Orthostatic intolerance and syncope associated with Chiari type I malformation

Abstract

The Chiari type I malformation (CM1) is characterized by herniation of cerebellar tonsils to at least 3-5 mm below the plane of foramen magnum and can present with a wide variety of clinical symptoms, frequently including occipital headaches, secondary to bulbar and/or medullary distress. Rarely, syncopal episodes have also been described and attributed to either compression of the midbrain ascending reticular system, or vascular compromise (vertebrobasilar artery compression, hypotension). We report the first case of a CM1 patient with frequently recurring syncope due to postural orthostatic tachycardia syndrome (POTS), a form of orthostatic intolerance, whose symptoms resolved completely after surgical intervention. It is important to stress that it is not clear whether the described association of POTS and CM1 in our patient is a fortuitous finding in an isolated case or a reflection of a more systematic association between the two pathologies.