title: A fatal case of Churg-Strauss syndrome presenting with acute polyneuropathy mimicking Guillain-Barré syndrome.
abstract: A 64-year-old woman, with asthma and sinusal polyposis in her history, suddenly developed a painful polyneuropathy with diplopia. Nerve conduction studies, performed at the very onset of the neuropathy, could not definitely rule out a Guillain-Barré syndrome (GBS) and high-dose i.v. immunoglobulins were administered. Clinical and laboratory findings subsequently supported the diagnosis of Churg-Strauss syndrome; corticosteroid therapy was started and clinical stabilisation of neuropathy was apparently achieved. No indicators of unfavourable outcome were present at that time. Nevertheless, 30 days after the onset the patient acutely worsened with severe polyneuropathy relapse and fatal systemic diffusion to heart, kidney and mesenteric district, which a single cyclophosphamide pulse failed to control. This case highlights the possibility that a GBS-like onset of Churg-Strauss syndrome neuropathy should be regarded as a part of multiorgan, severe or even life-threatening vasculitic involvement, requiring the most aggressive treatments, regardless of the presence of recognised factors of poor outcome.
authors.affiliation: Laboratory of Neurophysiology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132 Milan, Italy. detonifranceschini.luisa@hsr.it
authors.forename: Luisa
authors.initials: L
authors.lastname: De Toni Franceschini
authors.forename: Stefano
authors.initials: S
authors.lastname: Amadio
authors.forename: Marina
authors.initials: M
authors.lastname: Scarlato
authors.forename: Raffaella
authors.initials: R
authors.lastname: Fazio
authors.forename: Angelo
authors.initials: A
authors.lastname: Quattrini
authors.forename: Giacomo
authors.initials: G
authors.lastname: Dell'antonio
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Comi
authors.forename: Ubaldo
authors.initials: U
authors.lastname: Del Carro
mesh_terms: D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D020275:Guillain-Barre Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D011115:Polyneuropathies / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents