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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.53 no.1 São Paulo Mar. 1995

http://dx.doi.org/10.1590/S0004-282X1995000100013 

Síndrome de Sneddon relato de três casos*

 

Sneddon's syndrome: report of three cases

 

 

Vladimir Arruda ZaccariottiI; Luiz Fernando MartinsII; Valter da CostaII; Nilzio A. da SilvaIII; Alberto A. Las CasasIV; Sebastião Eurico de Melo-SouzaV

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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Full text available only in PDF format.

 

 

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Aceite: 9-agosto-1994.

 

 

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