Síndrome de Sneddon relato de três casos

Zaccariotti, Vladimir Arruda; Martins, Luiz Fernando; Costa, Valter da; Silva, Nilzio A. da; Casas, Alberto A. Las; Melo-Souza, Sebastião Eurico de

doi:10.1590/S0004-282X1995000100013

Resumos

Descrita por Sneddon (196S) a síndrome consiste na ocorrência de acidente vascular encefálico (AVE) isquêmico em pacientes com livedo reticular. Trata-se de doença vascular sistêmica de causa desconhecida, em que há comprometimento de artérias de médio e pequeno calibres. Pode haver positividade dos anticorpos antifosfolípides. São apresentados três casos desta entidade, todos do sexo masculino com 7, 16 e 54 anos de idade. Em todos a instalação do quadro deu-se por convulsões focais seguidas de hemiparesia ou paralisia labio-glossofaríngea. Os pacientes não tiveram outras manifestações neurológicas em 2 anos de acompanhamento. A investigação laboratorial demonstrou presença de anticorpos antifosfolípides e anticardiolipina em 1 caso. A investigação neuro-radiológica forneceu os seguintes resultados: TCC e RM com infarto nos 3 casos, e angiografia com obstrução de vasos de médio e pequeno calibre. A síndrome de Sneddon não parece ser tão rara e deve fazer parte de protocolos de investigação de AVE, especialmente em grupos mais jovens.

síndrome de Sneddon; doença cerebrovascular; anticorpos antifosfolípides

The Sneddon's syndrome consists of neurologic manifestations associated to the presence of livedo reticularis and cyanosis of the extremities. The pathological process is an endothelial obliteration of arterioles, leading to a reticular appearance of the skin, despite the environment temperature. The authors present three new cases, causasian males with 7, 16 and 54 years of age. The youngest started with hemilateralized motor seizures and showed a porencefalic area in the CT scan. The oldest had livedo reticularis, acrocyanosis and started with hemilateralized motor seizures, and a hemiparesis as sequela; CT scan with parasagittal infarct and occlusion presented of one anterior cerebral artery on angiography. The third patient started with hemifacial seizures, developed a labioglossolaringeal paresis and dysarthria as sequela; CT scan and MRI showed multiple infarcts, with multiple occlusions of cortical branchs on angiography. The skin biopsies showed endothelial vascular hyperplasia in all cases. Only one (54 years old) patient had a positive IgG antiphospholipid antibodies. The Sneddon's syndrome seems not to be so rare and have to be considered in the etiological investigation of cerebral infarcts, mainly in young people.

Sneddon's syndrome; cerebrovascular disease; antiphospholipid antibodies

Síndrome de Sneddon relato de três casos^* * Dr. Vladimir Arruda Zaccariotti - Praça T-18, 140 (Setor Bueno) - 74210-250 Goiânia Go - Brasil.

Sneddon's syndrome: report of three cases

Vladimir Arruda Zaccariotti^I; Luiz Fernando Martins^II; Valter da Costa^II; Nilzio A. da Silva^III; Alberto A. Las Casas^IV; Sebastião Eurico de Melo-Souza^V

^IMédico Residente, Instituto de Neurologia de Goiânia

^IINeurocirurgião, Instituto de Neurologia de Goiânia

^IIIReumatologista, Instituto de Neurologia de Goiânia

^IVCardiologista, Instituto de Neurologia de Goiânia

^VNeurologista, Coordenador de Ensino e Pesquisa

RESUMO

Descrita por Sneddon (196S) a síndrome consiste na ocorrência de acidente vascular encefálico (AVE) isquêmico em pacientes com livedo reticular. Trata-se de doença vascular sistêmica de causa desconhecida, em que há comprometimento de artérias de médio e pequeno calibres. Pode haver positividade dos anticorpos antifosfolípides. São apresentados três casos desta entidade, todos do sexo masculino com 7, 16 e 54 anos de idade. Em todos a instalação do quadro deu-se por convulsões focais seguidas de hemiparesia ou paralisia labio-glossofaríngea. Os pacientes não tiveram outras manifestações neurológicas em 2 anos de acompanhamento. A investigação laboratorial demonstrou presença de anticorpos antifosfolípides e anticardiolipina em 1 caso. A investigação neuro-radiológica forneceu os seguintes resultados: TCC e RM com infarto nos 3 casos, e angiografia com obstrução de vasos de médio e pequeno calibre. A síndrome de Sneddon não parece ser tão rara e deve fazer parte de protocolos de investigação de AVE, especialmente em grupos mais jovens.

Palavras-chave: síndrome de Sneddon, doença cerebrovascular, anticorpos antifosfolípides.

