title: Postural tachycardia syndrome in syringomyelia: response to fludrocortisone and beta-blockers.
abstract: Orthostatic intolerance is occasionally reported by patients with syringomyelia and is usually attributed to vestibular symptoms or neurogenic orthostatic hypotension. Postural tachycardia syndrome has not been previously described in syringomyelia. A patient with long-standing syringomyelia and a Chiari type I anomaly developed disabling "panic-like" attacks associated to orthostatic intolerance five years after posterior fossa decompression and shunting of the syrinx. A head-up tilt test showed an early phase of postural orthostatic tachycardia followed by progressive arterial hypotension and bradycardia as seen in neurally mediated syncope. A magnetic resonance imaging scan showed a collapsed syrinx from the 3rd cervical to the 12th thoracic vertebra without syringobulbia. Fludrocortisone and beta-blockers led to resolution of symptoms. Partial sympathetic denervation of the legs in syringomyelia might explain the occasional occurrence of postural tachycardia syndrome. Postural tachycardia syndrome may be included as a possible cause of orthostatic symptoms in syringomyelia patients.
authors.affiliation: Department of Neurology, Raúl Carrea Institute for Neurological Research (FLENI), Buenos Aires, Argentina. mnogues@fleni.org.ar
authors.forename: M
authors.initials: M
authors.lastname: Nogués
authors.forename: R
authors.initials: R
authors.lastname: Delorme
authors.forename: D
authors.initials: D
authors.lastname: Saadia
authors.forename: K
authors.initials: K
authors.lastname: Heidel
authors.forename: E
authors.initials: E
authors.lastname: Benarroch
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001341:Autonomic Nervous System / Q000503:physiopathology; D005260:Female; D005438:Fludrocortisone / Q000627:therapeutic use*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D011187:Posture / Q000502:physiology*; D013595:Syringomyelia / Q000150:complications* / Q000503:physiopathology / Q000523:psychology; D013610:Tachycardia / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology
chemical_list: D000319:Adrenergic beta-Antagonists; D000893:Anti-Inflammatory Agents; D005438:Fludrocortisone