caa a22 4500 44535755X CHVBK 20180317142905.0 cr unu---uuuuu 170323e20111001xx s 000 0 eng 10.1007/s10072-011-0591-7 doi (NATIONALLICENCE)springer-10.1007/s10072-011-0591-7 A fatal case of Churg-Strauss syndrome presenting with acute polyneuropathy mimicking Guillain-Barré syndrome [Elektronische Daten] [Luisa De Toni Franceschini, Stefano Amadio, Marina Scarlato, Raffaella Fazio, Angelo Quattrini, Giacomo Dell'Antonio, Giancarlo Comi, Ubaldo Del Carro] 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, 30days 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. Springer-Verlag, 2011 Vasculitis nationallicence Peripheral neuropathy nationallicence Guillain-Barré syndrome nationallicence Five-factor score nationallicence Pseudoconduction block nationallicence De Toni Franceschini Luisa Laboratory of Neurophysiology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut Amadio Stefano Laboratory of Neurophysiology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut Scarlato Marina Neuropathology Unit, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut Fazio Raffaella Department of Neurology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 48, 20132, Milan, Italy aut Quattrini Angelo Neuropathology Unit, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut Dell'Antonio Giacomo Department of Surgical Pathology, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut Comi Giancarlo Department of Neurology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 48, 20132, Milan, Italy aut Del Carro Ubaldo Laboratory of Neurophysiology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut Neurological Sciences Springer Milan 32/5(2011-10-01), 937-940 1590-1874 32:5<937 2011 32 10072 https://doi.org/10.1007/s10072-011-0591-7 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10072-011-0591-7 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- De Toni Franceschini Luisa Laboratory of Neurophysiology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut NATIONALLICENCE 700 1- Amadio Stefano Laboratory of Neurophysiology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut NATIONALLICENCE 700 1- Scarlato Marina Neuropathology Unit, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut NATIONALLICENCE 700 1- Fazio Raffaella Department of Neurology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 48, 20132, Milan, Italy aut NATIONALLICENCE 700 1- Quattrini Angelo Neuropathology Unit, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut NATIONALLICENCE 700 1- Dell'Antonio Giacomo Department of Surgical Pathology, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut NATIONALLICENCE 700 1- Comi Giancarlo Department of Neurology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 48, 20132, Milan, Italy aut NATIONALLICENCE 700 1- Del Carro Ubaldo Laboratory of Neurophysiology, INSPE, San Raffaele Scientific Institute, Università Vita-Salute, Via Olgettina 60, 20132, Milan, Italy aut NATIONALLICENCE 773 0- Neurological Sciences Springer Milan 32/5(2011-10-01), 937-940 1590-1874 32:5<937 2011 32 10072 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer