Falla auttonómica pura: Síndrome de Bradbury Eggleston

• Juan Idiáquez C ^[1] ; Horacio Kaufmann ^[3] ; Marco Soza M ^[2] ; Cecilia Necochea K ^[2]
  1. [1] Universidad de Valparaíso

     Universidad de Valparaíso

     Valparaíso, Chile

     
  2. [2] Pontificia Universidad Católica de Chile

     Pontificia Universidad Católica de Chile

     Santiago, Chile

     
  3. [3] Mount Sinai Medical School NY

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• Localización: Revista Médica de Chile, ISSN-e 0034-9887, Vol. 133, Nº. 2, 2005, págs. 215-218
• Idioma: español
• Títulos paralelos:
  • Pure autonomic failure. Bradbury Eggleston Syndrome: Case report
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• Resumen

  • 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 (Rev Méd Chile 2005; 133: 215-8)