Abstract
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.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.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.
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