Headache and fainting as initial symptoms of syringomyelia associated to Arnold-Chiari and facial angiomatous nevus

Introduction. Syringomyelia and Arnold-Chiari malformation are two dysraphism that often appear together. Clinical manifestations are diverse although some patients remain asymptomatic for years. Syncope, supposedly due to a dysfunction of medulla baroceptors and spinal cord intermediolateral horn, is uncommon, particularly as the initial symptom. Case report. 34 year old woman, with a facial angiomatous nevus, presented with two episodes of headache followed by syncope; neurological examination was apparently normal. MRI showed mild supratentorial hydrocephalus, Arnold-Chiari malformation and cervical syringomyelia, with no vascular anomalies. Conclusion. This case, which shares some features with Sturge-Weber syndrome and PHACE syndrome, enhances the importance of the search for anomalies in patients with paroxistical symptoms and cutaneous lesions.