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      Síndrome de Aicardi e papiloma do plexo coróide: uma associação rara. Relato de caso Translated title: Aicardi syndrome and choroid plexus papilloma: a rare association. Case report

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          Abstract

          Relata-se o caso clínico de uma criança de 5 meses de idade, do sexo feminino, com achados característicos da síndrome de Aicardi: agenesia do corpo caloso, espasmos infantis, anormalidades oculares ("chorioretinal lacunae" e microftalmia), atraso do desenvolvimento neuropsicomotor e malformações vertebrais com cifoescoliose. Os exames complementares confirmaram o diagnóstico e as imagens neurorradiológicas evidenciaram, além da agenesia do corpo caloso, massa localizada em região atrioventricular esquerda (papiloma do plexo coróide). A síndrome de Aicardi associada a papiloma do plexo coróide é rara, sendo este o sétimo caso relatado na literatura. Os autores sugerem que o papiloma do plexo coróide seja considerado tumor característico da síndrome de Aicardi, pois a sua freqüência é mais do que coincidental.

          Translated abstract

          The authors report a case of a 5 months old female child with clinical features of Aicardi syndrome: agenesis of the corpus callosum, occular abnormalities ("chorioretinal lacunae" and microphthalmus), infantile spasms, mental retardation, vertebral malformations and thoracic deformity. The pacient was submitted to complementary examinations that confirmed the diagnosis. The neuroradiologic images (MRI) showed besides corpus callosum agenesis a tumor located at the left ventricular atrium (choroid plexus papilloma). This association is a rare occurrence and the present case is the seventh described in literature. Furthermore, we suggest that the choroid plexus pappilloma could be a characteristic tumor of the Aicardi syndrome.

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          Author and article information

          Contributors
          Role: ND
          Role: ND
          Role: ND
          Role: ND
          Role: ND
          Role: ND
          Journal
          anp
          Arquivos de Neuro-Psiquiatria
          Arq. Neuro-Psiquiatr.
          Academia Brasileira de Neurologia - ABNEURO (São Paulo )
          1678-4227
          June 1996
          : 54
          : 2
          : 313-317
          Affiliations
          [1 ] Hospital de Base do Distrito Federal Brazil
          Article
          S0004-282X1996000200022
          10.1590/S0004-282X1996000200022
          8984993
          49e55842-50aa-47a7-8f65-a75afd4928d0

          http://creativecommons.org/licenses/by/4.0/

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          SciELO Brazil

          Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0004-282X&lng=en
          Categories
          NEUROSCIENCES
          PSYCHIATRY

          Neurosciences,Clinical Psychology & Psychiatry
          Aicardi syndrome,choroid plexus papilloma,síndrome de Aicardi,papiloma do plexo coróide

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