Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement, dysautonomia, respiratory failure, and autoantibodies

Muscle & Nerve
Philippe HantsonPeter Van Den Bergh

Abstract

We examined a 27-year-old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain-Barré syndrome (GBS) or acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium-sensing receptor (CaSR) autoantibodies. The patient was initially diagnosed with GBS and treated with intravenous immunoglobulin (IVIg). After almost complete recovery, relapse with quadriplegia and respiratory failure was observed 12 weeks after motor symptom onset. She then received IVIg and steroid pulse therapy followed by maintenance oral methylprednisolone and plasma exchange. She recovered completely 4 months after the relapse. The further clinical and serological course was consistent with systemic lupus erythematosus (SLE)-associated CIDP. Herein we evaluate the association between A-CIDP and some biological markers of autoimmunity.

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Citations

May 6, 2010·Expert Review of Clinical Immunology·Eduardo A De Sousa
Apr 30, 2013·La Presse médicale·Peter Y K Van den Bergh, Yusuf A Rajabally
Jun 7, 2011·Seminars in Arthritis and Rheumatism·Brandusa FloricaPaul R Fortin
Apr 3, 2012·The International Journal of Neuroscience·Giuliana Galassi, Francesca Girolami
Dec 13, 2012·Autoimmunity Reviews·Remco K M A C LuijtenRonald H W M Derksen
Dec 20, 2016·Arquivos de neuro-psiquiatria·Helena AlessiOrlando G P Barsottini
Jan 1, 2021·Seminars in Arthritis and Rheumatism·Paulo Rogério JulioSimone Appenzeller
Dec 3, 2010·Current Opinion in Rheumatology

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