RESUMO
BACKGROUND: Acrodysostosis is a rare syndrome characterized by peripheral dysostosis, nasal hypoplasia, and frequently mental retardation. Only two adult cases of acrodysostosis have been reported to have neurological symptoms. CASE DESCRIPTION: We report one additional adult case that presented with signs of spinal cord compression from spinal stenosis, and make the first histologic description in the literature of the bony anomalies seen in acrodysostosis. The patient had a T3 to T5 laminectomy and experienced a complete recovery. CONCLUSIONS: Special attention should be given to these patients to detect signs of spinal stenosis, as early decompression can lead to neurological recovery.
Assuntos
Descompressão Cirúrgica/métodos , Disostoses/complicações , Disostoses/cirurgia , Deficiência Intelectual/complicações , Deficiência Intelectual/cirurgia , Procedimentos Neurocirúrgicos/métodos , Osteocondrodisplasias/complicações , Osteocondrodisplasias/cirurgia , Canal Medular/cirurgia , Doenças da Coluna Vertebral/complicações , Doenças da Coluna Vertebral/cirurgia , Feminino , Humanos , Laminectomia , Exame Neurológico , Recuperação de Função Fisiológica , Canal Medular/patologia , Compressão da Medula Espinal/etiologia , Estenose Espinal/etiologia , Resultado do Tratamento , Adulto JovemRESUMO
Intracranial schwannomas not arising from a cranial nerve are very rare. Schwannomas of the dura are even rarer; in the literature, we found only two cases: a schwannoma of the falx and one of the torcula. We report a third case of a 9-year-old girl with a schwannoma of the tentorium cerebelli. The different theories concerning the origin of this tumor in this particular location are discussed.
