case report
Wien Klin Wochenschr (2011) 123: 508–511 DOI 10.1007/s00508-011-0030-z © Springer-Verlag 2011 Printed in Austria
Wiener klinische Wochenschrift The Central European Journal of Medicine
Acute pandysautonomia – restitutio ad integrum by high prednisolone therapy Walter Struhal, Stephanie Hödl, Samy Mazhar, Gerhard Ransmayr Autonomic Unit, Department for Neurology and Psychiatry, AKH, Linz, Austria
Received November 12, 2010, accepted after revision June 14, 2011, published online July 25, 2011
Akute Pandysautonomie – restituito ad integrum mit Hochdosis-Predinsolon Zusammenfassung. Die akute Pandysautonomie ist eine seltene autonome Neuropathie, gekennzeichnet durch eine akute, schwere, einen Großteil des sympathischen und parasympathischen Systems einbeziehende Dysautonomie. Das somatische Nervensystem wird durch den Krankheitsprozeß nicht betroffen. Die Ursache ist oft nicht bekannt, aber virale Infektionen können der Erkrankung vorhergehen. In einigen Fällen war die Pandysautonomie Zeichen einer Paraneoplasie. In der Mehrzahl besteht eine schlechte Prognose mit einem chronischen Verlauf und Defektheilung. Der Fall einer 24 jährigen bisher gesunden Patientin wird präsentiert. Sie entwickelte eine Akkomodationsstörung, Pupillotonie, Schluckstörungen, gastrointestinale Beschwerden und eine atone Blase mit 750ml Restharn. Die Ewing Batterie bestätigte eine sympathische und parasympathsiche Funktionsstörung und führte zur Diagnose der akuten Pandysautonomie. Eine eingehende Diagnostik konnte keine Ursache für die akute Neuropathie bestätigen, insbesondere keine paraneoplastische oder infektiöse Ätiologie. Die Patientin wurde mit einer Hochdosis-Prednisolontherapie behandelt, eine restitutio ad integrum konnte erreicht werden. Summary. 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-yearold 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 para-
Correspondence: OA Dr. Walter Struhal, Abteilung für Neurologie und Psychiatrie, AKH Linz, Linz, Austria, E-mail:
[email protected]
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sympathetic 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. Key words: Pandysautonomia, autoimmune autonomic ganglionopathy, autonomic neuropathy, dysautonomia, prednisolone.
Introduction Acute pandysautonomia is a rare disease due to an acute sympathetic and parasympathetic postganglionic failure with relative preservation of motor and sensory function [1]. In 1969 this disease was described for the first time by Young and colleagues [2]. Since then about 40 cases have been published. Synonyms of acute pandysautonomia in the literature are: autoimmune autonomic ganglionopathy, autoimmune autonomic neuropathy, idiopathic autonomic neuropathy, or subacute autonomic neuropathy. The disease can affect both sexes and all ages. It might have an acute, subacute (up to 8 weeks) and gradual onset. Common symptoms include orthostatic hypotension, decreased papillary reactivity, anhidrosis, bladder dysfunction, gastrointestinal hypomobility, and sicca symptoms. Acute pandysautonomia is caused by an autoimmune autonomic ganglionopathy with antibodies against the ganglionic acetylcholine receptor in 50% of the cases [3]. Epstein Barr virus infection has been suspected to cause the syndrome [4] but so far no infectious agent was consistently confirmed [5]. Paraneoplastic disease was also found as a possible etiology in a few cases [6, 7]. Several treatment options are reported in the literature including symptomatic therapy only, intravenous immunoglobulin (IVIg), plasma exchange (PE), corticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil, and rituximab [8, 9]. Corticosteroids have been used in combination with IVIg, PE and other immunosuppressant agents; in one case as monotherapy only (no details avail-
Acute pandysautonomia – restitutio ad integrum by high prednisolone therapy
© Springer-Verlag 15–16/2011 wkw
case report
able) and are thought to be a treatment option in milder forms as short term therapy [8]. Only one in 3 cases have a favorable outcome, most show incomplete recovery with chronic autonomic dysfunction [3].
Case report A previously healthy 24-year-old woman suffered from a short episode of nausea and vomiting after eating 6–7 mandarins. Three days later she slowly developed constipation, dry mouth, swallowing difficulties and a dry right eye. One week later she observed dilated pupils in the mirror. She also observed, that her hands stopped sweating as usual. She sought medical attention two weeks after the condition started. Neurological signs were a pupillotonia with mydriasis on both eyes. The remaining neurological examination was unremarkable. Ewing battery on the second day after admission showed a reduced heart rate variability in relaxed lying (Coefficient of variance: 1.58 (normal value [10] 3.15)) and deep breathing (expiration/inspiration ratio: 1.11 (1.21)) (valsalva ratio: 1.36 (1.22)). Noradrenaline levels were markedly reduced (Plasma Noradrenaline supine and tilted:
