Neuropathology in movement disorders. J Neurol Neurosurg Psychiatry 1989,52 (Suppl): 55-67.

[10] 10. Gibb WRB, Lees AJ. A comparison of clinical and pathological features of young- and old-onset Parkinson's disease. Neurology 1988, 38: 1402-1406.

[11] 11. Golbe LI. Young-onset Parkinson's disease: a clinical review. Neurology 1991,41: 168-173.

[12] 12. Hughes AJ, Daniel SE, Blankson S, Lees AJ. A clinicopathologic study of 100 cases of Parkinson's disease. Arch Neurol 1993, 50: 140-148.

[13] 13. Hunt JR. Progressive atrophy of the globus pallidus (primary atrophy of the pallidal system): a system disease of the paralysis type. Brain 1917, 40: 48-148.

[14] 14. Jankovic J, Kirkpatrick JB, Blomquist KA.Langlais PJ, Bird ED. Late - onset Hallervorden - Spatz disease presenting as familial parkinsonism. Neurology 1985, 35: 227-234.

[15] 15. Mutoh K, Okuno T, Ito M, Nakano S, Mikawa H, Fujisawa I, Asato R. MR imaging of a group I case of Hallervorden-Spatz disease. J Comp Assist Tomogr 1988,12: 851-853.

[16] 16. Rutledge JN, Hilal SK, Silver AJ, Defendini R, Fahn S. Study of movement disorders and brain iron by MR. AJNR 1987, 8: 397-411.

[17] 17. Scaff M, Barbosa ER, Assis JL, Canelas HM. Parkinsonismo juvenil: consideraçőes a respeito de 10 casos. Arq Neuropsiquiatr 1980, 38: 385-390.

[18] 18. Sethi KD, Adams RJ, Loring DW, El Gammal T. Hallervorden-Spatz syndrome: clinical and magnetic resonance imaging correlations. Ann Neurol 1988, 24: 692-694.

[19] 19. Shaffert DA, Johnsen SD, Johnson PC, Drayer BP. Magnetic resonance imaging in pathologically proven Hallervorden-Spatz disease. Neurology 1989, 39: 440-442.

[20] 20. Van Bogaert L. Contribution clinique et anatomique ŕ 1'étude de la paralysie agitante juvenile primitive. Rev Neurol (Paris) 1930, 2: 315.

[21] 21. Van der Knaap, Valk J, Nauta JJP. Pattern recogniton in magnetic resonance imaging of white matter disorders in children and young adults. Neuroradiology 1991, 33: 478-493.

[22] 22. Wang LN, Huang KW, Liu Ze Y. Sporadic late-onset Hallervorden-Spatz disease presenting as parkinsonism in a Chinese patient. Chin Med J 1990, 103: 686-688.

[23] 23. Willige H. Uber Paralysis agitans in jugendlichen. Alter Ztschr Gesamte Neurol Psychiatr 1911,4: 520-587.

[24] 24. Yokochi M, Narabayashi H, Iizuka R, Nagatsu T. Juvenile parkinsonism: some clinical, pharmacological, and neuropathological aspects. Adv Neurol 1984, 40: 407-413.