SUMMARY

The Sneddon's syndrome consists of neurologic manifestations associated to the presence of livedo reticularis and cyanosis of the extremities. The pathological process is an endothelial obliteration of arterioles, leading to a reticular appearance of the skin, despite the environment temperature. The authors present three new cases, causasian males with 7, 16 and 54 years of age. The youngest started with hemilateralized motor seizures and showed a porencefalic area in the CT scan. The oldest had livedo reticularis, acrocyanosis and started with hemilateralized motor seizures, and a hemiparesis as sequela; CT scan with parasagittal infarct and occlusion presented of one anterior cerebral artery on angiography. The third patient started with hemifacial seizures, developed a labioglossolaringeal paresis and dysarthria as sequela; CT scan and MRI showed multiple infarcts, with multiple occlusions of cortical branchs on angiography. The skin biopsies showed endothelial vascular hyperplasia in all cases. Only one (54 years old) patient had a positive IgG antiphospholipid antibodies. The Sneddon's syndrome seems not to be so rare and have to be considered in the etiological investigation of cerebral infarcts, mainly in young people.

Key words:Sneddon's syndrome, cerebrovascular disease, antiphospholipid antibodies.

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Aceite: 9-agosto-1994.

1. Brey RL, Hart RG, Sherman DG, Tegeles CH. Antiphospholipid antibodies and cerebral isquemia in young people. Neurology 1990, 40:1150-1196.
2. Burton TL. Livedo reticularis, porcelain, white scars, and cerebral thromboses. Lancet 1988, 1:1263-1265.
3. Champion R, Rook A. Livedo reticularis. Proc R Soc Med 1960, 53:961-962.
4. Cosmes A, Perroud AM, Mathieu A, Jourdain C, Touraine R. Livedo reticularis et accidents vasculaires cerebraux. Ann Dermatol Venereol 1986, 133:137-141.
5. Ellie E, Le Massa O, Julien J, Parneix I, Royer P, Beylot C. Gonadotrophins, livedo reticularis and stroke. Stroke 1190, 21:1377-1378.
6. Harris EN, Gharavi AE, Hughes GRY. Antiphospholipid antibodies. Clin Rheum Dis 1985, 11:591-609.
7. Kalashnikova CA, Asonde EL, Kushekbaeva AE, Graceva LA. Anticardiolipine antibodies in Sneddon's syndrome. Neurology 1990, 40:464-467.
8. Kalashnikova LA, Nasonov EL, Sotyanovich LZ, Kovalyov VU, Kosheleva NM, Reshetnysk TM. Sneddon's syndrome and the primary antiphospholipid syndrome. Cerebrovasc Dis 1994, 4:76-82.
9. Levine SR, Deegan MJ, Eutrell N, Welch KMA. Cerebrovascular and neurologic disease associated with antiphospholipid antibodies: 48 cases. Neurology 1990, 40:1181-1189.
10. Levine SR, Langer SL, Albers JW, Welch KMA. Sneddon's syndrome: an antiphospholipid antibody syndrome. Neurology 1988, 38:798-800.
11. Levine SR, Welch KMA. The spectrum of neurologic disorders associated with antiphospholipid antibodies: lupus anticoagulants and anticardiolipin antibodies. Arch Neurol 1987, 44:876-883.
12. Levine SR, Welch KMA, Langer SL, Albers WJ. Sneddon's syndrome (letter). Neurology 1989, 39:1138-1139.
13. Montalbon J, Ordi J, Barquinero J, Vilardell M. Sneddon's syndrome and anticardiolipine antibodies (letter). Stroke 1988, 19:785-786.
14. Routenberg W, Hennerici M, Aulich A, Holzke E, Zakomek HJ. Immunosuppressive therapy and Sneddon's syndrome. Lancet 1988, 2:629-630.
15. Rebollo M, Val JF, Garijo F, Quintana F, Berciano F. Livedo reticularis and cerebrovascular lesions (Sneddon's syndrome). Brain 1983, 106:965-979.
16. Rumpl E, Nechofer T, Pallus A, Willeit A, Vogl G, Stamplet G, Platz T.H. Cerebrovascular lesions and livedo reticularis (Sneddon's syndrome): a progressive cerebrovascular disorder? J. Neurol 1985, 231:324-330.
17. Schulze-Lahoff E, Kespl F, Bleil L, Bumester GR, Kolble K, Nublein HG, Rodl W, Kalden JR. IGM -containg immune complexes and antiphospholipid antibodies in patients with Sneddon's syndrome. Rheumatol Int 1989, 9:43-48.
18. Sneddon JB. Cerebrovascular lesions and livedo reticularis. Br J Dermatol 1965, 77:180-185.
19. Stephens WP, Ferguson JT. Livedo reticularis and cerebrovascular disease. Postgrad Med J 1982, 58:70-73.
20. Stockhammer G, Felber SR, Zelger B, Sepp N, Bisbsner GG, Frithch PO, Aichner FT. Sneddon's syndrome: diagnosis by skin biopsy and MRI in 17 patients. Stroke 1993, 24:685-690.

