title: [Pure autonomic failure. Bradbury Eggleston Syndrome. Case report].
abstract: Pure Autonomic Failure is a progressive, adult onset, degenerative disorder of the autonomic nervous system characterized clinically by orthostatic hypotension, bladder, sexual and sudomotor dysfunction. Since there are no other associated somatic neurological deficits, this condition must be considered in the differential diagnosis of orthostatic hypotension. We report a 64 years old man with a history of seven years of autonomic dysfunction, with severe orthostatic hypotension, erectile and bladder dysfunction. Autonomic tests showed low circulating norepinephrine levels, sweating abnormalities with regional anhydrosis of the left side of the trunk and abnormal cardiovagal response, indicating generalized autonomic failure. Peripheral somatic neuropathies with autonomic involvement were excluded by normal electrophysiologic tests and the patient was diagnosed pure autonomic failure. Treatment with fludrocortisone and midodrine improved orthostatic tolerance.
authors.affiliation: Cátedra de Neurología, Universidad de Valparaíso, Hospital Naval Almirante Nef, Viña del Mar Chile. idiaquez@123.cl
authors.forename: Juan
authors.initials: J
authors.lastname: Idiáquez
authors.forename: Horacio
authors.initials: H
authors.lastname: Kaufmann
authors.forename: Marco
authors.initials: M
authors.lastname: Soza
authors.forename: Cecilia
authors.initials: C
authors.lastname: Necochea
mesh_terms: D001342:Autonomic Nervous System Diseases / Q000175:diagnosis*; D003937:Diagnosis, Differential; D006801:Humans; D007024:Hypotension, Orthostatic / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D012119:Respiration; D013546:Sweating; D013577:Syndrome