RESUMEN
Summary Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. In such situations, biopsy can be required and delay proper diagnosis. We report the case of a patient with SNM, who underwent biopsy on the assumption of it being a spinal cord tumor.
Resumo Mielopatia necrotizante subaguda (MNS) ou síndrome de Foix-Alajouanine é uma doença rara que se caracteriza por disfunção neurológica progressiva causada por uma fístula arteriovenosa espinal dural. O diagnóstico radiológico é comumente suspeitado quando aparece captação não específica de contraste e de artefatos de fluxo (flow voids) perimedulares. Raramente, a captação de contraste exibe o aspecto em anel, constituindo um grande desafio diagnóstico. Nesses casos, o principal diagnóstico diferencial é um tumor intramedular, e os pacientes são encaminhados para biópsia da lesão, atrasando o diagnóstico definitivo. Relatamos o caso de uma paciente com MNS, a qual foi submetida à biópsia da lesão em virtude de suspeita de tumor intramedular.
Asunto(s)
Humanos , Femenino , Anciano , Enfermedades de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Médula Espinal/diagnóstico por imagen , Fístula Arteriovenosa/diagnóstico por imagen , Médula Espinal/patología , Médula Espinal/diagnóstico por imagen , Enfermedades de la Médula Espinal/cirugía , Enfermedades de la Médula Espinal/patología , Neoplasias de la Médula Espinal/patología , Síndrome , Biopsia , Angiografía , Fístula Arteriovenosa/patología , Diagnóstico DiferencialRESUMEN
Acute or subacute myelopathy with spontaneous thrombosis of the veins in a patient with a spinal arteriovenous malformation(AVM) has been referred to as "Foix-Alajouanine syndrome(FAS)". A previously healthy 15-year-old girl developed neurological illness with progressive lower extremity weakness and low back pain. The MRI demonstrated the diffuse swelling of the spinal cord around the 10th thoracic level, suggesting myelopathy. Her neurological symptoms deteriorated to paraplegia, voiding difficulty and areflexia. Spinal arteriography did not demonstrate a vascular lesions such as arteriovenous malformation(AVM) or arteriovenous fistula(AVF). Operation revealed diffuse necrosis of the spinal cord and one abnormal thrombosed drainage vein. Progressive clinical illness, the histopathology of the patchy necrosis of spinal cord tissue and many thickened blood vessels are compatible with Foix-Alajouanine syndrome. We report a case of Foix-Alajouanine syndrome with a review of the literature.