Doença de Hallervorden-Spatz: descrição de um caso

Adamo Jr., J.

doi:10.1590/S0004-282X1993000100021

Resumos

É relatado o caso de paciente do sexo masculino de 19 anos de idade, com quadro progressivo de distúrbio de comportamento e de postura distônica, com início nas extremidades do hemicorpo direito há 5 anos. Ao exame apresenta síndrome extrapiramidal e piramidal, sendo o diagnóstico de doença de Hallervorden-Spatz confirmado pela ressonância nuclear magnética do crânio (RM). O objetivo deste registro é ressaltar a importância da RM no estudo de doenças degenerativas do sistema nervoso.

distonia; doença degenerativa; doença de Hallervorden-Spatz; ressonância nuclear magnética

It is reported the case of a male patient with 19 years old presenting progressive behavior disturbance and dystonic posture beginning on right, with approximately five years of evolution. Extrapyramidal and pyramidal syndrome was evidenced at examination. This finding suggests Hallervorden-Spatz disease which was confirmed by resonance imaging (MRI). The purpose of reporting this case is to call attention for the importance of MRI in the study of degenrative diseases of the nervous system.

dystonia; degenerative disease; Hallervorden-Spatz disease; magnetic resonance imaging

Doença de Hallervorden-Spatz: descrição de um caso

Hallervorden-Spatz disease: a case report

J. Adamo Jr.

Médico Assistente, Clínica Neurológica, Hospital do Servidor Público Municipal de São Paulo

RESUMO

É relatado o caso de paciente do sexo masculino de 19 anos de idade, com quadro progressivo de distúrbio de comportamento e de postura distônica, com início nas extremidades do hemicorpo direito há 5 anos. Ao exame apresenta síndrome extrapiramidal e piramidal, sendo o diagnóstico de doença de Hallervorden-Spatz confirmado pela ressonância nuclear magnética do crânio (RM). O objetivo deste registro é ressaltar a importância da RM no estudo de doenças degenerativas do sistema nervoso.

Palavras-chave: distonia, doença degenerativa, doença de Hallervorden-Spatz, ressonância nuclear magnética.

SUMMARY

It is reported the case of a male patient with 19 years old presenting progressive behavior disturbance and dystonic posture beginning on right, with approximately five years of evolution. Extrapyramidal and pyramidal syndrome was evidenced at examination. This finding suggests Hallervorden-Spatz disease which was confirmed by resonance imaging (MRI). The purpose of reporting this case is to call attention for the importance of MRI in the study of degenrative diseases of the nervous system.

Key words: dystonia, degenerative disease, Hallervorden-Spatz disease, magnetic resonance imaging.

Full text available only in PDF format.

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Aceite: 01-julho-1992.

Dr. Jayme Adamo Júnior Rua Conselheiro Furtado 1155 apto. 63 01511-001 São Paulo SP Brasil.

