title: Acute pandysautonomia--restitutio ad integrum by high prednisolone therapy.
abstract: Acute pandysautonomia is a rare disease defined as acute widespread and severe sympathetic and parasympathetic failure and sparing of somatic nerve fibers. The causes of this syndrome are often an autoimmune disease leading to autonomic ganglionopathy. The majority of cases have a poor prognosis with a chronic debilitating course. We present a previously healthy 24-year-old female patient, who developed a loss of accommodation, pupillotonia, lacrimation, swallowing disturbances, gastrointestinal symptoms and an atonic bladder with 750 ml residual volume. The Ewing battery showed signs of parasympathetic and sympathetic dysfunction leading to the diagnosis of acute pandysautonomia. Further tests failed to find a cause of acute neuropathy especially where there was no evidence for paraneoplastic or infectious etiology. The patient was treated with high dose intravenous prednisolone and completely recovered.
authors.affiliation: Autonomic Unit, Department for Neurology and Psychiatry, AKH, Linz, Austria. walter.struhal@akh.linz.at
authors.forename: Walter
authors.initials: W
authors.lastname: Struhal
authors.forename: Stephanie
authors.initials: S
authors.lastname: Hödl
authors.forename: Samy
authors.initials: S
authors.lastname: Mazhar
authors.forename: Gerhard
authors.initials: G
authors.lastname: Ransmayr
mesh_terms: D000208:Acute Disease; D058668:Adrenergic alpha-1 Receptor Antagonists / Q000008:administration & dosage; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage* / Q000009:adverse effects; D018723:Bethanechol / Q000008:administration & dosage; D001794:Blood Pressure / Q000187:drug effects; D004305:Dose-Response Relationship, Drug; D017292:Doxazosin / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D004562:Electrocardiography / Q000187:drug effects; D005260:Female; D005772:Gastrointestinal Transit / Q000187:drug effects / Q000502:physiology; D006339:Heart Rate / Q000187:drug effects; D006801:Humans; D007262:Infusions, Intravenous; D009460:Neurologic Examination / Q000187:drug effects; D009638:Norepinephrine / Q000097:blood; D010277:Parasympathomimetics / Q000008:administration & dosage; D011239:Prednisolone / Q000008:administration & dosage* / Q000009:adverse effects; D054969:Primary Dysautonomias / Q000175:diagnosis / Q000188:drug therapy*; D011379:Prognosis; D014563:Urodynamics / Q000187:drug effects / Q000502:physiology
chemical_list: D058668:Adrenergic alpha-1 Receptor Antagonists; D000893:Anti-Inflammatory Agents; D010277:Parasympathomimetics; D018723:Bethanechol; D011239:Prednisolone; D017292:Doxazosin; D009638:Norepinephrine