*

Dr. Vladimir Arruda Zaccariotti - Praça T-18, 140 (Setor Bueno) - 74210-250 Goiânia Go - Brasil.

Datas de Publicação

Publicação nesta coleção
19 Jan 2011
Data do Fascículo
Mar 1995

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

[1] 1. Brey RL, Hart RG, Sherman DG, Tegeles CH. Antiphospholipid antibodies and cerebral isquemia in young people. Neurology 1990, 40:1150-1196.

[2] 2. Burton TL. Livedo reticularis, porcelain, white scars, and cerebral thromboses. Lancet 1988, 1:1263-1265.

[3] 3. Champion R, Rook A. Livedo reticularis. Proc R Soc Med 1960, 53:961-962.

[4] 4. Cosmes A, Perroud AM, Mathieu A, Jourdain C, Touraine R. Livedo reticularis et accidents vasculaires cerebraux. Ann Dermatol Venereol 1986, 133:137-141.

[5] 5. Ellie E, Le Massa O, Julien J, Parneix I, Royer P, Beylot C. Gonadotrophins, livedo reticularis and stroke. Stroke 1190, 21:1377-1378.

[6] 6. Harris EN, Gharavi AE, Hughes GRY. Antiphospholipid antibodies. Clin Rheum Dis 1985, 11:591-609.

[7] 7. Kalashnikova CA, Asonde EL, Kushekbaeva AE, Graceva LA. Anticardiolipine antibodies in Sneddon's syndrome. Neurology 1990, 40:464-467.

[8] 8. Kalashnikova LA, Nasonov EL, Sotyanovich LZ, Kovalyov VU, Kosheleva NM, Reshetnysk TM. Sneddon's syndrome and the primary antiphospholipid syndrome. Cerebrovasc Dis 1994, 4:76-82.

[9] 9. Levine SR, Deegan MJ, Eutrell N, Welch KMA. Cerebrovascular and neurologic disease associated with antiphospholipid antibodies: 48 cases. Neurology 1990, 40:1181-1189.

[10] 10. Levine SR, Langer SL, Albers JW, Welch KMA. Sneddon's syndrome: an antiphospholipid antibody syndrome. Neurology 1988, 38:798-800.

[11] 11. Levine SR, Welch KMA. The spectrum of neurologic disorders associated with antiphospholipid antibodies: lupus anticoagulants and anticardiolipin antibodies. Arch Neurol 1987, 44:876-883.

[12] 12. Levine SR, Welch KMA, Langer SL, Albers WJ. Sneddon's syndrome (letter). Neurology 1989, 39:1138-1139.

[13] 13. Montalbon J, Ordi J, Barquinero J, Vilardell M. Sneddon's syndrome and anticardiolipine antibodies (letter). Stroke 1988, 19:785-786.

[14] 14. Routenberg W, Hennerici M, Aulich A, Holzke E, Zakomek HJ. Immunosuppressive therapy and Sneddon's syndrome. Lancet 1988, 2:629-630.

[15] 15. Rebollo M, Val JF, Garijo F, Quintana F, Berciano F. Livedo reticularis and cerebrovascular lesions (Sneddon's syndrome). Brain 1983, 106:965-979.

[16] 16. Rumpl E, Nechofer T, Pallus A, Willeit A, Vogl G, Stamplet G, Platz T.H. Cerebrovascular lesions and livedo reticularis (Sneddon's syndrome): a progressive cerebrovascular disorder? J. Neurol 1985, 231:324-330.

[17] 17. Schulze-Lahoff E, Kespl F, Bleil L, Bumester GR, Kolble K, Nublein HG, Rodl W, Kalden JR. IGM -containg immune complexes and antiphospholipid antibodies in patients with Sneddon's syndrome. Rheumatol Int 1989, 9:43-48.

[18] 18. Sneddon JB. Cerebrovascular lesions and livedo reticularis. Br J Dermatol 1965, 77:180-185.

[19] 19. Stephens WP, Ferguson JT. Livedo reticularis and cerebrovascular disease. Postgrad Med J 1982, 58:70-73.

[20] 20. Stockhammer G, Felber SR, Zelger B, Sepp N, Bisbsner GG, Frithch PO, Aichner FT. Sneddon's syndrome: diagnosis by skin biopsy and MRI in 17 patients. Stroke 1993, 24:685-690.