1. Beall SS, Patten BM, Mallette L, Jankpvoc J. Abnormal systemic metabolism of iron, porphyrin and calcium in Fahr's syndrome. Ann Neurol 1989, 26:569-575.
2. Bolshauser E, Lang W, Janzer R, Briner J, Spiess H, Kleihues P, Isler W. Computed tomography in Hallervorden-Spatz disease. Neuropediatrics 1987, 18:81-83.
3. Bresolin AU, Pascuzzi L, Melaragno RFş, Pecora M, Souza Dias JC. Hallervorden-Spatz syndrome-infantile neuroaxonal dystrophy complex: case report. Arq Neuro-Psiquiat 1988, 46:69-72.
4. Dooling EC, Richardson EPJr, Davis KR. Computed tomography in Hallervorden-Spatz disease. Neurology 1980, 30:1128-1130.
5. Jankovic J, Kirkpiatrick JB, Blomquist KA, Langlais PJ, Bird ED. Late onset Hallervorden-Spatz disease presenting as familial parkinsonism. Neurology 1985, 35:227-234.
6. Morphy MA, Feldman JA, Kilburn G. Hallervorden-Spatz disease. J Clin Psqchiatry 1989, 50:66-68.
7. Mutoh K, Okuno T, Ito M, Nakano S, Mikawa H, Fujisawa I, Asato R. MRI of group I case of Hallervorden-Spatz disease. J Comput Assist Tomogr 1988, 78:851-853.
8. Roy RC, McLain S, Wise A, Shaffner LS. Anesthesic management of a patient with Hallervorden-Spatz disease. Anesthesiology 1983, 58:382-384.
9. Russo LSJr. Focal dystonia and lacunar infarction of the basal ganglia. Arch Neurol 1983, 40:61-62.
10. Rutledge JN, Hilal SK, Silver AJ, Defendini R, Fahn S. Study of movement disorders and brain iron by MR. Am J Neuroradiol 1987, 8:397-411.
11. Schaffert DA, Johnsen SD, Johnson PC, Drayer BP. Magnetic resonance imaging in pathologically proven Hallervorden-Spatz disease. Neurology 1989, 39:440-442.
12. Sethi KD, Adams RJ, Loring DW, El Gammal T. Hallervorden-Spatz syndrome: clinical and magnetic resonance imaging correlations Ann Neurol 1988, 24:692-694.
13. Swaiman KF, Smith SA, Trock GL, Siddiqui AR. Sea blue histiocytes, lymphocytic cyto-somes, movement disorder and Fe-uptake in basal ganglia: Hallervorden-Spatz disease or ceroid storage with abnormal isotope scan? Neurology 1983, 33:301-305.
14. Swaiman KF. Hallervorden-Spatz syndrome and brain iron metabolism. Arch Neurol 1991, 48:1285-1293.

Datas de Publicação

Publicação nesta coleção
19 Jan 2011
Data do Fascículo
Mar 1993

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

[1] 1. Beall SS, Patten BM, Mallette L, Jankpvoc J. Abnormal systemic metabolism of iron, porphyrin and calcium in Fahr's syndrome. Ann Neurol 1989, 26:569-575.

[2] 2. Bolshauser E, Lang W, Janzer R, Briner J, Spiess H, Kleihues P, Isler W. Computed tomography in Hallervorden-Spatz disease. Neuropediatrics 1987, 18:81-83.

[3] 3. Bresolin AU, Pascuzzi L, Melaragno RFş, Pecora M, Souza Dias JC. Hallervorden-Spatz syndrome-infantile neuroaxonal dystrophy complex: case report. Arq Neuro-Psiquiat 1988, 46:69-72.

[4] 4. Dooling EC, Richardson EPJr, Davis KR. Computed tomography in Hallervorden-Spatz disease. Neurology 1980, 30:1128-1130.

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

[6] 6. Morphy MA, Feldman JA, Kilburn G. Hallervorden-Spatz disease. J Clin Psqchiatry 1989, 50:66-68.

[7] 7. Mutoh K, Okuno T, Ito M, Nakano S, Mikawa H, Fujisawa I, Asato R. MRI of group I case of Hallervorden-Spatz disease. J Comput Assist Tomogr 1988, 78:851-853.

[8] 8. Roy RC, McLain S, Wise A, Shaffner LS. Anesthesic management of a patient with Hallervorden-Spatz disease. Anesthesiology 1983, 58:382-384.

[9] 9. Russo LSJr. Focal dystonia and lacunar infarction of the basal ganglia. Arch Neurol 1983, 40:61-62.

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

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

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

[13] 13. Swaiman KF, Smith SA, Trock GL, Siddiqui AR. Sea blue histiocytes, lymphocytic cyto-somes, movement disorder and Fe-uptake in basal ganglia: Hallervorden-Spatz disease or ceroid storage with abnormal isotope scan? Neurology 1983, 33:301-305.

[14] 14. Swaiman KF. Hallervorden-Spatz syndrome and brain iron metabolism. Arch Neurol 1991, 48:1285-1293.